Rare Bone Tumours


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a collection of rare bone tumor and tumor like conditions for students of orthopaedic surgery.

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Rare Bone Tumours

  1. 1. Rare Bone Tumors Vinod Naneria Consultant Orthopaedic Surgeon Choithram Hospital & Research Centre, Indore, M.P., India
  2. 2. Contents • Synovial Chondromatosis, • Fibromatosis • Heamengiomas • Osteochondritis dissecans • Multiple Myeloma • Osteoid Osteoma • Osteochondroma • Lipoma
  3. 3. Contents…. • Fibroma • Non-ossifying Fibroma • Fibro-cortical defects • Paget disease • Osteogenesis Imperfecta • Osteopetrosis • Hyper-parathyroidism • Neurofibromatosis • Neurilemoma
  4. 4. Synovial chondromatosis Monoarticular synovial proliferative disease in which cartilaginous or osteocartilaginous metaplasia occurs within the synovial membrane of joints, bursae, or tendon sheaths. Three phases: (1) early, with synovial chondrometaplasia but no loose bodies, (2) transitional, with active synovial disease and loose bodies, and (3) late, with loose bodies but no synovial disease.
  5. 5. Routine investigations - X-ray, arthroscopy, CT scan, & MRI. common in the knee and hip, but almost any joint, bursa, or tendon sheath may be affected. Treatment is controversial; synovectomy and removal of the loose bodies, simple removal of the loose bodies, either by arthroscopy or open operation. Recurrence after surgery is not unusual, and there are rare reports of malignant transformation to chondrosarcoma.
  6. 6. Synovial Chondromatosis cases….. • A 60 yrs. male had some pain lt hip 1990 • Plain x-rays were negative. • CT scan showed some loose bodies in Fovea centralis. • Since then managed conservatively. • A 17 years follow up.
  7. 7. CT Scan 1990
  8. 8. MRI – 2006 to complete the investigation did not provide much information, a big loose body is seen clearly. Joint appears normal.
  9. 9. Case 2
  10. 10. Case 3 Knee joint
  11. 11. Case 4 Large loose bodies
  12. 12. Case 5
  13. 13. Case 6 a 40 years female with chronic synovitis Of the knee, underwent Arthroscopic debridement. Multiple multi-color loose bodies removed.
  14. 14. Arthroscopic Removal of loose bodies
  15. 15. Case 7 3/2005 5/ 2 0 0 6
  16. 16. Case 8
  17. 17. Case 9 Case 9
  18. 18. Clinical photos with pain free Range of movements
  19. 19. Case 10 Two huge loose bodies in the sub scapular bursa Accommodated in the infra-clevicular area. These bodies were Removed 25 years ago.
  20. 20. Calcific Tendonitis of Ligamentum patellae
  21. 21. Skin painted with lead oxide (Geru)
  22. 22. Fibromatosis Many fibrous proliferative lesions recur after removal but do not metastasize. As a group these are known as the fibromatoses. Many have been defined precisely and have been named, but occasionally one is encountered that can be recognized as a fibromatosis but cannot be classified exactly.
  23. 23. • Desmoid tumors. • Fibromatosis colli (congenital torticollis). • Keloid. • Irradiation fibromatosis. • Palmar and plantar fascial fibromatoses. • Peyronie disease. • Juvenile aponeurotic fibroma. • Elastofibroma dorsi. • Progressive myositis fibrosa. • Congenital generalized fibromatosis. • Infantile dermal fibromatosis. • Fibrous hamartoma of infancy.
  24. 24. Desmoid tumors are locally aggressive lesions of connective tissue origin. Infiltrate surrounding tissues and have a marked propensity for persistence. These lesions occur most frequently in the anterior abdominal wall of women who have borne children. In other locations are often known as extraabdominal desmoids.
  25. 25. They are highly collagenized and rather sparsely cellular and infiltrate the muscle in which they develop. Grossly they are dense, hard, rubbery, and grayish white. Extraabdominal desmoids occur most frequently in the shoulder girdle, arm, thigh, neck, pelvis, forearm, and popliteal fossa.
  26. 26. The natural history is usually a slow, relentless growth with invasion of contiguous structures. Spontaneous regression does occur, reported 29% of patients treated by partial excision were without evidence of disease at last follow-up. Metastases have been reported but are extremely rare. Most institutions currently recommend wide or marginal excision followed by radiotherapy. Regression following the use of indomethacin and ascorbic acid, tamoxifen and testolactone, and chlorothiazide has been reported.
  27. 27. Popliteal swelling with FFD
  28. 28. Swelling in popliteal space Anterior knee- patient in prone position
  29. 29. Heamengiomas • Cavernous hemangiomas consist of widely dilated vessels or thicker vessels that may resemble veins; Hemangiomas occur deep in skeletal muscle and other soft tissues of the extremities. • Recur unless completely removed. • Occasionally a histologically benign hemangioma stubbornly persists after surgery and becomes disabling. Some intramuscular hemangiomas are infiltrative and most difficult to excise except by radical surgery. Unlike many soft tissue tumors, hemangiomas may be quite painful.
  30. 30. Intrmascular Heamengioma Intra-osseous, Intra-mascular Intra-neural heamengioma
  31. 31. Intra mascular Heamengioma
  32. 32. OSTEOCHONDRITIS DISSECANS OF KNEE • The classic medial femoral condyle localization had a better prognosis than an unusual one. • Patients with no effusion, a lesion less than 20 mm, and no gross detachment had significantly better results after conservative treatment than those who had undergone operation. If signs of detachment were present, the results were better after operative than after conservative treatment. • Arthroscopic drilling of osteochondritis dissecans lesions -no signs of improvement after 6 months of conservative treatment. • MRI is a highly sensitive method for detection of unstable osteochondritis dissecans. The presence of an underlying high- signal-intensity line, cystic area, or a focal articular defect indicates instability and may help in preoperative planning. Whether the lesion is drilled, excised, curetted, replaced and pinned, or bone grafted depends on the size, stability, and weight-bearing nature of the lesion, which can be determined only at surgery.
  33. 33. The loose body was found free & was removed arthroscopically
  34. 34. Multiple myeloma • Multiple myeloma is the most common primary malignancy of bone. • 43% of primary malignancies of bone. • Its peak incidence is in the fifth through seventh decades with a 2:1 male predominance. • Multiple myeloma and metastatic carcinoma should be included in the differential diagnosis for any patient over the age of 40 with a new bone tumor.
  35. 35. Plasmacytoma
  36. 36. 2007 2002
  37. 37. Osteoid Osteoma • Benign neoplasm most often seen in young males. • Found in the first three decades of life, but an occasional lesion has been reported in older patients. • Almost any bone can be involved, although there is a predilection for the lower extremity, with half the cases involving the femur or tibia. • The tumor may be found in cortical or cancellous bone, producing a distinct roentgenographic appearance of cortical sclerosis; 5% of tumors are subperiosteal. • Multicentric foci have been reported. • No malignant change has ever been documented. The typical patient has pain that is worse at night and relieved by aspirin. • When the lesion is near a joint, swelling, stiffness, and contracture may occur. When in a vertebra, scoliosis may occur. • Occasionally, osteoid osteoma occurs with minimal pain. In children, overgrowth and angular deformities may occur.
  38. 38. Osteoid Osteoma • Routine roentgenograms often are diagnostic, but bone scans or CT are often required to localize the lesion accurately. • CT may detect the nidus, whereas roentgenograms show only sclerosis. • A bone scan is helpful in detecting the quot;double- density sign,quot; which is a focal area of increased activity with a second smaller area of increased uptake superimposed on it, is said to be diagnostic of osteoid osteoma.
  39. 39. Osteoid Osteoma - Tx • The entire nidus must be removed. • Block resection of the nidus. • An alternative method - shave the reactive bone with a sharp osteotome until the nidus is encountered, then curet the exposed nidus. • Intraoperative localization of the nidus is possible with preoperatively injected technetium- labeled methylene diphosphonate and a sterile, wrapped Geiger counter. • If block excision is performed, intraoperative roentgenograms of the specimen.
  40. 40. Osteoid Osteoma - Tx • Excision of the osteoid osteoma nidus using CT–assisted localization, a Kirschner wire inserted into the nidus, and a biopsy punch inserted over the Kirschner wire into the bone. • They recommend using a trephine 2 mm larger than the lesion for complete removal. • Recurrence after apparently complete excision has been reported but is rare. • Percutaneous radiofrequency ablation of osteoid osteomas. • Spontaneous disappearance of osteoid osteomas after extended observation and treatment of symptoms.
  41. 41. Giant osteoid osteoma
  42. 42. Tibia posterior & lateral border
  43. 43. Lamina D11
  44. 44. Medial wall Acetabulum
  45. 45. Osteochondroma • The most common of the benign bone tumors. • They are probably developmental malformations rather than true neoplasms and are thought to originate within the periosteum as small cartilaginous nodules. • The lesions consist of a bony mass, often in the form of a stalk, produced by progressive endochondral ossification of a growing cartilaginous cap. • Most lesions are found during the period of rapid skeletal growth. About 90% of patients have only a single lesion. Osteochondromas may occur on any bone preformed in cartilage but usually are found on the metaphysis of a long bone near the physis. They are seen most often on the distal femur, the proximal tibia, and the proximal humerus. Rarely do they develop in a joint.
  46. 46. Lipoma • Lipomas most common benign tumors of connective tissue. • They may occur at any age and in either sex. • These tumors usually develop subcutaneously but may involve the deeper structures. They occasionally affect the synovium (lipoma arborescens) and rarely the periosteum. • Clinically they are soft, circumscribed, movable masses that are painless and grow slowly. • On roentgenograms the larger ones appear as discrete radiolucent areas within soft tissue. • Grossly a lipoma is a well-encapsulated nodule of fat that may contain fibrous tissue. • Microscopically it is composed of mature fat cells.
  47. 47. Fibromas • Most of these lesions are found in the upper extremity and are 1 to 2 cm in diameter. • They differ histologically from giant cell tumor of tendon sheath by the absence of xanthoma cells and giant cells. • Proper treatment is marginal excision. • In the hand, persistence as high as 24% has been reported. • Reexcision of the recurrence has been curative in most patients.
  48. 48. Fibromas
  49. 49. NONOSSIFYING FIBROMA • Nonossifying fibromas have the same histological structure as fibrous cortical defects, but they are larger. • Most occur in children and adolescents between the ages of 10 and 20 years. • The roentgenographic appearance usually is characteristic. • The osteolytic area is eccentrically located and oval. Multilocular appearance or ridges in the bony wall, sclerotic scalloped borders, and erosion of the cortex are frequent findings. • CT & biopsy be necessary for diagnosis. • Regress spontaneously.
  50. 50. Non Ossifying Fibroma of right Acetabulum
  51. 51. 1999 1999 2007 2007
  52. 52. Fibrous cortical defects • Fibrous cortical defects, although classified as bone tumors, are probably developmental abnormalities and are believed to occur in as many as 35% of children. • Found incidentally. • Metaphyseal region of long bones in ages of 2 and 20 years and occur predominantly in males. Approximately 40% are found in the femur, 40% in the proximal tibia, and 10% in the fibula, although some occur in the upper extremity, clavicle, ilium, ribs, vertebrae, skull, mandible, or small bones of the hand and feet.
  53. 53. Fibro-cortical defects
  54. 54. Paget disease Paget disease is an entity of unknown cause characterized by osteoclastic resorption of bone followed by osteoblastic regeneration of a primitive woven bone. These processes may result in (1) a roentgenographic appearance simulating a malignant tumor, (2) deformity, or (3) a pathological fracture of a long bone. Biopsy may be required to establish the diagnosis. Intraosseous arteriovenous shunts may develop and lead to impressive bleeding during surgical procedures on involved bone. Bowing deformities of long bones may make internal fixation of pathological fractures technically difficult. Nerves passing through foramina in quot;pagetoidquot; bone may be entrapped and lead to troublesome symptoms. Occasionally decompressive surgical procedures may be indicated. Sarcomatous change in pagetoid bone is not rare (Fig. 23-15).
  55. 55. Osteogenesis Imperfecta • Osteogenesis imperfecta is a disease apparently of the mesodermal tissues with abnormal or deficient collagen that has been demonstrated in bone, skin, sclerae, and dentine. The so-called diagnostic triad of blue sclerae, dentinogenesis imperfecta, and generalized osteoporosis in a patient with multiple fractures or bowing of the long bones usually is used clinically.
  56. 56. Osteopetrosis • osteopetrosis is failure of osteoclastic and chondroclastic resorption; - bones become exceedingly dense; - marrow space is filled with unresorbed dense bone; • There is absence of marrow elements & these patients are susceptible to infectious diseases. • Most common is osteomyelitis of Mendible. • Sub trochanteric fractures are common and usually ends up in nonunion
  57. 57. A very old x-ray showing sclerosis at Subtrochanteric level on both sides. Developed fracture right femur at this site
  58. 58. Fractured healed after 3 surgeries
  59. 59. CT Scan Showing Bone Marrow obliteration of femur
  60. 60. HYPERPARATHYROIDISM Caused by overactivity of the parathyroid glands, usually resulting from neoplasm or hyperplasia of the glands. The symptoms may be renal, psychiatric, or skeletal. The skeletal change usually is limited to diffuse demineralization. Only rarely does the change become markedly focal and produce a quot;brown tumor“. Blood tests - serum calcium, phosphorus, alkaline phosphatase, and hyperparathyroid hormone levels. Sonography of neck for adenoma and abdomen for stones.
  61. 61. In hyperparathyroidism: Histopathology (1) the giant cells are a little smaller, often occurring in a somewhat nodular arrangement, especially around areas of hemorrhage; (2) the stromal cells are more spindle shaped and delicate; and (3) evidence of osseous metaplasia within the stroma is prominent. The bone surrounding the lesion should also be examined; in hyperparathyroidism it may show intense osteoclastic and osteoblastic activity associated with peritrabecular fibrosis.
  63. 63. Neurofibromatosis • Neurofibromas occur as a manifestation of von Recklinghausen disease, in which many such tumors may be found associated with café au lait spots and various other lesions. Sometimes a special type of neurofibroma occurs in which entire peripheral nerves are involved, this is referred to as a plexiform neurofibroma, and excising it completely may be impossible. Other manifestations of neurofibromatosis are hypertrophy of soft tissue, including the skin, hypertrophy of bone, scoliosis, bone cysts, and other abnormalities.
  64. 64. Case history • 4 yrs M, unable to stand due to painful swelling in left gluteal region since infency. • Clinically hyperalgesia in gluteal muscle mass with defuse soft to firm and nodular at places. Limb lengthening by 4cm. • Skin – hyperpigmentation. • Mother had same nodules and pigmentation.
  65. 65. café au lait spots
  66. 66. X-ray MRI Defuse soft-tissue tumor mass in the gluteal region and around sciatic Nerve, extending into pelvis, upto spine S1 area. Also in brain.
  67. 67. Nerve sheath Nerve sheath Nerve sheath
  68. 68. Nerve sheath Tumor mass Normal Nerve
  69. 69. 14cm long tissue along the sciatic nerve Tissue removed from the Gluteal region
  70. 70. Neurilemoma is typically a solitary encapsulated lesion that may be cystic when it is as large as 3 to 4 cm in diameter. Usually it involves one of the larger peripheral nerves. There may be little if any neurological deficit. Often the lesion simply spreads the nerve fibers apart without anatomical or functional interruption, so the tumor can be removed by careful blunt dissection after a longitudinal incision in the perineurium.
  71. 71. Thus there may be little if any increase in the dysfunction of the nerve after surgery. Occasionally the tumor may recur, but usually the lesion or recurrent lesion can be removed without sacrificing a significant number of nerve fibers. Excision that interrupts the continuity of the nerve should be avoided. Often there is cystic degeneration in the center of the nerve.
  72. 72. Case 2 • A 40 M with chronic pain rt.leg 5 years • Clinically a ill defined swelling in the antero-lateral aspect of leg from head of fibula to mid leg. • A small swelling on left leg lower part on anterior border of fibula.
  73. 73. DISCLAIMER • Information contained and transmitted by this presentation is based on personal experience and collection of cases at Choithram Hospital & Research centre, Indore, India, during last 25 years. • It is intended for use only by the students of orthopaedic surgery. • Views and opinion expressed in this presentation are personal opinon. • Depending upon the x-rays and clinical presentations viewers can make their own opinion. • For any confusion please contact the sole author for clearification. • Every body is allowed to copy or download and use the material best suited to him. I am not responsible for any controversies arise out of this presentation. • For any correction or suggestion please contact • naneria@yahoo.com