A 16-year-old boy gave a history of pain in his knee area for six months and a palpable tumor mass for the last two months. This AP radiograph of the distal femur shows permeative lytic changes in the distal metaphysis on the lateral side with cortical breakthrough and a slight Codman's reactive triangle proximally.
On this technetium bone scan there is evidence of bony activity in the distal femoral metaphysis laterally suggesting either a neoplasm, infection, or possible injury.
Sagittal T1-weighted MRI of the distal femur shows extensive low signal abnormality throughout the distal femoral metaphysis with sharp margination proximally between the high signal fat above and the low signal tumor below. This helps the surgeon determine the level of resection. Notice the breakout posteriorly a distance of approximately 2 cm which is hard to see because of the minimal contrast to the low signal muscle tissue posteriorly.
Coronal T2-weighted MRI of the distal femur shows the high signal features of an osteosarcoma filling the entire distal metasphysis and demonstrates beautifully the breakout into the adjacent soft tissues a distance of 1-2 cm. The tumor is easy to see because it contrasts against the low signal normal tissues.
Axial T1-weighted MRI demonstrates the low signal tumor in the medullary canal with permeation through the cortex and circumferential tumor invasion nearly 2 cm away from the metaphyseal cortical bone. The popliteal vessels are directly posterior to the tumor mass with approximately 2-3 mm of normal high signal fat separating the vessels from the tumor.
An axial T2-weighted MRI showing the high signal features of the soft tissue tumor outside the cortex on the back side of the distal femoral metaphysis.
The chest CT scan which is part of the routine staging process reveals early pulmonary metastases in both lungs, demonstrated by white spots seen in a subpleural position, measuring approximately 1 cm in diameter. The presentation of pulmonary metastases prior to chemotherapy indicates a poor prognostic outlook for this patient.
This gross specimen is a typical amputation specimen of an osteosarcoma of the distal femur with circumferential extracortical tumor mass present to such an extent that a limb salvage procedure could not be considered.
On this gross macrosection of the entire distal femur, there is evidence of the tumor originating in the medullary canal of the femur between the growth plate distally and the fatty marrow proximally. There is extensive tumor tissue outside the bone under the periosteum with a fairly prominent Codman's reactive triangle proximally.
Close-up macrosection of the same specimen shows in better detail the Codman's reactive triangle that is an interface between reactive bone produced beneath the elevated periosteum and subadjacent osteosarcoma.
Low power photomicrograph shows evidence of neoplastic osteoid formation. The darker portion is mineralized osteoid and the lighter pink tissue is the unmineralized osteoid that has a chaotic pattern suggesting a bone-forming sarcoma.
Under higher power there is evidence of malignant osteoblasts with a pleomorphic hyperchromatic appearance forming young tumor osteoid which is diagnostic of classic osteosarcoma.
Another high power photomicrograph showing bizarre anaplastic osteoblasts forming young tumor osteoid that is diagnostic of a high-grade bone-forming sarcoma.
Lateral radiograph of a 14-year-old girl with a classic osteosarcoma of the distal femur. There are dense osteosclerotic changes seen in the entire distal femoral metasphysis with cortical breakthrough anteriorly, strongly suggestive of the diagnosis of an osteoblastic osteosarcoma.
Coronal T1-weighted MRI of the distal femori shows the typical low signal abnormality displacing the normal fat in the distal four inches of the left femoral metaphysis with evidence of early breakout circumferentially under the periosteum.
Coronal T2-weighted MRI shows the high signal features of osteosarcoma filling the distal end of the femoral metaphysis with high signal abnormality circumferentially under the periosteum.
This patient was treated with systemic adjuvant chemotherapy for approximately two months prior to limb salvage resection of the distal 20 cm of the femur and reconstructed by means of a rotating hinge prosthesis with a cemented tibial component and proximal fixation with a new technique referred to as a compression prosthesis or compliant pre-stress system (ComPreSs, Biomet, Inc., Warsaw, IN) with an 800 pound Belleville washer spring to fix the prosthetic device to the proximal stump of the femur. This operative photograph shows the early exposure for the resection of the distal femur with a special measuring device in position to mark the exact 20 cm level of resection in the midshaft of the femur.
The resected distal end of the femur with the adjacent proximal tibial epiphyseal area. Tumor is seen circumferentially surrounding the entire distal femur with a wide margin of resection so as to reduce the chance for local recurrence to approximately 5-7%.
The metal guidance tower is placed on the top of the tibial resection and the reamer is in position to prepare the medullary canal of the tibia for cementing of the tibial component.
The drill guide system is utilized to place 5 transverse fixation pins in the femoral diaphysis for fixation of the anchor plug which gives the compression fixation necessary to stabilize the prosthesis to the proximal femur.
The surgeon drills holes through the femoral diaphysis utilizing the drill guide system in position.
The traction bar protrudes from transected femur after the distal stump of the femur has been milled with a special high compliance reaming device in preparation for placement of the spindle.
The spindle of the ComPreSs device is in position on the femoral stump with the T-bar wrench in position to tighten the compression nut that transfers the 800 pounds of compression from the prosthetic device onto the interface between the spindle and the femoral interface.
The compression plug has been removed and the compression nut on top of the stacked Belleville washers that creates the 800 pounds of pressure on the prosthetic-bone interface can be seen.
The spindle is in position with the intercalary portion of the prosthetic device placed over the spindle flanked by a 2° morse taper lock. Porous plasma spray titanium can be seen on the surface of the intercalary segment to allow for soft-tissue ingrowth.
Photograph taken at completion of the prosthetic reconstruction showing the rotating hinge to the left and the intercalary porous device to the right, measuring a total of 20 cm. The patella can be seen retracted at the top so that the entire extensor mechanism is still intact and ready for closure and early range of motion activity.
AP radiograph taken immediately following the procedure with the prosthesis in position. The anchor plug is seen at the top inside the medullary canal and the Finn rotating hinge knee (Biomet, Warsaw, IN) in place distally.
Close-up lateral radiograph of the ComPreSs showing the five transverse pins used for fixation of the anchor plug, and the interface between the porous spindle device and the distal stump of the femur.
AP radiograph in another similar case showing what the interface between the prosthesis and the bone looks like five years after the procedure. There is hypertrophy and reactive new bone formation growing into the porous interface of the spindle demonstrating a stable osseointegration that is not seen with the cemented stems that are typically used for limb salvage today, It is hoped that this new compression fixation device will greatly reduce mechanical failures of prosthetic fixation because of its stress sharing capability and osseointegration that prevents polyethylene debris from gaining access to the medullary canal and stimulating macrophage osteolysis.
A 16-year-old girl gave a history of a painful mass in her proximal anterior thigh for four months. This coronal T2-weighted MRI shows a high signal abnormality arising from the upper portion of the femoral diaphysis with extensive high signal extension out into the surrounding soft tissue circumferentially, especially on the lateral side under the vastus lateralis muscle. There are lower signal characteristics in the central portion that represent calcification. The diagnosis of osteogenic sarcoma was established by means of a small incisional biopsy.
Axial gadolinium contrast MRI at the mid-portion of the tumor shows a high signal lesion located within the medullary canal and breaking out extensively into the surrounding soft tissue. There is a fairly large area of signal void secondary to necrosis within the central portion of the tumor.
After two cycles of chemotherapy, the patient was taken to the operating room and a wide resection of the proximal half of the femur was carried out. This is the resected specimen with the femoral head and neck to the left side and a cuff of normal muscle tissue completely surrounding the osteogenic sarcoma.
Following resection of the tumor, the patient was reconstructed using a proximal femoral replacement bipolar prosthesis with a ComPreSs (Biomet, Warsaw, IN) system to attach the prosthesis to the lower end of the femur. This photograph shows distal stump of the femur with the anchor plug and traction bar placed into the medullary canal in preparation for placement of the spindle over the traction bar.
The spindle is placed over the traction bar and the Belleville washers have been compressed with a T-bar wrench in order to create 800 pounds of pressure between the porous portion of the implant and the proximal stump of the femur to allow for osteointegration within 6 months without stress-shielding the femur.
Operative photograph showing the entire modular prosthetic device attached to the distal femur. The bipolar system is ready for reduction into the normal acetabulum. Notice that one of the tooth washers is in position on the prosthetic trochanter. It will be utilized to fix the abductor gluteus medius tendon to the implant before closure.
Close-up picture of the proximal portion of the prosthesis with a better view of the tooth washer in position on the greater trochanter.
Photograph showing the bipolar prosthesis reduced into the acetabulum in preparation for attachment of the gluteus medius tendon to the prosthetic trochanter.
Photograph following fixation of the abductor mechanism down to the prosthetic trochanter with two titanium tooth washers. The muscle belly of the vastus lateralis muscle is seen distal to the washers.
Surgical specimen after it was cut in the pathology lab showing the large soft tissue component of the osteogenic sarcoma bulging laterally to the upper femur.
AP radiograph of the prosthetic reconstruction with the bipolar device above and the ComPreSs device below with five pins across the anchor plug holding the spindle tightly onto the distal stump of the femur with 800 pounds of spring pressure, This patient did well following the surgery. She walks with a normal gait and had no signs of local recurrence two years later.
Here we see the radiographic appearance 5 years post op with excellent osteointegration and no tumor recurrence or pain symptoms. She has nearly normal function and walks without a limp.
A 17-year-old boy had noted pain in his thigh for six months. This AP radiograph of the femur shows a motheaten, lytic process within the distal diaphysis of the femur with extensive sclerotic periosteal new bone formation. The lesion proved to be a high-grade classic osteogenic sarcoma.
Lateral radiograph of the femur showing motheaten, lytic destructive changes of the distal metaphyseal-diaphyseal portion of the femur with breakout anteriorly and periosteal new bone formation.
Sagittal T1-weighted MRI of a similar case shows a low signal abnormality filling the entire mid-portion of the diaphysis of the femur, eroding into the cortex and breaking out anteriorly into the anterior compartment muscles.
Photograph of the thigh several weeks after the incisional biopsy that was placed properly through the vastus medialis muscle so as to avoid contamination of other major compartments of the thigh. The biopsy site can easily be excised later at the time of limb salvage reconstruction. As a result of the positive diagnosis of osteosarcoma, the patient was placed on adjuvant chemotherapy for two months prior to the limb salvage reconstruction.
Low power photomicrograph of the biopsy specimen shows tumor osteoid formation being produced by a malignant-appearing, hyperchromatic, pleomorphic osteoblastic cell which clearly signifies the diagnosis of a high-grade osteosarcoma.
This patient was taken to the operating room and a wide resection of the distal 10 inches of the femur was carried out. Photograph of the resected specimen shows the cuff of normal muscle surrounding the bulge of the tumor site with the skin biopsy site still intact with the subadjacent specimen, signifying a good wide resection of the tumor.
The resected distal femur was taken to the pathology laboratory where it was cut with band saw. There is significant necrosis within the tumor mass as a result of successful adjuvant chemotherapy. Microscopically, the pathologists found 95% necrosis of the tumor tissue. With a favorable response like this, there is a good chance the patient will live past five years in 80% of cases.
Photograph taken following resection of the distal femur shows the large surgical defect that requires a reconstruction with a limb salvage prosthetic device.
Typical modular tumor system for resection of the distal femur, utilizing a standard rotating hinge total knee device for excellent functional result following the procedure. These components are made of titanium with porous pads on the shoulder area. There is a taper lock linkage between the various length intercalary components and the rotating hinge device.
Typical modular tumor system for resection of the distal femur, utilizing a standard rotating hinge total knee device for excellent functional result following the procedure. These components are made of titanium with porous pads on the shoulder area, There is a taper lock linkage between the various length intercalary components and the rotating hinge device.
Surgical photograph following completion of the limb salvage procedure with the modular rotating hinge device in position, ready for wound closure.
Postoperative radiograph showing the patient's knee seven years following the limb salvage procedure. There is no evidence of local recurrence and the patient did not develop pulmonary metastases. He had equal leg lengths with excellent function but was warned against impact loading activities. Note the extensive osteopenia of the cortical bone just above the shoulder of the implant, secondary to stress shielding, that is one of the major concerns with cemented, rigid stem devices and results in mechanical loosening in about 50% of cases at ten years. There has been an attempt to solve this problem with the ComPreSs device engineered by Biomet, Inc. (Warsaw, IN).
AP radiograph of the distal femur of a 13-year-old boy shows a motheaten, lytic process of the distal metaphysis with extensive breakout into the surrounding soft tissue. There is reactive new bone formation and a classic Codman's reactive triangle, quite suggestive of osteosarcoma.
Sagittal T1-weighted MRI shows a low signal abnormality in the distal femoral metaphysis that is breaking out posteriorly into the popliteal space, butting close to the popliteal artery that lays directly on the soft tissue component of the tumor.
Axial T1-weighted MRI shows low signal tumor activity filling the entire medullary canal, breaking out through the posterior cortex into the popliteal space a distance of 2-3 cm and coming within 5-6 mm of the popliteal artery posteriorly.
Photomicrograph of the biopsy specimen that was taken before the start of chemotherapy shows malignant-appearing osteoid in a lacey pattern being produced by aggressive, malignant-appearing, hyperchromatic nuclei in osteoblastic cells. This picture is quite diagnostic of a high-grade osteoblastic osteosarcoma.
The patient was immediately placed on adjuvant chemotherapy for a period of two months, following which a wide resection of the distal femur was carried out in preparation for a limb salvage reconstruction with a rotating hinge total knee and an expandible device in the femoral component. The resected specimen was taken to the pathology lab and cut with a band saw, demonstrating a very clear margin of resection in the medullary canal. The growth plate is still open which is a strong indication for utilizing an expandible device.
Surgical photograph showing the rotating hinge prosthesis in position with an expandible, telescoping sleeve just above the large femoral component that will allow for repeated surgical expansions during the remaining growth of the patient.
This photograph shows the expandible device open temporarily a distance of about 2 cm to demonstrate how much lengthening will occur within the next year following this primary procedure.
Lateral radiograph of the reconstructed distal femur with a cemented stem in the upper femur. The patient was expanded on two occasions and later his stem loosened, requiring placement of a ComPreSs system (Biomet.Inc., Warsaw, IN) for fixation to the remaining portion of the upper femur. However, the area became infected and the entire reconstruction was removed and a total femur put in place. The patient was cured of both his infection and his sarcoma and had an excellent end result.
AP radiograph of the distal femur of a 23-year-old female shows a lytic destructive lesion arising from the medullary canal of the distal femur with cortical breakthrough that on biopsy proved to be a classic osteoblastic osteosarcoma.
The patient was placed on adjuvant chemotherapy and following two months of treatment was taken to the operating room where the distal femur was widely resected, This is a photograph of the surgical specimen following resection.
A photomicrograph from the biopsy specimen shows the lacey, neoplastic, osteoid formation being produced by malignant-appearing osteoblasts, determing the diagnosis of osteoblastic osteosarcoma.
Surgical photograph taken at the time of the reconstruction with a modified spherocentric knee and a modular tumor system designed in the early 1980's. The polyethylene button has already been cemented in position.
Surgical photograph showing the entire knee reconstruction in position with a cemented stem above, readying for wound closure.
Lateral view of the limb salvage reconstruction just before closure.
Postoperative radiograph taken immediately after the surgery with a cemented stem in position in the proximal femur. The patient completed her six months of chemotherapy and then disappeared from follow-up for 13 years, at the end of which time she returned with severe pain in her hip area and 2 inches of shortening.
Radiograph taken 13 years following surgery shows severe stress shielding of the remaining portion of the upper femur with total subsidence of the stem up through the greater trochanter and a fracture through the neck of the femur that made it impossible for further local reconstruction without a total femoral replacement.
Surgical specimen resulting from the total femoral replacement, demonstrating the extensive degree of stress shielding in the upper end of the femur that resulted from a stiff cemented stem in the femoral diaphysis over a period of 13 years. This situation might have been avoided with use of the ComPreSs (Biomet, Inc., Warsaw, IN) device that reduces dramatically the amount of stress shielding in the upper femur.
Radiograph following the total femoral replacement at which time full length and function was restored to the leg.
A 17-year-old boy gave a history of pain in his hip for three months. This AP radiograph shows motheaten, lytic destruction of the peritrochanteric area of the right hip that on biopsy turned out to be a high-grade osteogenic sarcoma.
Lateral radiograph of the hip joint with extensive motheaten destruction of the head, neck and trochanteric portion of the upper femur, highly suggestive of a malignant process.
Technetium bone scan shows increased activity in the upper 5 inches of the proximal femur, suggesting extensive metabolic activity in the area compatible with high-grade sarcoma.
Coronal T1-weighted MRI of the proximal femur showing a low signal neoplastic infiltration involving the upper 5 inches of the femur with cortical breakout medially a distance of about 2 cm. This image allows the reconstructive surgeon to design a custom implant to replace the upper femur.
Axial T1-weighted MRI shows the low signal neoplastic activity within the medullary canal breaking out through the cortex anteriorly a distance of approximately 3 cm and coming within 5-6 mm of the femoral vessels which will allow the reconstructive surgeon to get a clean margin at this level.
Low power photomicrograph of the biopsy specimen that was taken through a small percutaneous trochanteric approach showing neoplastic osteoid being produced by pleomorphic and hyperchromatic osteoblastic tumor cells.
After two months of preoperative adjuvant chemotherapy the patient was taken to the operating room and a modular tumor was system was used to reconstruct the upper end of the femur. This photograph shows the various length modular components of the device with a 6 inch titanium stem that is cemented into the distal femur and a taper lock system above for either a bipolar or total hip replacement. The holes placed in the bend of the upper component allow for reattachment of the gluteus medius tendon.
Photograph taken just before the incision is made with the biopsy placed properly over the greater trochanter and the surgeon's inked outline of his approach to the reconstructive surgery.
Photograph of the resected specimen with a large cuff of normal muscle around the upper end of the femur and the skin biopsy site still intact with the subadjacent proximal femur.
The surgical specimen was sent to the pathology lab where it was sectioned with a band saw. This photograph shows the tumor nicely encased by normal tissue with about a 3 cm margin in the medullary canal distally. The tumor tissue was found to be extremely necrotic as a result of preoperative chemotherapy with a 93% necrosis, giving this patient an excellent chance for survival (about 70-80%).
The surgical wound created from the resection of the upper 7 inches of the proximal femur, ready for a modular tumor reconstruction.
Close-up photograph of the acetabulum following the resection showing the hyperemic changes in the acetabular synovial tissues, a response to the antigen stimulation of the nearby tumor in the upper femur. There is no crossover into the acetabular structures preventing the need for a more aggressive internal hemipelvectomy reconstruction.
Surgical photograph taken after the modular tumor system was placed in the acetabulum showing the surgeon attaching the gluteus medius tendon to the holes in the intercalary component and, at the same time, attaching the tendon to the surrounding soft tissue including the fascia lata and the vastus lateralis of the quadriceps mechanism.
The sutures are tied down over the trochanteric portion of the device and the fascia of the vastus lateralis is ready to be sutured into position to close the deep tissue, allowing for excellent abduction within two months after the surgery.
Postoperative radiograph of the reconstruction with a cemented total hip in position. At 7 years following the procedure, the patient was doing well without local recurrence and had excellent function of his limb salvage reconstruction.
AP radiograph of a 7-year-old boy with a classic osteosarcoma of the distal femur showing sclerotic changes throughout the distal metaphysis of the femur with associated neoplastic bone formation seen circumferentially beneath the periosteum with a typical Codman's reactive triangle at the upper end.
Technetium bone scan shows increased uptake in the area of the distal right femur in the location of the primary sarcoma.
Sagittal T1-weighted MRI shows a low signal tumor filling the entire distal femoral metaphysis up to the growth plate with a significant breakout posteriorly into the popliteal space butting directly up against the popliteal artery.
Coronal T2-weighted MRI shows the high signal characteristics of the subperiosteal mass surrounding the entire distal femoral metaphysis and the low signal heavily ossified portion of the tumor seen in the medullary canal proximal to the growth plate, A brighter signal is seen more proximally in the canal where there is less ossification within the tumor.
Axial T1-weighted MRI shows the low signal tumor within the canal, breaking out through the cortex circumferentially a distance of 1-2 cm. There is a large breakout posteriorly into the popliteal space butting directly up against the popliteal artery.
Because of this patient's young age and small size, it was felt that an expandible prothetic device would not be practical because of the small size required and the many years of growth of this patient necessitating multiple revisions to expand the implant. A modified amputation by means of a Van Ness turn-up-plasty was elected for the reconstructive procedure. In this procedure, the bone amputation level is high above the tumor in the proximal femur but the soft tissue amputation is distal just above the knee, utilizing the proximal tibia as a vascularized graft. The tibia is turned upside down into the defect created by the bone resection, leaving the patient with a functional end result similar to a routine supracondylar amputation. This photograph shows the traditional design of the skin flaps for a supracondylar amputation.
The proximal 10 inches of the tibia has been mobilized from its surrounding soft tissue, leaving only the anterior compartment and the deep posterior compartment intact. The popliteal vessels are intact proximally just beneath the tibial plateau, still supplying vascular nutrition to the resected tibia.
In this surgical photograph, the surgeon has placed the proximal 10 inches of the tibia upside down into the thigh with a sideplate utililized proximally to fix the distal part of the tibia to the proximal part of the femur. We are looking directly at the tibial plateau surface of the knee joint that now acts as the distal end of the femoral stump to replace the resected distal femur. The resected distal femur and its tumor content is seen lying next to the wound.
In this photograph taken several months later, the wound is nicely healed and the patient has a classic supracondylar amputation that gives excellent function with a suction socket, compared to a higher level amputation that would not function nearly as well.
AP radiograph taken several weeks following the Vanness turn-up-plasty shows the upside down tibial graft in excellent position with the proximal tibial growth plate seen distally taking on the appearance of resected distal femur but without the tumor.
Radiograph taken a year and a half later shows excellent healing of the two viable bones with no evidence of tumor recurrence.
Radiograph taken 5 years postoperative showing the excellent healing of the two viable bone structures with the proximal tibial growth plate in the distal thigh area still growing normally to keep up with the growth of the patient until maturity.
AP radiograph of the distal femur of a 17-year-old girl who sustained a pathological fracture through a relatively small diaphyseal osteogenic sarcoma that had been diagnosed by an open biopsy. At that time the surgeon elected to fix the fracture site with multiple short side plates in order allow for a wide resection at a later date with a double ComPreSs reconstruction rather than perform a complete resection of the distal femur with a rotating hinge device at the knee. The patient was placed on two cycles of preoperative adjuvant chemotherapy following which the fracture site and fixation plates were widely resected, leaving the knee joint intact. The segmental defect was reconstructed with an intercalary titanium device with fixation to the upper and lower bone sites with a compression device that allows for excellent fixation without stress shielding. This device is the ComPreSs system manufactured by Biomet, Inc. (Warsaw, IN).
AP radiograph taken 10 months following the surgical procedure shows excellent osteointegration of the bony components to the prosthetic device. The patient was able to ambulate with a normal gait without pain. Unfortunatey, because of the contamination at the time of the fracture and the surgical fixation, the tumor recurred locally and an amputation was required at a high level.
A close-up radiograph of the proximal juncture between the prosthesis and the upper femur with excellent osseointegration and hypertrophy of the bone at the juncture site with the titanium plasma spray interface. The ComPreSs device uses 600 pounds of pressure for stabilization purposes.
Photograph of the amputated specimen shows the intercalary device in position at the lower mid-portion of the femur. There is excellent osteointegration at both ends of the titanium implant.
Close-up photograph of the upper juncture site where bone can be seen growing into the stabilizing flutes in the titanium shoulder with titanium plasma spray on its surface. This patient was fitted with a prosthesis but one year following this amputation, she developed pulmonary metastases and eventually succumbed to her disease.
AP radiograph of the distal femur of a 32-year-old male with classic intramedullary osteogenic sarcoma shows permeative lysis throughout the entire mid-third of the femoral diaphysis, an unusual location for osteosarcoma. There is minimal evidence of bone formation outside the cortex that is heavily infiltrated by the tumor mass taking on the appearance of a round cell sarcoma such as Ewing's sarcoma.
Coronal T2-weighted MRI shows a signal abnormality in the medullary canal as well as a rather large extra-cortical mass surrounding the entire distal diaphysis of the femur with mixed high and low signal, The lower signal areas are the areas where osteoid formation is most prominent. The very bright signal areas suggest either hematoma or cystic degeneration within the tumor tissue.
Axial T2-weighted image shows the bright signal characteristics of the high grade osteosarcoma with small signal void areas where bone is being formed and a cystic area with a fluid level secondary to either necrosis within the tumor or hemorrhage into the tumor mass.
The patient was placed on systemic adjuvant chemotherapy. After six weeks he sustained a pathological fracture through the tumor site for which he was placed in a fiberglass cast. This lateral radiograph shows the displaced fracture in position in a fiberglass cast. The patient was continued on adjuvant chemotherapy for another two cycles over the next two months at which time the distal femur was resected and reconstructed with a metallic prosthesis.
Coronal MRI shows the fracture site through the tumor with a large soft tissue mass on the medial side of the distal femoral diaphysis.
Coronal gadolinium contrast MRI taken after three or four cycles of adjuvant chemotherapy shows extensive necrosis of the tumor tissue with rim enhancement seen at the outer edge of the tumor mass where there is viable hyperemic response at the periphery of the necrotizing tumor.
The patient was taken to the operating room three months following his pathological fracture and the distal 10 inches of the femur was widely resected. This photograph of the resected specimen shows a safe cuff of normal muscle tissue surrounding the entire tumor mass, including the fracture callous formation.
The specimen was inked for margins and cut with a band saw. This photograph shows the cut specimen with green ink staining the surface, demonstrating the pathological fracture site partially healed with a fairly large tumor mass extruding through the fracture site into the soft tissues on the medial side of the femoral diaphysis. There is normal callous formation containing the tumor formation internally.
Gross macrosection of the pathological specimen shows the pathological fracture with the tumor breaking out into the surrounding soft tissue.
Low power photomicrograph of the original biopsy specimen shows neoplastic osteoid being produced by malignant osteoblasts prior to the use of adjuvant chemotherapy.
Lateral radiograph of the reconstructed distal femur. The patient continued on adjuvant chemotherapy postoperatively. Nine months later he developed pulmonary metastases and died of his disease.
AP radiograph of the knee of a 13-year-old boy who presented with a tumor mass in his distal thigh area that had been present for four months. This film reveals a fairly dense, sclerotic, bone-forming tumor in the distal femoral metaphysis that is bulging through the lateral cortex. It proved to be an osteoblastic, classic osteogenic sarcoma.
Lateral radiograph shows a dense, sclerosing, bone-forming tumor mass in the distal posterior femoral metaphysis with breakout into the popliteal space where the tumor is forming heavily calcified or ossifying tissue that could suggest the diagnosis of a parosteal osteosarcoma or even an osteochondroma.
Technetium bone scan of the lower extremities shows heavy pickup of the bone isotope in the distal femoral metaphysis.
CT scan through the distal femur shows the heavily ossified tumor within the distal femoral metaphysis posteriorly, breaking out through the posterior cortex and producing a bony mass in the popliteal space that could suggest the diagnosis of a parosteal osteosarcoma. It is not the typical picture for an osteochondroma.
Axial T1-weighted MRI shows the very dark signal of the heavily ossifying tumor mass that fills about one-third of the distal femoral metaphysis with an extensive breakout into the popliteal space. There is also low signal involvement of the remaining entire distal femoral metaphyseal area produced by tumor infiltration that is definitely not the picture of a parosteal osteosarcoma or an osteochondroma.
Coronal T1-weighted MRI of the distal femur shows the very dark, low signal, heavily ossified tumor mass in the metaphysis of the femur. Surrounding it is a grayish, intermediate signal where the less heavily ossified tumor is permeating the entire distal femoral metaphysis, suggesting a high-grade osteosarcoma.
Sagittal T1-weighted MRI shows the very low signal, heavily ossified tumor mass posteriorly in the femoral metaphysis bulging into the popliteal space and butting directly against the popliteal artery. There is an intermediate low signal in the anterior part of the distal femoral metaphysis extending three inches from the distal growth plate that represents infiltrative high-grade osteosarcoma. This patient was treated by a combination of adjuvant chemotherapy and wide resection of the distal 6 inches of the femur. He was reconstructed with a custom rotating hinge prosthetic device.
This 16 year old male patient presented with a three month history of knee pain for which this x-ray revealed a bone forming lesion in the distal femoral metaphysis with an exophytic extention protruding in a posterior and lateral direction.
These coronal MRI images reveal a large signal abnormality filling the entire distal meta-diaphyseal area of the femur with a cortical breakthru in the medial cortex which on biopsy revealed the diagnosis of a classic osteogenic sarcoma.
The same is seen in these sagittal images.
More is seen in the axial images with the exophytic portion of the tumor seen well in the gad contrast image.
The patient was placed on several cycles of pre-op chemotherapy following which the distal end of the femur was resected widely with the surgical specimen seen to your left. The reconstruction was carried out with a rotating hinge Compress system as seen in the middle photo. The post op x-ray is seen to your right.
This 8 year old girl presented with a 3 month history of a painful mass in the distal thigh. This initial x-ray shows a large sun burst pattern of neoplastic bone forming circumferentially about the distal two thirds of the femur which on open biopsy revealed a classic osteoblastic osteogenic sarcoma.
On this initial MRI study we see the typical low signal of neoplastic bone formation both within and outside the entire distal femur in this coronal T-1 sequence.
On both the coronal T-2 and coronal Gad contrast we see the very large bright signal mass surrounding the entire distal femur and medullary involvement down to the distal growth plate. The gad contrast shows minimal tumor necrosis.
On this axial T-2 and Gad contrast we again see the large bright signal soft tissue mass surrounding the entire distal femur and in direct contact with the femoral vessels. This patient also had pulmonary metastases on a CT chest scan. She received three cycles of chemotherapy during which time the tumor did not reduce in in size and a repeat MRI study following the three cycles showed no change compared to the one seen here. All this gives this girl a very poor chance of survival. Because of the poor chemo response, a limb salvage procedure would most likely fail with local recurrence and thus a palliative hip disarticulation was suggested.
AP radiograph of the upper tibia of an 11-year-old boy shows an osteoblastic lesion that stops abruptly at the upper growth plate replacing the upper tibial metaphysis . A biopsy revealed the diagnosis of osteoblastic classic osteogenic sarcoma.
Lateral radiograph of the upper tibia shows the dense blastic osteoid-forming tumor with permeation into surrounding cortical structures. A breakout into the popliteal space can be seen on the posterior side.
Coronal T1-weighted MRI shows the low signal abnormality filling the entire upper tibial metaphysis, stopping abruptly at the upper growth plate, with permeation into the surrounding cortex and breakout slightly into the surrounding soft tissue.
Coronal T2-weighted image shows the low signal abnormality filling the entire upper third of the tibia because of excessive bone formation. There is a cuff of high signal tumor abnormality surrounding the entire upper metaphysis where the tumor has broken out under the periosteal sleeve.
Axial T2-weighted MRI shows the low signal, heavily calcified osteoblastic osteosarcoma in the medullary canal of the tibia surrounded by a cuff of high signal, less heavily calcified anaplastic tissue outside the cortex of the tibia. Because of excessive involvement of soft tissue in the deep compartment posteriorly including the neurovascular bundles, it was elected to treat this patient with an above-knee amputation, along with systemic adjuvant chemotherapy.
Low power photomicrograph of the surgical biopsy of the tibia shows the neoplastic osteoid in a field of aggressive-appearing osteoblastic cells that have hyperchromatic and pleomorphic nuclei and are forming the tumor osteoid. This picture clearly suggests the diagnosis of osteoblastic osteogenic sarcoma.
AP radiograph of the proximal tibia in a 15-year-old boy shows a dense blastic bone-forming tumor arising from the proximal tibial metaphysis and breaking out laterally into the anterior compartment, suggesting the diagnosis of an osteogenic sarcoma.
Lateral radiograph of the upper tibia shows the dense sclerosing osteoblastic osteosarcoma of the proximal tibial metaphysis. There is little evidence of cortical breakthrough on this projection.
Axial T1-weighted MRI shows the low signal abnormality in the upper tibial metaphysis laterally, secondary to a sclerosing osteoblastic osteosarcoma. There is minimal breakout into the soft tissue laterally.
This patient was treated by a combination of adjuvant chemotherapy and an above-knee amputation. This gross macrosection made from the amputated specimen shows the geographic appearance of the osteosarcoma filling the proximal epiphyseal-metaphyseal portion of the tibia laterally where the tumor butts directly against the articular surface of the lateral plateau. There is evidence of cortical breakthrough under the periosteum laterally.
Low power photomicrograph from the biopsy specimen shows dense osteoblastic osteoid formation being produced by a malignant osteoblastic tumor cell. Approximately 50% of osteosarcomas are the osteoblastic type.
This 17 year old female dancer presented with pain symptoms in the proximal tibia made worse with dancing with this x-ray that was felt to be a benign dysplastic lesion second to monostotic fibrous dysplasia.
3 months later this CT scan shows a dense blastic lesion in the proximal tibia which again suggested a benign dysplastic process but her pain persisted.
A month later this bone scan reveals considerable pickup in the area of concern that could be related to stress reaction around the dysplastic lesion or a more aggressive lesion such as OGS.
A bit earlier this T-1 MRI shows a low signal lesion in the proximal tibia that is well marginated and inside the cortical boundaries suggesting a benign lesion.
The axial T-2 and Gad contrast also suggests benign disease confined by cortical anatomy but with some slight periosteal response that could be stress reaction.
5 months later we now see obvious x-ray evidence of neoplastic bone breaking out into the anterior compartment with a soft tissue mass that strongly suggests the diagnosis of OGS which was proven to be the case with an open biopsy.
The bone scan is now typical for OGS.
Likewise the T-1 MRI shows tumor outside the cortical confines of the tibia unlike monostic fibrous dysplasia.
2 months after preop chemotherapy we still see high signal tumor activity on this T-2 MRI breaking out into the anterior compartment.
This patient was treated surgically with a wide proximal tibial resection and reconstructed with a Compress rotating hinge total knee replacement.
Lateral radiograph of the distal tibia in a 14-year-old girl shows a bone-forming sarcoma arising from the distal tibia and breaking out posteriorly into the soft tissue. The chaotic bone pattern strongly suggests an osteosarcoma.
AP radiograph shows the dense, sclerotic, ossifying pattern of an osteosarcoma of the distal tibia with breakout both laterally and medially.
The patient was taken to the operating room and a below-knee amputation was performed. This gross macrosection shows osteosarcoma filling the entire distal diaphyseal-metaphyseal portion of the tibia and stopping abruptly at the distal growth plate. There is evidence of extensive soft tissue breakout circumferentially around the tibia and a classic Codman's reactive triangle.
On this low power photomicrograph taken from the biopsy specimen there is combined chondroid and osteoid matrix formation being produced by malignant stromal cells. Classic osteosarcoma frequently has a mixture of both chondroid and osteoid matrix material.
This 14 year female had this x-ray taken because of an injury and as an incidental finding this typical asymptomatic non-ossifying fibroma was noted as an incidental finding.
4 years later this followup x-ray reveals no change and is still asymptomatic.
14 years after the first x-ray the lesion began to enlarge and became symptomatic.
The bone scan now shows dramatic increase in activity suggesting the diagnosis of OGS.
Likewise the T-2 weighted MRI shows high signal activity breaking out thru the anterior cortex into the soft tissue suggesting malignant disease.
The axial T-2 MRI also shows the high signal activity outside the tibial cortex typical of OGS.
This biopsy photomicrogragh shows the classic findings for a high grade OGS following which the patient was treated with chemotherapy and a wide resection of the proximal tibia and reconstructed with a Compress rotating total knee replacement.
AP radiograph of the leg of a 14-year-old girl shows an osteoblastic tumor arising centrally from the midportion of the fibula with circumferential malignant-appearing osteoid formation and heavy calcification. On biopsy, this tumor was proven to be osteosarcoma.
Oblique radiograph shows in better detail the ossification pattern of this chaotic bone formation arising centrally from the midportion of the fibula. Because of extensive involvement of the neurovascular structures, this patient was treated by an above-knee amputation as well as adjuvant chemotherapy. She was alive and well ten years following this procedure.
Oblique radiograph of the leg of an 8-year-old boy shows an aggressive, permeative lytic process involving the upper third of the fibula, with extensive soft tissue involvement circumferentially and a classic Codman's reactive triangle distally. Histologically, this lesion was proven to be a high-grade osteosarcoma for which an above-knee amputation was carried out. The patient was also treated with adjuvant chemotherapy.
AP radiograph of the left shoulder of a 17-year-old boy who had symptoms of pain for four months shows a densely osteoblastic bone tumor arising from the proximal half of the humerus. There is circumferential breakout and aggressive neoplastic bone formation on the medial side that strongly suggests the diagnosis of a classic osteogenic sarcoma.
Coronal T1-weighted MRI shows the low signal abnormality of the osteosarcoma filling the entire upper third of the proximal humerus, breaking out into the surrounding soft tissue circumferentially but stopping at the growth plate at the upper epiphysis.
This axial T2-weighted MRI shows the low signal portion of the heavily ossified sarcoma with a few peripheral high signal areas where the most anaplastic portion of the tumor would be found. There is extensive involvement of all the soft tissues around the upper humeral diaphysis.
The patient was placed on two cycles of adjuvant chemotherapy, following which the upper 8 inches of the humerus was resected. The gross specimen has a cuff of normal muscle surrounding the entire tumor site and the humeral head is seen clearly at the top edge of the photograph.
The tumor was sectioned in the pathology lab. The sectioned specimen shows dense, white, heavily calcified tumor osteoid formation with a chaotic pattern that is typical of a high-grade osteogenic sarcoma.
Low power photomicrograph shows a high-grade osteogenic sarcoma producing a large amount of heavily calcified osteoid. The tumor cells are hyperchromatic and pleomorphic, quite diagnostic of an osteoblastic osteogenic sarcoma.
Surgical photograph following placement of combined allograft and cemented Neer prosthesis, ready for relocation into the glenoid socket above and closure of the wound.
Postoperative radiograph showing a long (10 inch) Neer prosthesis fixed with cement. There is mild subluxation of the glenohumeral joint that is commonly seen immediately following this type of surgery.
AP radiograph of the proximal humerus in a 12-year-old girl shows a destructive, permeative lytic lesion of the upper 7 inches of the humerus with extensive cortical invasion medially. The lesion was biopsied and proven to be an osteogenic sarcoma. Other considerations in the differential diagnosis would include Ewing's sarcoma.
The patient was placed on adjuvant chemotherapy for two cycles, following which the tumor was widely resected. The surgical specimen has been cut in the pathology lab showing the characteristic yellowish-tan coloration of the high-grade sarcoma arising from the proximal humerus.
Postoperative radiograph taken five years after the surgical procedure shows the custom proximal humeral prosthesis in place with a cemented stem in the distal humerus. At that time, there was no evidence of local recurrence or pulmonary metastases and the patient had excellent function of the glenohumeral joint.
AP radiograph of the upper humerus in a 16-year-old boy who had symptoms of pain for the past four months shows a motheaten tumor arising from the head, neck and upper diaphysis of the proximal humerus with a large soft tissue tumor mass medially showing evidence of ossification. Histologically, this was proven to be a high-grade osteoblastic osteogenic sarcoma of the classic type.
The patient was placed on adjuvant chemotherapy for two months, following which the proximal 7 inches of the humerus was resected. The gross specimen shows a large extracortical tumor mass on the medial side of the head, neck and upper shaft of the humerus.
The surgical specimen cut open in the pathology lab shows extensive invasion of the tumor throughout the entire head, neck and upper shaft of the humerus. There is a large soft tissue breakout medially into the axillary space of the shoulder.
Low power photomicrograph taken from the original biopsy specimen shows a fairly dense, heavily calcified osteoblastic osteosarcoma with malignant osteoblasts producing the osteoid tissue, proving the diagnosis of an osteoblastic classic osteogenic sarcoma.
The appearance of the wound following resection of the upper end of the humerus, looking directly at the glenoid fossa above and the remaining soft tissues surrounding the area of the upper humerus.
Surgical photograph showing the custom long-stem Neer prosthesis cemented into position in the distal humerus and articulated with the glenoid fossa, ready for a reattachment of the rotator cuff tendon.
Immediate postoperative radiograph showing excellent position of the cemented long-stem Neer prosthesis in its normal anatomical relationship with the glenoid fossa.
This X-ray of an 18 year male shows a large extra-cortical ossifying tumor mass that strongly suggests the diagnosis of a classic OGS following which a biopsy proved this to be the case.
Following two cycles of pre-op chemotherapy a wide resection of the proximal humerus was carried out resulting in this photograph of the surgical specimen.
Here we see the surgical defect ready for reconstruction.
A cemented Neer prosthesis was used for a reconstructive procedure as seen in this surgical photograph
Here we see the post op x-ray 9 months later showing proximal migration of the implant because of soft tissue instability resulting from a lack of rotator cuff reconstruction which is currently handled with an alloprosthetic reconstruction. Notice the pulmonary mets which suggests a poor prognosis for survival.
This 14 year old male presented with radiographic evidence of very large OGS involving almost the entire humerus with extensive soft tissue involvement.
Here we see a close up one month following chemotherapy with minimal shrinkage of the tumor mass.
This MRI shows extensive soft tissue involvement that did not respond well to two cycles of pre-op chemotherapy making limb salvage surgery a poor choice.
This coronal T-2 MRI shows extensive soft tissue involvement outside the entire shaft of the humerus making it very difficult to obtain a safe wide surgical resection.
This axial PD MRI also shows a large soft tissue mass outside the humeral shaft.
This patient was treated with a shoulder disarticulation in order to obtain a safe surgical margin
This is the photomicrogragh obtained from the biopsy specimen showing features of a classic OGS.
This 15 year male presented with a pathologic fracture thru the surgical neck of the humerus which on biopsy proved to be a classic OGS.
Another x-ray shows the fracture thru an area of permeative lytic changes strongly suggesting a malignant diagnosis. This patient was treated with preoperative chemotherapy following which a wide resection of the proximal humerus was carried out along with an alloprosthetic reconstruction.
CT scan of the chest and abdomen in a 27-year-old female shows a bone-forming sarcoma arising from the 10th rib. There is tumor bone formation in the central portion of the mass that is bulging into the retroperitoneal space at the renal level. The mass was widely resected and the patient placed on adjuvant chemotherapy. She developed osteosarcoma of the pelvis two years later.
Two years after the rib resection the patient developed pain in the hip area. This AP radiograph shows permeative lytic destruction of the supra-acetabular area on the right side, along with a poorly defined blastic lesion in the body of the ilium at the notch level that on biopsy proved to be a metachronous osteosarcoma.
CT scan of the pelvis in the sacroiliac area shows a large bone-forming tumor mass arising from the medial aspect of the body of the ilium and bulging retroperitoneally against the body of S2.
CT scan cut at the sciatic notch level shows the ossifying sarcoma arising from the ilium with extra-cortical breakthrough, both laterally and medially, with a large mass in the retroperitoneal space.
Technetium bone scan shows increased pickup in the area of the osteosarcoma involving the upper two-thirds of the ilium.
The patient was placed on adjuvant chemotherapy for two months, following which an internal hemipelvectomy was performed. Here is the resected hemipelvis on the right side with the acetabulum to the right and the sciatic notch at the lower edge, above which is a large tumor mass covered by a normal muscle envelope.
The tumor was taken to the back table and debulked for surgical pathology. The remaining bone was placed in the autoclave for 3 minutes at 130° centigrade to kill the tumor but preserve the bone, creating a custom allograft. This is the autograft ready for reimplantion into the pelvis.
Here is the autoclaved autograft placed back into the patient and fixed into position with various screws and pins, along with a cemented conventional total hip replacement on top of the autoclaved acetabulum.
Radiograph obtained shortly after the surgical procedure shows excellent reconstruction of the right hemipelvis. The patient was able to walk with only a slight limp.
The radiographic appearance two years after the internal hemipelvectomy. There is excellent preservation of function and anatomy of the hemipelvis except for a minor, non-disabling stress fracture through the autoclaved bone just below the sciatic notch that did not require surgical correction. This patient lived approximately five years and then succumbed to pulmonary metastases without local recurrence of the tumor in the pelvis.
Coronal T2-weighted MRI of the pelvis in an 18-year-old boy with osteosarcoma of the left ilium shows a large, high signal neoplastic activity filling the entire left ilium down to the femoral head. There is extracortical involvement on both sides of the iliac bone. Histologically, this proved to be a high-grade osteosarcoma.
Axial T2-weighted MRI at the sacroiliac level shows high signal tumor activity filling the entire ilium up to the sacroiliac joint and involving soft tissue on both sides of the body of the ilium. The tumor responded quite dramatically to adjuvant chemotherapy.
The patient was taken to the operating room and the entire left hemipelvis was resected, because of tumor activity from the pubic symphysis to the SI joint.
The patient was reconstructed with an internal hemipelvectomy utilizing a standard total hip device with multiple pins and screws for fixation, CD rods were placed on both sides of the lumbar spine from the L2 level down, with one of the rods extending into the hip area and down to the tip of the ischium.
The appearance of the pelvic reconstruction with methyl methacrylate used to reconstruct the entire hemipelvis, including the acetabular area. There is a conventional total hip in position.
Postoperative radiograph showing the CD rods above with the extension of one rod down to what used to be the ischium with two Steinman pins crossing the symphysis into the opposite pubic ramus. The acetabulum has been reconstructed in its normal anatomical position with bone cement so that the patient was able to walk. The patient had a significant limp because of weak abductors and required a cane.
Postoperative oblique radiograph shows in better detail the position of the CD rod on the left side that extends from L3 down to the ischium.
The radiographic appearance 2-1/2 years later. The patient walked quite well with a cane, without pain, and without tumor recurrence. However, a year later he developed pulmonary metastases and local recurrence and died of his disease.
This AP radiograph of the pelvis of a 14-year-old boy who developed osteosarcoma of the left hemipelvis shows a blastic lesion filling the entire upper two-thirds of the innominate bone but sparing the hip joint.
CT scan at the S2 level shows the entire ilium filled with an osteoblastic osteosarcoma with a lacey breakout into the soft tissue both laterally and medially.
Axial T2-weighted MRI shows the high signal neoplastic activity filling the entire ilium up to the S-I joint with a large tumor mass located both extra- and intrapelvically.
Coronal T2-weighted MRI shows the osteosarcoma filling the upper half of the ilium but sparing the supra-acetabular area and hip joint so that a reconstruction can be carried out without sacrificing the hip.
After two months of adjuvant chemotherapy, the boy was taken to the operating room and the proximal half of the innominate bone was resected up to the S-I joint, sparing the supra-acetabular area. The defect was reconstructed with multiple pins, screws and plates with methyl methacrylate, resulting in excellent function so the patient could walk without a limp and without pain.
Ten years later there is no local recurrence and no signs of loosening in the sacroiliac area. However, there has been a progressive inflammatory arthritis of the hip joint associated with pain and shortening for which we are planning a total hip replacement with a cemented acetabular component that will bond with the existing stable cement and screws from the previous reconstruction.
Here are the surgical photos from that procedure with the removed femoral head seen above with no cartilage left behind. The upper right shows the acetabulum ready for a cemented cup with three screws set in position to reduce chances of cup loosening. Below we see the cup cemented in to the left and the completed THA with a ceramic head on poly to the right.
Here we see the post op x-ray following the THA with all components in good position and the patient is now pain free.
This AP radiograph of the pelvis of a 38-year-old male shows a small fibrous cortical defect located in the left supra-acetabular area that was picked up as an incidental finding when the patient had an x-ray taken for back pain.
This radiograph of the pelvis was taken twelve years later when he developed symptoms of pain in his left hip. It shows an osteolytic neoplasm filling the supra-acetabular area, along with central matrix calcification that on biopsy proved to be an osteogenic sarcoma.
The patient was placed on adjuvant chemotherapy for two months, following which an internal hemipelvectomy was performed. Here is a photograph of the entire hip joint with the sciatic notch to the left and the tumor mass laying in the middle of the ilium covered by normal muscle tissue.
The specimen was taken to the back table and debulked of the major tumor mass which was sent to pathology. The remaining bone was placed in the autoclave for 5 minutes at 130° centigrade and placed back into the patient's pelvis for reconstructive purposes. Pins were placed through the autoclaved autograft along with screws and plates. A routine total hip prosthesis was cemented into position on top of the threaded Steinmann pins.
The surgical appearance after completion of the reconstruction with the entire acetabulum recemented in anatomical position that allows for excellent weight bearing function.
Radiographic appearance two years after the reconstruction. The patient walked with a slight limp but did not use a cane or crutch and there was no recurrence of tumor.
However, a year later his tumor did recur locally. The entire reconstructed area was resected, leaving the patient with a pseudarthrosis and severe instability, necessitating the use of a crutch. Shortly after that he developed pulmonary metastases and died.
A 23-year-old male presented with a history of low back pain for four months. This AP radiograph of the lumbosacral spine shows dense osteoblastic, neoplastic activity in the area of L5-S1 that was biopsied and proved to be an osteoblastic osteosarcoma.
Lateral radiograph of the lumbosacral spine shows the osteosarcoma filling the entire body of L5 and extending posteriorly into the vertebral canal and down into S1 posteriorly.
CT scan at the L5 level shows a dense osteoblastic tumor arising from the posterolateral elements of L5 and extending into the vertebral canal a distance of 1 cm.
Low power photomicrograph shows the microscopic appearance of this osteoblastic osteosarcoma with tumor osteoid being produced by malignant-appearing osteoblasts. The absence of giant cell activity tends to rule out the diagnosis of an aggressive osteoblastoma which can have a similar clinical appearance. This patient was treated with adjuvant chemotherapy, along with a wide resection of the tumor but went on to develop local recurrence and pulmonary metastases and died two years later.
Lateral radiograph of the lumbar spine of a 21-year-old male shows a permeative lytic process involving the entire L3 vertebra at the mid-lumbar level, extending into the pedicle posteriorly. A biopsy proved the lesion to be an osteogenic sarcoma.
Technetium bone scan showing increased activity of the osteosarcoma in the mid-lumbar spine area.
CT scan through the involved lumbar vertebra with extensive motheaten lytic destruction of 80% of the vertebral body extending into the posterolateral elements on one side only. The tumor obviously extends into the vertebral canal causing mild compression of the cauda equina.
Sagittal T2-weighted MRI shows a signal abnormality in the posterior two-thirds of the body of L3 with bulging into the floor of the vertebral canal causing mild paraparesis and extension into the posterolateral elements at the same level behind the vertebral canal.
Low power photomicrograph of the biopsy specimen shows an osteoblastic sarcoma with malignant osteoblasts producing the tumor osteoid.
This patient was treated with adjuvant chemotherapy and a wide resection of the entire L3 vertebra which was reconstructed with an allograft placed anteriorly as a strut graft. Fixation was carried out with pedicle screws and two posterior laminar plates.
Sagittal T1-weighted MRI of a 45-year-old female shows a low signal abnormality filling the posterior half of the body of L4 and extending through the pedicle on that side into the posterolateral elements. On biopsy, it was proven to be an ostegenic sarcoma.
Axial T2-weighted MRI at the L4 level shows the tumor arising from the posterolateral corner of the vertebral body and extending back through the pedicle and out into the soft tissues beneath the paraspinous muscle on the involved side.
CT scan through the L4 vertebra with motheaten lytic destruction of the posterolateral corner of the vertebra extending slightly into the vertebral canal and bulging out into the soft tissue beneath the paraspinous muscle laterally. This patient was treated by a combination of adjuvant chemotherapy and local resection of the tumor mass, however, the tumor recurred and the patient died two years later.
AP radiograph of the foot of a 20-year-old male shows an osteosarcoma arising from the first metatarsal bone at its midshaft, It demonstrates tumor osteoid formation circumferentially around the cortex of the mid-diaphysis of the metatarsal.
Lateral radiograph of the osteosarcoma arising from the first metatarsal with bone formation seen on the dorsal aspect of the mid-diaphysis.
Low power photomicrograph demonstrating the diagnostic features of a classic osteogenic sarcoma with neoplastic osteoid and malignant osteoblasts. This patient was treated with a below-knee amputation and adjuvant chemotherapy. He was alive and well five years later.
AP radiograph of the foot of a 76-year-old female shows a lytic destructive lesion of the first metatarsal with an ossifying soft tissue mass extending dorsally at the mid-foot area that was histologically proven to be an osteogenic sarcoma.
Lateral radiograph of the foot showing the ossifying mass arising from the dorsal aspect of the proximal base of the first metatarsal. The patient underwent a below-knee amputation and, because of her advanced age, no chemotherapy was used.
This AP radiograph of the great toe in a 17-year-old male shows a fairly geographic lytic process of the distal phalanx that was initially felt to be an enchondroma.
Radiograph taken a year-and-a-half later shows progressive enlargement and destruction of the surrounding bony structure. At this time, the lesion was biopsied and proved to be an osteogenic sarcoma.
Technetium bone scan shows increased activity in the area of the tumor in the distal phalanx of the great toe.
This patient was placed on adjuvant chemotherapy following which the distal phalanx was resected back to the IP articular surface and the area reconstructed with cancellous allograft. This radiograph shows the appearance shortly after that procedure. The patient did not develop local recurrence and was doing well many years later.
An 18-year-old female presented with a painful lump at the base of her fourth metacarpal that had been present for three months. This AP radiopgraph of the hand shows a densely calcific lesion involving the proximal 20% of the fourth metacarpal. There is a periosteal Codman's reactive triangle located distally that might suggest the diagnosis of an osteoblastoma, however on biopsy this proved to be a classic osteosarcoma. The hand is a rare location for this tumor.
This coronal gadolinium contrast MRI prior to chemotherapy shows high signal activity around the periphery of the proximal end of the fourth metacarpal. There is a black signal void in the central area where the osteosarcoma is heavily ossified.
Axial gadolinium contrast MRI shows a dense signal void in a cut taken through the heavily ossified portion of the tumor.
Another axial gadolinium contrast MRI cut in an area where there is a higher signal around the central portion of the ossified tumor suggesting aggressive anaplastic tumor formation.
Radiographic appearance of the hand two years after chemotherapy and a surgical resection of the proximal half of the metacarpal and reconstruction with allograft and side plate fixation. There is excellent healing of the allograft and no recurrence of tumor. There was no evidence of metastatic disease and five years following the procedure, the patient remained free of disease.
A 70-year-old male presented with a history of a painful mass on the dorsal aspect of his forefoot that on radiograph showed evidence of an ossifying mass in the soft tissue dorsal to the distal metatarsal area. On biopsy, the lesion proved to be a soft tissue osteosarcoma with no connection to the subadjacent bony structures.
AP radiograph of the foot again shows an ossifying mass located dorsal to the fifth metatarsal with no evidence of subadjacent bony involvement.
Low power photomicrograph taken from a biopsy of the soft tissue osteosarcoma shows osteoid formation by a pleomorphic hyperchromatic osteoblastic stem cell that suggests the diagnosis of an osteogenic sarcoma. This is an unusual location for a soft tissue osteosarcoma in an older age group. Treatment consisted of a below-knee amputation. The patient was considered too old for chemotherapy. He survived his disease without metastatic involvement to the lung.
A 55-year-old male presented with a painful mass in the anterior portion of his ankle. This lateral radiograph shows a lytic lesion in the anterior part of the body of the talus, breaking out anteriorly into the ankle joint with soft tissue involvement extending a distance of 1-2 cm in front of the ankle joint. The lesion was biopsied and proven to be a classic osteogenic sarcoma. There was no pulmonary involvement.
This mortise view shows in better detail the lytic destructive changes in the body of the talus just beneath the ankle joint on the medial side. Because of the location of the tumor, the patient was treated with a below-knee amputation, No chemotherapy was used. At five years following the amputation, the patient was doing well with no pulmonary metastases.
Gross macrosection of the entire hindfoot of an amputation specimen of a osteosarcoma in a 19-year-old male shows extensive permeative tumor activity filling the entire body of the os calcis and breaking through into the subtalar joint and the inferior portion of the talar body. There is also soft tissue breakout behind the ankle joint.
A 40-year-old female gave a history of pain and swelling of the jaw for 4 months. This oblique view of the ramus shows a diffuse lytic process involving the body of the mandibular ramus, just beneath and including the roots of the molar teeth of the left side.
The patient was taken to the operating room and a hemimandibulectomy was carried out. This surgical specimen shows extensive tumor involvement of the entire ramus of the mandible with soft tissue breakout on the inferior edge and the tumor permeated through the cortical structure of the mandible. The patient was protected with adjuvant chemotherapy and, because of the better prognosis for survival with facial osteosarcoma, she was alive and well five years later.
Oblique radiograph of the mandible in a 75-year-old female shows an osteoblastic lesion at the angle of the mandible in an edentulous elderly female. On biopsy, this proved to be an osteosarcoma and the patient was treated by surgical hemimandibulectomy. Because of her age, no adjuvant chemotherapy was advised.
A 36-year-old male presented with symptoms of pain and a flank mass for the past four months. This AP radiograph of the ribs taken in December 1989 shows an osteoblastic aneurysmal lesion arising from the 11th rib posteriorly. At that time, the radiologist felt that this represented a benign fibrous dysplastic lesion and no treatment was carried out.
AP radiograph taken a year-and-a-half years later shows continued growth of the ossifying tumor mass which is not characteristic for benign fibrous dysplasia. A biopsy at this point revealed a high-grade osteogenic sarcoma.
This bone scan of the axial skeleton shows significant pickup of the technetium isotope in the area of the ossifying osteogenic sarcoma. This patient was treated by a wide surgical resection and adjuvant chemotherapy. He survived five years without local recurrence or metastases.
CT scan through the mid-lumbodorsal spine shows an ossifying mass arising from a rib on the left side. There is expansion into the abdominal cavity as well as bulging exteriorly. On physical examination a tender lump could be felt which on biopsy proved to be an osteogenic sarcoma.
Another CT cut through the same rib showing that most of the tumor is bulging into the abdominal cavity on the left side.
Low power photomicrograph of the biopsy specimen reveals a highly malignant osteogenic sarcoma. The lesion was widely resected and treated simultaneously with adjuvant chemotherapy.
A 29-year-old female presented with a fairly large, painful ossifying mass on right clavicle that on biopsy proved to be an osteogenic sarcoma. This AP radiograph of the shoulder shows a fairly large, ossifying mass located circumferentially around the mid-portion and proximal aspect of her right clavicle.
This laminargraphic radiograph shows in better detail the osteoblastic tumor mass arising from the right clavicle and bulging into the apex of the right lung. In addition, it fills the supraclavicular space where the tumor was obvious on physical examination. This patient was taken to the operating room and a wide resection of the entire right clavicle was performed under the protection of adjuvant chemotherapy. At the time of surgery, the tumor was found to invade directly into the superior vena cava.
Postoperative radiograph following a wide resection of the entire right clavicle. The patient was alive and well five years later without local recurrence or pulmonary metastases.
Lateral radiograph of the knee of a 21-year-old male who had pain in the knee for several months shows a motheaten lytic destructive process involving the entire patella. On biopsy, this lesion proved to be an osteogenic sarcoma.
A sunset view of the knee joint shows again the motheaten lysis of patella. This patient underwent a total patellectomy but did not require adjuvant chemotherapy. He never developed metastatic disease, This is a very unusual location for osteosarcoma.
AP radiograph of the forearm of a 19-year-old female shows a permeative destructive lesion of the distal ulna with a Codman's reactive triangle suggesting a high-grade sarcoma. On biopsy, this lesion proved to be an osteogenic sarcoma and the patient was treated by wide resection of the distal ulna and adjuvant chemotherapy. She never developed pulmonary metastases.
Tangential view of the scapula in a 38-year-old male shows an ossifying tumor mass arising from the inferior half of the scapular blade that on biopsy proved to be an osteogenic sarcoma. It is an unusual location of an osteosarcoma. The patient was treated by a wide resection and adjuvant chemotherapy and was without local recurrence five years later.
A 14-year-old female track star presented with local tenderness in the proximal tibia after working out for cross country running for three weeks. This lateral radiograph shows periosteal new bone formation off the posterior cortex with a sclerotic band extending anteriorly that could suggest the diagnosis of a bone-forming sarcoma or, as in this case, a stress fracture related to her sport activity.
A bone scan shows increased uptake in the area of the stress fracture located in the proximal tibial diaphysis.
Because of concern about the possibility of a bone tumor, an MRI was ordered. This coronal T1-weighted image shows a low signal abnormality in the medullary canal of the proximal tibial diaphysis that could be mistaken for a bone tumor.
This axial T2-weighted MRI shows a high signal abnormality involving the medullary canal as well as a cuff of high signal activity around the tibial diaphysis that could suggest a malignant tumor or, in this case, active physiological callous formation from a stress fracture.
This low power photomicrograph obtained from a biopsy of the stress fracture shows reactive bone and cartilage formation, quite typical for callous formation in a healing stress fracture.
Radiograph of the upper extremity in a 6-month-old girl who presented with a painful mass in the forearm shows an osteoblastic periosteal lesion surrounding the entire shaft of the ulna. There is some slight periosteal changes in the adjacent radius. The differential diagnosis here would include congenital lues, osteomyelitis, hypervitaminosis A, osteosarcoma and Caffey's disease. In this case, the diagnosis is Caffey's disease.
Low power photomicrograph taken from a biopsy specimen of the ulna shows woven bone formation by an aggressive osteoblastic proliferation that was misinterpreted as an osteogenic sarcoma for which an amputation was performed.
Gross macrosection cut transversely through the amputated ulna shows reactive periosteal new bone formation surrounding the entire cortex of the ulna in a radial pattern that would suggest a benign reactive process rather than a malignant diagnosis of osteosarcoma that was made incorrectly in this case. There is also evidence of endosteal new bone formation and inflammatory osteomyelitis of the medullary contents compatible with the diagnosis of Caffey's disease.
Radiograph of the chest and shoulders of the same patient showing hypertrophic changes in both scapular blades as well as the clavicles bilaterally and the mandible above that are compatible with the diagnosis of Caffey's disease.
This radiograph of the mandible shows periosteal new bone formation throughout both sides of the mandible, very compatible with the diagnosis of Caffey's disease, a benign idiopathic hyperostotic condition seen in infants that usually resolves spontaneously without treatment. It is felt by some to have a viral etiology but can be misdiagnosed as a bone-forming sarcoma, as in this case.
A 25-year-old female gave a history of a painful mass in her distal femur for four months. AP and lateral radiographic views of the distal femur shows an aggressive osteolytic lesion arising from the distal metadiaphyseal portion of the femur with extensive soft tissue breakout medially that could easily suggest the diagnosis of a malignant osteogenic sarcoma. In this case, the patient was found to have a primary parathyroid adenoma (subsequently removed) that was producing large amounts of parathormone. The production of the parathormone caused secondary osteoclastic changes in the distal femur that could suggest the diagnosis of either giant cell tumor or a malignant primary osteogenic sarcoma.
Low power photomicrograph taken from a biopsy of the distal femoral lesion shows aggressive but benign giant cell activity with hemorrhage. There is extensive osteoclastic activity that could suggest the diagnosis of a giant cell tumor but is also compatible with the diagnosis of a brown tumor of hyperparathyroidism.
Low power photomicrograph taken from the periphery of the pseudotumor shows giant cell activity and fibroblastic response in the bone marrow along with osteoid formation demonstrated by the thick, poorly mineralized osteoid seam seen on the trabecular pattern. This picture should suggest to an experienced pathologist the diagnosis of osteomalacia such as we see in patients with early hyperparathyroidism
A 12-year-old boy with hemophilia presented with a painful mass in his distal thigh area, This AP radiograph of the distal femur shows extensive permeative lysis through the distal end of the femur with aggressive-appearing osteoid formation seen in the soft tissue around the distal metaphysis that might suggest the diagnosis of a hemorrhagic osteosarcoma. However, in this patient with a known history of hemophilia, the more likely diagnosis is a pseudotumor of hemophilia with hemorrhagic periostitis and a stress fracture through the distal femoral metaphysis. In addition, there is inflammatory hemorrhagic synovitis of the knee joint as demonstrated by the chondrolytic changes of the femoral condyles, widening of the intercondylar dimensions and diffuse osteopenia around the entire knee joint, quite characteristic for pseudotumor of hemophilia and not hemorrhagic osteosarcoma.
ION :Lateral radiograph of the same patient showing a stress fracture through distal femoral metaphysis. There is extensive soft tissue swelling posteriorly that could be misdiagnosed as an osteosarcoma. This case occurred before Factor VIII and cryoprecipitate treatment and, because of this, these patients were treated with amputation above the lesion. Fifty per cent of patients undergoing this treatment died from hemorrhagic shock frequently complicated by septicemia. Now with Factor VIII therapy this is no longer a problem.
AP radiograph of the leg of a 44-year-old male with a history of a crushing fracture of the tibia 25 years before shows extensive, mature heterotopic bone formation in the anterior compartment just anterior to the fibula that could be misdiagnosed as a bone-forming tumor. However, this x-ray shows evidence of an old healed compartment syndrome with extensive heterotopic bone formation in the muscle secondary to an old crushing injury that was never treated surgically.
A 64-year-old female came into the emergency room for a routine x-ray of the knee after an injury. This AP radiograph of the knee shows a dense blastic lesion located in the lateral aspect of the metaphyseal-epiphyseal juncture of the distal femur with no evidence of any periosteal reaction or lytic changes seen in the surrounding bone. A low-grade osteogenic sarcoma could be considered, however the real diagnosis is a large bone island that had been present for many years without symptoms.
Lateral radiograph again shows the dense blastic lesion located in the distal femur without evidence of any reactive periosteal new bone formation or lytic changes in the surrounding medullary canal, typical for a large bone island.
On this technetium bone scan there is mild uptake by the large bone island but with no overflow activity seen in the surrounding structures as would be seen with an osteogenic sarcoma.
An MRI was performed to rule out a possible malignant bone tumor. On this coronal T1-weighted image there is very well-defined black signal void in the area of the large bone island with no inflammatory changes seen in the surrounding bone marrow and no evidence of periosteal new bone formation on the surface as would be seen with a typical osteosarcoma. With this type of lesion, it is not necessary to perform a biopsy but the lesion should be followed with x-rays every six months for several years to make sure it remains stable.
AP radiograph of the femur of a 10-year-old girl with a known diagnosis of osteogenesis imperfecta shows a large, fluffy callous formation around a mid-shaft diaphyseal fracture that occurred six weeks ago but went without treatment. Without an attempt at external immobilization a large callous formed because of excessive motion at the fracture site but the clinicians who initially examined this child were concerned about a possible osteosarcoma.
This radiograph obtained 2-1/2 years later shows that the fracture has gone to a solid union and remodelled considerably, taking on the appearance of a healed fracture and not the appearance of a malignant osteosarcoma.
AP radiograph of the femur of a 17-year-old male who was a avid soccer player shows extensive fusiform periosteal thickening of the upper diaphysis of the femur that was secondary to repetitive stress periostitis from kicking a soccer ball. The clinicians who treated the young man were concerned that the clinical picture could represent a sclerosing osteogenic sarcoma.
Low power photomicrograph taken from a biopsy specimen to rule out the possibility of an osteosarcoma shows dense, mature woven bone formation that represents a long period of chronic periosteal new bone formation as a response to tugging of the quadriceps muscle against the periosteal membrane of the femur. This boy was treated by simple observation over a period of years to make sure this lesion remained stable which it did. It was obviously not a malignant tumor.
Transcript of "An Atlas of Musculoskeletal Oncology: Volume 2"
Classic Osteogenic Sarcoma Osteogenic sarcoma is the most common primary malignanttumor of bone, making up 20 % of all primary malignancies,with approximately 500-1000 new cases diagnosed each year inthe United States. The classic or most common form of osteo-sarcoma is seen typically in children and young adults, with amale preference. It occurs in the metaphyseal areas of fast growingbones with the most common location being the distal femur,second the proximal tibia, and third the proximal humerus.50% of the lesions will be found around the knee joint. This tumoris rare in in small bones such as the hand or the foot, or in vertebralsegments. Patients usually present with spontaneous symptomsof pain in the area, followed several month later with a tumormass that is usually diagnosed by biopsy within six months afteronset of symptoms. The radiographic appearance of the lesionis typically a permeative lytic lesion seen in the metaphyseal area
of a long bone with cortical breakthrough and periosteal elevationcreating a Codman’s reactive triangle, followed later by a sunburstpattern of chaotic bone formation in the soft tissue outside the peri-osteal sleeve. In a small percentage of cases, a so-called skip lesionwill appear as a separate nodule of tumor activity totally separatefrom the primary lesion which, when found, suggests a very poorprognosis for survival. Fifty percent of osteosarcomas are of theosteoblastic type, but in a smaller percentage of cases, there willbe a prominence of cartilage or fibrous tissue that does not seem toinfluence the prognosis for survival. The staging process for this disease includes a MRI study of theprimary tumor that helps identify soft tissue invasion by the tumorand defines the medullary extent of the tumor which helps theoperating surgeon determine the level of amputation or limbsalvage resection. A bone isotope scan is performed to rule out thepossibility of other bony foci in the skeletal system and a CT scanof the chest is obtained to rule out the possibility of metastatic
disease to the lung. The final staging process includes a biopsyof the primary site performed in such a way as to not contaminatevital structures that might interfere with the potential for a limbsalvage resection at a later date. Prior to 1970, the prognosis for survival with this disease wasonly 20% even though early amputation was performed at a highlevel. Pulmonary metastasis was the reason for a fatal outcome inthese early cases, however, with the advent of multi-drug chemo-therapy the prognosis for survival has now increased to approx-imately 60%. The drugs most commonly used for systemic controlof the disease include high dose methotrexate, adriamycin,cysplatin, and ifosfamide. These drugs are administered througha central venous line on a cyclic basis every three to four weeksfor approximately two months prior to a surgical removal of thetumor. Chemotherapy is then continued for approximately fourmonths after surgical treatment. At the present time, 90% of patients with osteosarcoma are
treated by limb salvage resection. The most common type ofreconstruction consists of a total joint replacement such as arotating hinge at the knee. A smaller group of patients are treatedwith allograft reconstruction or combinations of the above.Excisional arthrodesis was a popular technique many years agobut now patients prefer a reconstruction that involves normaljoint motion. The prognosis for survival is influenced by thedegree of tumor necrosis produced by the preoperative chemo-therapy protocol, so that at the time of surgical resection if thereis more than 90% necrosis of the tumor, the patient has a muchbetter prognosis for survival (approximately 85% at five years).Pulmonary metastasis is still the major concern following treat-ment for osteosarcoma and, if this does occur, aggressive surgicalresection of the lesions thru the chest wall is frequently performed.There is a 30% survival rate at five years following this procedure.As with other forms of cancer, recent molecular genetic studieshave revealed a high incidence of abnormality in the P-53suppressor genes found in this tumor.