Rare Bone Tumors

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Information about Rare Bone Tumors

Published on October 21, 2007

Author: naneria

Source: authorstream.com

Rare Bone Tumors:  Rare Bone Tumors Vinod Naneria Consultant Orthopaedic Surgeon Choithram Hospital & Research Centre, Indore, M.P., India Slide2:  Synovial Chondromatosis, Fibromatosis Heamengiomas Osteochondritis dissecans Multiple Myeloma Osteoid Osteoma Osteochondroma Lipoma Slide3:  Fibroma Non-ossifying Fibroma Fibro-cortical defects Paget disease Osteogenesis Imperfecta Osteopetrosis Hyper-parathyroidism Neurofibromatosis Neurilemoma Slide4:  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. Slide5:  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. Synovial Chondromatosis:  Synovial Chondromatosis A Senior Physician had some pain lt hip 1990 Plain x-rays were negative. CT scan showed some loose bodies in Fovea centralis. Since then managed conservatively. Slide8:  CT Scan 1990 Slide10:  MRI – 2006 to complete the investigation did not provide much information, a big loose body is seen clearly. Joint appears normal. Slide11:  Case 2 Slide12:  Case 3 Knee joint Slide14:  Case 4 Large loose bodies Slide15:  Case 5 Slide16:  Case 6 a 40 years female with chronic synovitis Of the knee, underwent Arthroscopic debridement. Multiple multi-color loose bodies removed. Slide17:  Arthroscopic Removal of loose bodies Slide18:  3/2005 5/ 2 0 0 6 Case 7 Slide19:  Case 8 Slide20:  Case 9 Case 9 Slide22:  Clinical photos with pain free Range of movements Slide23:  Case 10 Two huge loose bodies in the sub scapular bursa Accommodated in the infra-clevicular area. These bodies were Removed 25 years ago. Slide24:  Calcific Tendonitis of Ligamentum patellae Slide25:  Skin painted with lead oxide (Geru) Fibromatosis:  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. Slide27:  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. Slide28:  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. Slide29:  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. Slide30:  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. Slide33:  Popliteal swelling with FFD Slide34:  Anterior knee- patient in prone position Swelling in popliteal space Heamengiomas:  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. Slide37:  Intra-osseous, Intra-mascular Intra-neural heamengioma Intrmascular Heamengioma Slide38:  Intra mascular Heamengioma OSTEOCHONDRITIS DISSECANS OF KNEE :  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. Slide41:  The loose body was found free & was removed arthroscopically Multiple myeloma:  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. Slide43:  Plasmacytoma Slide46:  2002 2007 Osteoid Osteoma:  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. Osteoid Osteoma:  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 "double-density sign," 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. Osteoid Osteoma - Tx:  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. Osteoid Osteoma - Tx:  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. Slide52:  Giant osteoid osteoma Slide53:  Tibia posterior & lateral border Slide54:  Lamina D11 Slide55:  Medial wall Acetabulum Osteochondroma:  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. Lipoma:  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. Fibromas:  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. Slide71:  Fibromas NONOSSIFYING FIBROMA :  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. Slide73:  Non Ossifying Fibroma of right Acetabulum Slide74:  1999 1999 2007 2007 Fibrous cortical defects:  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. Fibro-cortical defects:  Fibro-cortical defects Paget disease:  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 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 "pagetoid" 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). Osteogenesis Imperfecta:  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. Osteopetrosis:  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 Slide84:  A very old x-ray showing sclerosis at Subtrochanteric level on both sides. Developed fracture right femur at this site Slide85:  Fractured healed after 3 surgeries Slide86:  CT Scan Showing Bone Marrow obliteration of femur 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 "brown tumor“. Blood tests - serum calcium, phosphorus, alkaline phosphatase, and hyperparathyroid hormone levels. Sonography of neck for adenoma and abdomen for stones. HYPERPARATHYROIDISM Slide88:  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. Slide92:  "BROWN TUMOR" OF HYPERPARATHYROIDISM Neurofibromatosis:  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. Case history:  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. Slide97:  café au lait spots Slide98:  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. Slide100:  Nerve sheath Nerve sheath Nerve sheath Slide101:  Tumor mass Normal Nerve Nerve sheath Slide102:  14cm long tissue along the sciatic nerve Tissue removed from the Gluteal region Slide106:  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. Slide107:  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. Case 2:  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. DISCLAIMER:  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

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