Volume 6
Upcoming SlideShare
Loading in...5
×
 

Volume 6

on

  • 488 views

 

Statistics

Views

Total Views
488
Views on SlideShare
486
Embed Views
2

Actions

Likes
1
Downloads
27
Comments
0

1 Embed 2

http://orthosurg.ucsf.edu 2

Accessibility

Categories

Upload Details

Uploaded via as Adobe PDF

Usage Rights

© All Rights Reserved

Report content

Flagged as inappropriate Flag as inappropriate
Flag as inappropriate

Select your reason for flagging this presentation as inappropriate.

Cancel
  • Full Name Full Name Comment goes here.
    Are you sure you want to
    Your message goes here
    Processing…
Post Comment
Edit your comment

Volume 6 Volume 6 Presentation Transcript

  • Volume 6 Fibrous Tumors of BoneFibrous cortical defects------------Case 130-131 & 700-702Non-ossifying fibroma-------------Case 132-133 & 703-713Fibrous dysplasia Monostotic------------------------Case 134-135 & 714-748 Polyostotic------------------------Case 136 & 749-757 Mazabraud syndrome-----------Case 758-759 Albright’s disease---------------Case 137 & 760-764Desmoplastic fibroma-------------Case 138-139 & 765-771Fibrosarcoma-----------------------Case 140-141 & 772-783Malignant fibrous histiocytoma--Case 142-143 & 784-800Leiomyosarcoma-------------------Case 144-45
  • Fibrous Bone Tumors
  • Fibrous Cortical Defects View slide
  • Fibrous Cortical Defects The fibrous cortical defect is a very small metaphyseal lesion seenin long bones, typically around the knee joint in children. They areusually picked up on a routine radiograph as an incidental finding.They are considered dysplastic or hamartomatous lesions and arevery common, with about a 25% incidence in normal childrenaround the age of four to five years. As the child matures, thelesions will disappear by a process of skeletal remodeling and,in most cases, the diagnosis is obvious based on a simple radio-graphic study. Microscopically, the lesions are filled with benignfibroblastic cells forming collagen and usually demonstrate nomacrophage or inflammatory activity. View slide
  • CLASSICCase #13016 year femalefibrous corticaldefect distal femur
  • Photomic
  • Case #13118 year malefibrous cortical defectdistal femur
  • Lateral view
  • Case #70018 year malefibrous cortical defectdistal femur
  • Lateral view
  • Bone scan
  • Case #701 15 year male with fibrous cortical defect distal femur
  • Case #70242 year female with fibrous cortical defect femoral neck
  • Frog leg lateral
  • Bone scan
  • CT scan shows bilateral lesions
  • Non-ossifying Fibroma
  • Non-ossifying Fibroma The non-ossifying fibroma is considered to be a larger form of thefibrous cortical defect seen in the lower extremities of children.The lesions are usually eccentrically located but the larger ones canbecome more central. Radiographically, they have a soap-bubblyappearance with slight thinning and dilitation of the adjacentcortical structure. The diagnosis can usually be established by x-rayalone without biopsy. These lesions are a common cause for path-ologic fracture in children. They are usually asymptomatic untilthe day of injury. There can be multiple lesions in the same patient,similar to fibrous dysplasia, and can even be associated with café-au-laite skin lesions. Histologically, the lesion consists of benign fibrous tissuespeckled with giant cells, histiocytes, and occasional foam cellsand even cholesterol deposits. Treatment usually consists of curette-ment and bone grafting in high risk athletic males.
  • CLASSIC Case #13222 year female with non-ossifying fibroma proximal tibia
  • Close up lateral
  • Photomic showing giant cells
  • Post op x-ray afterbone grafting fibular strut
  • Case #13312 year malenon-ossifying fibromadistal tibia withpathologic fracture
  • Case #70316 year malenon-ossifying fibromadistal tibia
  • Coronal T-2 MRI
  • giant cellsfoam cells Photomic with foam cells and giant cells
  • Case #704 stress fracture 9 year female with non-ossifying proximal tibia
  • stress fracture Lateral view
  • Coronal GRE T-2* MRI showing stress fracture
  • Coronal GRE T-2 MRI showing stress fracture
  • Case #70511 year femalenon-ossifying fibromatibia with path fracture
  • Case #70613 year malenon-ossifying fibromadistal tibia
  • Case #707 18 year male with non-ossifying fibroma distal tibia
  • Case #7089 year malenon-ossifying fibromadistal fibula
  • Case #70912 year malenon-ossifying fibromadistal fibula
  • Case #710 xanthofibroma34 year femalexanthofibroma andABC prox fibula ABC
  • Lateral view
  • Case #711 17 year male with non-ossifying fibroma distal femur
  • Bone scan
  • Photomic
  • Case #7125 year malenon-ossifying fibromafemur with pathfracture
  • Case #71316 year femalenon-ossifying fibromafemur with path fracture
  • Fibrous Dysplasia
  • Fibrous Dysplasia Fibrous dysplasia of bone is a relatively common hamartomatousdevelopmental process in the skeletal system that can be eithermonostotic or polyostotic. In the case of polyostotic, there can alsobe endrocrine abnormalities such as precocious puberty in femaleswith the McCune-Albright syndrome. This condition occursprimarily in females and can be associated with café-au-laite spotson the skin which have a rough serrated edge compared to thesmooth edges in neurofibromatosis. The monostotic form has anappearance similar to non-ossifying fibroma except that the lyticcomponent has a typical smokey appearance caused by metaplasticbone formation in the fibrous tissue. In the more severe polyostoticform, representing only about 10% of fibrous dysplasia, the diseasetends to amplify on one side of the body more than the othercausing leg length discrepancy, especially when there is a character-istic chronic deformity of the proximal femur; referred to in the
  • literature as the shepherd’s crook deformity; that frequentlyrequires surgical correction. The most common locations for fibrous dysplasia are theproximal femur, followed by the tibia, humerus, ribs and,occasionally, caranio-facial anatomy. Other endrocrinopathiesassociated with polyostotic fibrous dysplasia include hyperpara-thyroidism, acromegaly, Cushing’s disease and, occasionally,hypophosphatemic osteomalacia. Occasionally, one will see softtissue myxomas with fibrous dysplasia which is referred to as theMazabraud’s syndrome. Radiographically, there will be diffusedysplastic changes throughout the involved bones with gradualdilation and thinning of the cortices, and a soap-bubbly appear-ance but with a smoky look compared to non-ossifing fibroma.Histologically, there is evidence of benign fibrous tissue matrixstudded with islands of metaplastic bone with a so-calledalphabet soup appearance but without osteoblastic rimming of the
  • trabeculae. Occasionally one will see macrophages, osteoclasts,foam cells and even cholesterol deposits similar to what is foundin non-ossifying fibroma. In approximately 1% of cases, there willbe a conversion to a secondary sarcoma in adult life that couldpresent as an osteosarcoma, or malignant fibrous histiocytoma oreven a chondrosarcoma. Treatment usually consists of corrective osteotomies for secondarydevelopmental deformities, along with bone grafting, includingfibular strut grafts and occasionally intramedullary devices to preventrepeated stress fractures. Recently there has been enthusiasm forthe use of biphosphonates to inhibit the osteoclastic activity ingrowing children that produces weakening of the bone. It is bestnot to perform bone grafting procedures in children because of thehigh recurrence rate in children under 16 years of age.
  • MonostoticFibrous Dysplasia
  • CLASSICCase #13445 year femalemonostoticfibrous dysplasiaproximal tibia
  • Bone scan
  • Coronal T-1 MRI
  • Sagittal T-2 MRI
  • Photomic showing alphabet soup metaplastic bone
  • metaplastic osteoclastsbone fibrous tissue Close up photomic
  • Case #13559 year femalemonostotic fibrousdysplasia humerus
  • Bone scan
  • Sagittal T-1 MRI
  • Case #135.124 year female with monostotic fibrous dysplasia humerus
  • Case #71430 year malemonostotic fibrousdysplasia femur
  • Lateral view
  • Coronal T-1 MRI
  • Photomic with metaplastic bone
  • Monostotic fibrous dysplasiaCase #714.1 Bone scan 46 year male with mild stress pain right hip
  • Cor T-1 STIR
  • Sag T-1 PD Gad
  • P.O. DHS and cancellousallograph
  • Case #71518 year femalemonostotic fibrousdysplasia prox femur
  • Bone scan
  • metaplastic bone Photomic
  • Case #71621 year femalemonostotic fibrousdysplasia prox femur
  • Case #71769 year femalemonostotic fibrousdysplasia femur
  • Lateral view
  • Bone scan
  • Coronal T-1 MRI
  • Coronal T-2 MRI
  • Photomic
  • Case #717.125 year malemonostotic fibrousdysplasia femur
  • Post op x-rayfollowing surgicalcorrection of varusdeformity
  • Case #71827 year malemonostotic fibrousdysplasia femur
  • Lateral view
  • Case #71947 year malemonostotic fibrousdysplasia femurLateral view
  • Bone scan
  • Case #72010 year femalemonostotic fibrousdysplasia proximalfemur with pathologicfracture
  • Case #72116 year femalesolitary bone cystfemoral neck
  • X-ray picture oneyear following bonegrafting procedure
  • Then developes tibial pain because of fibrous dysplasia
  • Macro cyst seen in fibrous dysplasia beneath cortical window
  • cortical window macro cyst Surgical specimen following curettement
  • Photomic with alphabet soup bone
  • Appearance following curettement
  • Placement of ipsilateral fibular bone strut
  • fibular strutX-ray a year later
  • Case #72238 year femalemonostotic fibrousdysplasia tibia
  • Close up view
  • Bone scan
  • Case #72339 year femalemonostotic fibrousdysplasia tibia
  • CT scan
  • Case #724 20 year female with monostotic fibrous dysplasia tibia
  • Coronal T-2 MRI
  • Axial T-2 MRI
  • osteoclastsmetaplastic bone Photomic
  • Case #725 27 year male monostotic fibrous dysplasia tibia
  • Another view
  • Case #72637 year malemonostotic fibrousdysplasia fibula
  • Bone scan
  • Sagittal T-1 MRI
  • Sagittal T-2 MRI
  • Case #7278 year femalemonostotic fibrousdysplasia humeruswith path fracture
  • X-ray one year laterwith 2nd path fracture
  • CT scan
  • Case #72837 year femalemonostotic fibrousdysplasia humerus
  • Lateral view
  • Bone scan
  • Coronal T-1 MRI
  • Post op x-ray fibularfollowing bone strutgraft
  • Case #72945 year female withmonstotic fibrous dysplasiahumerus
  • Lateral viewdistal humerus
  • Surgical debriedment of fibrous tissue
  • Photomic
  • Placement of IM nail prior to cementation
  • Cementation completed
  • nailPost op x-ray2.5 years later
  • Case #73028 year femalemonostotic fibrousdysplasia humerus
  • Case #731 36 year male with monostotic fibrous dysplasia scapula
  • Case #73219 year femalemonostotic fibrousdysplasia prox radius
  • Sagittal T-1 MRI
  • Sagittal T-2 MRI
  • Case 73314 year malemonostotic fibrousdysplasia radius
  • Case #734 Lateral view 13 year female with monostotic fibrous dysplasia ulna
  • AP view
  • Case #735 16 year female with monstotic fibrous dysplasia ulna
  • Case #73611 year femalemonostotic fibrousdysplasia 1stmetatarsal
  • Case #737 26 year female with monstotic fibrous dysplasia 3rd rib
  • Macro section of resected rib
  • Photomic
  • Case #73839 year femalemonostotic fibrousdysplasia 12th rib gall stones
  • Case #739Monostotic fibrousdysplasia 10th ribin a 25 year female
  • Case #740 46 year female with monostotic fibrous dysplasia pelvis
  • CT scan
  • 3-D CT anterior oblique reconstruction
  • 3-D CT posterior oblique reconstruction
  • Case #741 14 year male with monostotic fibrous dysplasia pelvis
  • CT scan
  • Lower cut
  • Case #741.137 year female withincidental finding onX-ray of pelvis
  • Axial T-1 T-2 FS Gad
  • Cor T-1 T-2 FS Gad
  • Sag T-2 FS Gad
  • Case #742 22 year male with fibrocartilaginous dysplasia sacrum
  • Bone scan
  • Axial T-1 MRI
  • tumorAxial T-2 MRI
  • Coronal T-1 MRI
  • tumorCoronal T-2 MRI
  • cartilage fibrous bone Photomic
  • cartilagefibrous Photomic
  • Photomic showing chondroid matrix
  • Case #743 CT scan23 year female with monostotic fibrous dysplasia L-spine
  • Another cut
  • Another cut
  • Case #744 17 year female with monostotic fibrous dysplasia skull
  • Case #74516 year malemonostotic fibrousdysplasia maxilla
  • Skull x-ray
  • Photomic
  • Case #746 Monostotic fibrous dysplasia frontal bone
  • Case #747 22 year male monostotic fibrous dysplasia mandible
  • Case #748 23 yr female Monostotic fibrous dysplasia mandible
  • PolyostoticFibrous dysplasia
  • CLASSICCase #13625 year malepolyostotic fibrousdysplasia
  • Photo of left faceinvolvement includingeye displacement fromorbital disease
  • Skull x-ray showingchanges left side ofskull and face
  • Shepherd’s crookdeformity proximalfemur
  • Macro section ofshepherd’s crookdeformity
  • Photomic showing alphabet soup metaplastic bone
  • Soap-bubblyhumeral defect
  • R LMajor changes left and minor changes right hand
  • Case #749 Pelvis & hip x-ray 25 year male with polyostotic fibrous dysplasia
  • tibiaX-ray femur and femur femurtibia
  • Autopsy specimen offemur cut in path lab
  • Macro section ofproximal femur
  • Photomic with punctate metaplastic bone
  • Case #75032 year male withpolyostotic fibrousdysplasia
  • Views front and back
  • Shepherd’s crookdeformity femur
  • Skull deformities
  • feetHand and footX-rays hands
  • Case #75120 year femalepolyostotic fibrousdysplasia
  • Side view
  • path fractureX-rays hand and humerus
  • AP x-ray pelvis and hips with path fracture femoral neck
  • Leg x-ray withvarus deformity
  • Case # 752 large café-au-lait spot 29 year male with polyostotic fibrous dysplasia
  • AP x-ray showingextensive thinningand dilatation oftibial cortex
  • Lateral view
  • Amputation specimen cut in path lab
  • Photomic
  • Case #75358 year male withpolyostotic fibrousdysplasia of femoriAnd feet
  • Femoral head specimen from total hip surgery
  • Photomic
  • Post op x-ray withlong stem femoralcomponent
  • Lateral view showingold healed pathfracture
  • Opposite hip withsimilar defects L
  • Involvement of both feet
  • Case #753.1 31 year female with mid thigh pain for 1 year
  • Bone scan
  • Coronal T-1 Coronal T-2
  • Coronal T-1 Coronal T-2
  • Axial T-1 Axial T-2
  • Case #753.2 Polyostotic fibrous dysplasia 16 year male with deformity of proximal thumb for years
  • X-rays of right upper extremity
  • Post op x-ray following curettement and bone grafting
  • Case #754 34 year female with polyostotic fibrous dysplasia
  • Case #75519 year malepolyostotic fibrousdysplasia withshepherd’s crookdeformity femur
  • Skull involvement
  • L R Changes in right hand more than left
  • Case #75626 year malepolyostotic fibrousdysplasia left hip
  • Case #756.1 40 yr male with fibrous dysplasia humerus and prox ulna with prior surg 20 yrs ago and now aneurysmal changes
  • Sagittal T-1 MRI Sagittal T-2 MRI
  • Coronal T-1 Coronal STIR Coronal Gad MRI
  • Case #75728 year femalepolyostotic fibrousdysplasia LS spine
  • Oblique views LS spine
  • Lateral view
  • Sagittal T-1 MRI
  • Axial T-1 MRI sacrum
  • Sagittal T-2 MRI with cystic changes
  • Axial T-2 MRI with fluid-fluid level in sacrum
  • cystAxial gad contrast MRI with rim enhancement
  • Sagittal gad contrast MRI with lumbar cysts
  • Photomic from sacral biopsy
  • MazabraudSyndrome
  • Case #75839 year femaleMazabraud syndromepolyostotic fibrousdysplasia with softtissue myxomasCT skull
  • fibrous dysplasia myxomamyxoma Coronal T-1 MRI
  • Coronal T-2 MRI showing high signal myxomas
  • Case #759 fibrous dysplasia 62 year female with Mazabraud’s syndrome
  • AP shoulder
  • Bone scan
  • Bone scan skull
  • Bone scan femur
  • fibrousCoronal T-1 MRI showing multiple myxomas
  • fibrousCoronal T-2 MRIshowing high signalmyxomas
  • fibrousAxial T-1 MRI with low signal myxomas
  • fibrousAxial T-2 MRI with high signal myxomas
  • Coronal T-2 MRI with high signal myxomas
  • Albright’s Disease
  • CLASSICCase #1377 year femaleAlbright’s disease
  • X-ray at 13 yearsminimal changes offibrous dysplasia
  • More changes at 18 years
  • Stress symptoms in both hips at age 24
  • Close up right hipage 24
  • cancellous graftPost op x-ray with fibular struts in both femoral necks
  • Cibagram showing thedifference between therough serrated edges ofthe café-au-lait spots offibrous dysplasia on topcompared to the smoothedges of neurofibromatosisseen on the bottom
  • Case #760 35 year female with Albright’s disease
  • Close up left hip
  • Frog lateral left hip
  • R L CT scan shows more disease on left side
  • Café-au-lait spot in left axilla
  • Photomic from proximal femoral biopsy
  • fibrousHigher power showing metaplastic bone
  • Case #760.1 40 year female a short left leg and early menarchy
  • Case #76127 year femaleAlbright’s diseasehumerus
  • Shepherd’s crookdeformity proximalfemur
  • Post op x-ray showingattempt to correct thevarus deformity
  • X-ray 1 year later withfailure of fixation dueto nail cut out
  • Side plate on left tibiato reduce stress pain
  • One year later with new DHS fixation to replace failed nail
  • Case #762 10 year female with Albright’s of pelvis and spine
  • Coronal CT of LS spine
  • Sagittal CT scan
  • Axial CT scan
  • Sagittal T-1 MRI LS spine with multi level disease
  • Axial T-2 MRIof LD spine
  • Case #763 mandibular lesion 37 year female with Albright’s disease
  • View of opposite side of mandible
  • AP pelvis
  • IM nail for stresspain
  • Case #76464 year femaleAlbright’s diseasewith stress painproximal femur
  • Bone scan
  • Post op bipolarprosthesis
  • DesmoplasticFibroma of Bone
  • Desmoplastic Fibroma of Bone Desmoplastic fibroma of bone is an extremely aggressive, butbenign, fibrous lesion seen in long bones as well as the mandible.It represents 1% of all bone tumors and occurs typically in childrenand young adults with equal sex distribution. The long bonesinvolved include the distal femur, proximal tibia, proximalhumerus and the lower half of the pelvis. Radiographically, theselesions have an aggressive and moth-eaten appearance, erodinginto cortical bone and breaking out into the adjacent soft tissue,much like the low grade fibrosarcoma. Histologically, the tumorappears identical to aggressive fibromatosis seen in soft tissue withintense collagen formation by aggressive-appearing fibroblasts withan occasional mitotic figure. With curettement alone, the recurr-ence rate is very high, in the neighborhood of 40%, and for thisreason most authorities suggest a wide resection and possibleprosthetic replacement. This tumor will not metastasize to the lung.
  • CLASSICCase #13827 year femaledesmoplastic fibromadistal femur
  • Lateral view
  • Photomic
  • Case #13913 year femaledesmoplastic fibromaproximal fibula
  • Lateral view
  • Coronal T-1 MRI
  • Coronal T-2 MRI
  • Proximal fibular resection specimen
  • growth plate Cut specimen in path lab
  • Case #765 25 year female with desmoplastic fibroma prox tibia
  • Lateral view
  • Bone scan
  • CT scan
  • Another CT scan
  • Coronal T-1 MRI
  • Axial PD MRI
  • Post op x-ray followingcurettement andcementation
  • Lateral view
  • Case #76625 year femaledesmoplastic fibromaproximal femur
  • Post op x-ray followingcurettage, IM nail andbone graft
  • X-ray 3 years laterand nail removed
  • Case #76728 year maledesmoplastic fibromadistal femur
  • 6 months later withprogression of diseaserequiring distal femoralresection and TKA
  • Lateral view
  • Case #76819 year femaledesmoplastic fibromadistal femur
  • Case #769 30 year male with desmoplastic fibroma distal radius
  • Lateral view
  • Photomic
  • Case #770 6 year male with desmoplastic fibroma distal radius
  • Case #771 22 year female with desmoplastic fibroma prox ulna
  • Fibrosarcoma of Bone
  • Fibrosarcoma of Bone Fibrosarcoma of bone is a malignant spindle cell sarcoma seen inan older age group with a peak incidence in the fourth decade oflife. It is ten times less frequent than osteosarcoma but involvessimilar bones including the distal femur, proximal tibia, pelvis,proximal femur and proximal humerus. It is rare in the spine, handor foot. Radiographically, the fibrosarcoma has a permeative lyticappearance in the metaphyseal ends of long bones, similar to thatseen in lymphomas or malignant fibrous histiocytoma. Histo-logically, the tumor demonstrates aggressive fibroblastic activitywith collagen formation similar to what is seen in desmoplasticfibromas but with a much higher mitotic index. The lesions can below grade or high grade. The low grade lesions carry a goodprognosis with a low incidence of metastasis to the lung, whereasthe high grade lesions, just like with osteosarcoma, are moreaggressive with a worse prognosis and a higher potential for
  • metastasis. Treatment consists of wide local resection of the tumor,if possible. If the lesion is high grade, adjuvant therapies includinglocal radiation therapy and chemotherapy are frequently implementedto reduce the chance of pulmonary metastasis. Limb salvageprosthetic reconstructions are frequently utilized as in the case ofosteosarcoma.
  • CLASSICCase #14061 year malepathologic fracture thrufibrosarcoma distal femur
  • Lateral view
  • 3 months later afterORIF not knowingthis was a sarcoma tumor
  • Another 3 monthsgo by with continuedtumor growth
  • Photomic from biopsy and amputation 6 months out
  • Case #141 29 year female with fibrosarcoma 5th metatarsal
  • Case #77226 year malefibrosarcomadistal femur
  • Cut specimen in pathlab after amputation
  • Macro section tumor
  • Photomic
  • Case #773 44 year female with fibrosarcoma femoral neck
  • Cut specimen in path lab after internal hemipelvectomy
  • Macro section tumor
  • Photomic
  • Case #774 43 year female with fibrosarcoma femoral neck
  • Laminogram cut
  • Marginal resection of head and neck cut in path lab
  • Photomic
  • 1 year post op withbipolar hip recon
  • Case #77562 year femalepath fracture prox femurthru primary fibrosacomathought to be metastaticbreast disease
  • Post op x-ray followingORIF at which time theunexpected biopsydiagnosis of primarysarcoma was made
  • Surgical specimenafter wide resectionentire proximal femurwith fixation devise
  • Specimen cut inpath lab tumor
  • Photomic
  • Proximal femoral recon with custom bipolar arthroplasty
  • Post op x-ray
  • Case #776 48 year female with fibrosarcoma distal femur
  • Resected distalfemur cut in path lab tumor
  • Photomic
  • Post op x-ray withcustom prosthesis
  • Case #77742 year femalefibrosarcoma prox tibia
  • 2 years later
  • 4 more years withfinal diagnosis andamputation
  • Lateral view
  • Photomic
  • Pulmonary met 4 years after amputation
  • metmet Autopsy photo of multiple lung mets
  • Case #77864 year malefibrosarcomaproximal humerus
  • Case #77973 year malefibrosarcomadistal humerus
  • Lateral view
  • Case #780 69 year female with fibrosarcoma proximal ulna
  • AP view
  • tumor CT scan
  • tumorradialheadProximal radius and ulna resection with custom implant
  • Photomic
  • Prosthesis in place ready for skin closure
  • median nerve radial nerve Another view of implant prior to closure
  • Post op x-ray
  • Case #781 tumor 20 year female with fibrosarcoma sacrum
  • Case #78221 year malefibrosarcomamandible
  • Case #78338 year femalefibrosarcomaarising from monostoticfibrous dysplasiaproximal tibia
  • Previous fibrous dysplasianew fibrosarcoma
  • Lateral view
  • Malignant Fibrous Histiocytoma of Bone
  • Malignant Fibrous Histiocytoma of Bone Malignant fibrous histiocytoma of bone is a relatively new patho-logical entity in bone tumors, compared to soft tissue tumorswhere malignant fibrous histiocytoma is a more common tumor.Malignant fibrous histiocytoma (MFH) of bone occurs in a middleor older age group, more commonly in males than females. It isfound typically in the same locations as fibrosarcoma or osteo-sarcoma, including the distal femur, proximal tibia, and proximalhumerus. Radiographically, it is an aggressive permeative lesionin metaphyseal bone with significant cortical destruction that canbreak out into the adjacent soft tissue. Histologically, there isevidence of malignant fibroblasts as well as atypical histiocytesand giant cells, exactly as we see in soft tissue MFH. Because thisis usually a high grade tumor, the prognosis is quite guarded witha high potential for metastasis to the lung, similar to fibrosarcomaand osteosarcoma. Treatment consists of a wide surgical resection
  • with prosthetic reconstruction when possible, along withadjuvant chemotherapy to reduce the chance of pulmonarymetastases.
  • CLASSIC Case #14226 year male with pathfracture thru MFHfemoral neck
  • Coronal T-1 MRI
  • Coronal T-2 MRI
  • Axial T-1 MRI
  • Cut femoral head in path lab
  • Photomic
  • Case # 143 50 year female MFH distal femur
  • Sagittal T-1 MRI
  • tumor tumorAxial proton density MRI
  • Photomic
  • Post op x-raycustom TKA
  • Lateral view proxend of implant
  • Case #143.1 Multifocal MFH 37 year male with painful lump behind knee for 3 months
  • Cor T-1 T-2 Gad
  • Axial T-1 T-2Gad
  • 1906Cor T-2 Axial T-2
  • tumorWide surgical resection and rotating hinge recon
  • Post op X-ray
  • Case #784 27 year female MFH proximal femur
  • Surgical incision to include biopsy site over trochanter
  • biopsyProximal femoral resection with biopsy site
  • Proximal femoral resection cut in path lab
  • Photomic
  • Custom proximal femoral resection bipolar prosthesis
  • Ready for abductor attachment
  • Closure soft tissue flaps
  • Abductor tendon sutured to implant
  • Post op x-ray
  • 10 years later withstem loosening due tostress shielding
  • X-ray 2 years afterrevision with theCompress system toavoid further stressshielding
  • 5 years out showinghypertrophic bonyresponse at the bonespindle interphase
  • Case #78527 year femaleMFH distal femur
  • Lateral view
  • X-ray of cementedstem of customimplant soon aftersurgery
  • 11 years later withstem loosening fromstress shielding
  • 4 years after revisionsurgery with stemfracture which wasrevised to a Compresssystem which becameinfected resulting inamputation
  • Case #78630 year femaleMFH distal femur
  • Bone Scan
  • Coronal T-1 MRI
  • Coronal gad contrast MRI
  • Another coronal gad contrast MRI
  • Case #787 35 year male with MFH femoral head
  • Frog lateral
  • CT scan
  • Bone scan
  • Case #78837 year malepath fracture thruMFH proximal femur
  • Bone scan
  • Biopsy site at time of surgical resection
  • biopsy siteProximal femoral resection specimen
  • Tumor site uncovered on back table
  • Photomic
  • Custom proximal femoral bipolar prosthesis ready for implant
  • Post op x-ray
  • Case #789 49 year male with path fracture thru MFH prox femur
  • Bone scan
  • Post op x-ray afterORIF for what thesurgeon thought wasa metastatic tumor
  • Surgical specimenafter wide resectionproximal femur withentire fixation devicefor primary sarcoma
  • Photomic
  • Reconstruction withcustom proximalfemoral bipolarprosthesis
  • Case #790 36 year male with myxoid MFH distal femur
  • Lateral view
  • Oblique view
  • CT scan
  • Bone scan
  • Coronal T-1 MRI
  • Sagittal T-1 MRI
  • Sagittal T-2 MRI
  • Case #79175 year maleMFH patella
  • CT scan
  • tumorSoft tissue window CT scan
  • Axial T-1 MRI
  • Sagittal T-1 MRI
  • Case #79234 year maleMFH proximal tibia
  • biopsy siteWide resection proximal tibia
  • Custom long stemspherocentric TKAcomponents
  • Post op x-ray afterautoclaving the surgicalspecimen and replacingit over a long stemtibial component
  • Case #793 infarct36 year femaleMFH at site of aprevious bone infarct
  • infarct Different rotation
  • glenoidWide resectionproximal humerus biopsy
  • Resected surgicalspecimen biopsy
  • Photomic
  • Close up
  • Autoclaved specimencemented over longstem Neer prosthesis
  • Reimplanted
  • Post op x-ray
  • 11 years laterwith proximal migrationand loss of autoclavedbone
  • Case #795 tumor 41 year female with MFH pelvis
  • tumorCoronal T-1 MRI
  • tumor Axial T-1 MRI
  • Case #795.1 54 year old male with right hip pain for 3 months
  • CT scan
  • Bone scan
  • Cor T-1 STIR Gad
  • Axial T-1 T-2
  • Chest CT
  • Case #79643 year femaleMFH pelvis
  • Case #79775 year femaleMFH sacrum tumor
  • Case #798 43 year female with multifocal MFH tibia and radius
  • Sagittal T-1 MRI
  • Coronal T-2 MRI
  • Distal radius
  • Case #79955 year malemetal associatedMFH with pathfracture at tip offemoral component
  • Close up x-rayshowing permeativelysis at fracture site
  • OR x-ray showing cortical lysis at fracture site
  • Biopsy photomic at time of surgery
  • Immediate post opX-ray
  • X-ray several weekslater tumor
  • Case #80084 year malemetal associated MFHaround femoralcomponent THA loose stem
  • X-ray 3 months afterrevision surgery
  • 3 months later we seecortical lysis medially
  • 5 months after anotherrevision surgery withextensive corticallysis 2nd to MFH
  • More lysis at tipof stem from MFHHemipelvectomywas carried outand died soon afterwith pulmonary mets
  • Leiomyosarcoma of Bone
  • Leiomyosarcoma of Bone Leiomyosarcoma of bone is an extremely rare diagnosis withfewer than 50 cases in the world literature. It occurs typically inmiddle to older age adults in the same location in the skeleton asfibrosarcoma, MFH, and osteosarcoma are found in; distal femur,proximal tibia and upper humerus. Radiographically, these lesionshave a permeative lytic appearance, similar to a lymphoma,withextensive cortical breakdown and extension into the surroundingsoft tissue. Histologically, the malignant spindle cells with collagenformation look like a fibrosarcoma or MFH so that the only waya diagnosis can be established is by using the specific immuno-histochemical staining for smooth muscle protein. Treatment usuallyconsists of wide surgical resection and prosthetic implantation.Because of the poor prognosis with high grade lesions, they arefrequently treated with adjuvant systemic chemotherapy andradiation which is not very effective, especially in old patients.
  • CLASSIC Case #144 69 year female with leiomysarcoma proximal humerus
  • Rotational view
  • Bone scan
  • Surgical photo ready for wide resection including biopsy site
  • tumor bulge humeralcut head glenoid Wide resection proximal humerus
  • cutSurgical defect ready for reconstruction
  • Resected specimen
  • Specimen cutin path lab
  • Photomic
  • Custom long stemNeer prosthesis inposition
  • Cement reconstructionaround proximal stem
  • Immediate post opX-ray cement
  • Case #14546 year femaleleiomyosarcomaproximal humerus
  • Coronal proton density MRI