Cases reported "Osteosarcoma"

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1/7. Multifocal osteogenic sarcoma in Paget's disease.

    The most serious complication of Paget's disease is sarcomatous degeneration of pagetic bone. Multifocal sarcomatous degeneration occurs mainly in polyostotic Paget's disease. Multifocal Paget's sarcoma is uncommon and can arise in any site. We report two cases of synchronous multifocal sarcomatous degeneration. The two patients were elderly women (aged 77 and 86 years, respectively) who developed sarcomatous lesions concomitantly, in the first case report in left ilium, left tibia, and first lumbar vertebra and in the second case report in the skull, right ilium, and sacrum. Whether these cases are due to the simultaneous development of several primaries or to metastases from a single primary remains unclear.
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keywords = sacrum
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2/7. Reconstruction after total en bloc sacrectomy for osteosarcoma using a custom-made prosthesis: a technical note.

    STUDY DESIGN: A report of an innovative technique to restore the lumbosacral junction after resection of primary highly malignant osteosarcoma of the sacrum involving the whole sacrum, soft tissues, and adjacent posterior parts of both iliac wings. OBJECTIVES: To describe the planning and design of a custom-made sacral prosthesis, the surgical technique, and clinical and functional outcome of the patient. SUMMARY OF BACKGROUND DATA: Although there have been case reports about reconstruction methods after total sacrectomy, to date, there has not been a reported clinical case of successful reconstruction using an individual designed prosthesis based on a three-dimensional real-sized model. methods: A 42-year-old woman was referred with progressive neurologic impairment due to primary osteosarcoma of the sacrum invading surrounding structures. Based on a three-dimensional real-sized model, a detailed surgical plan was developed to assure safe, wide surgical margins. In addition, the model enabled design and testing of a custom-made sacral prosthesis, to provide stable lumbosacral reconstruction. RESULTS: After induction chemotherapy, a staged anteroposterior resection-reconstruction was successfully performed. After surgery, a superficial wound dehiscence was promptly treated. Within 3 weeks after surgery, mobilization began, and the adjuvant chemotherapy was continued. At the 36-month follow-up, the patient was disease free, had a stable, painless spinopelvic junction, and could walk short distances using ankle orthoses and crutches. Radiographs show complete incorporation of the pelvic grafts and unchanged position of the implant. CONCLUSIONS: In planning and performing a total sacrectomy, including substantial parts of iliac wings, a three-dimensional real-sized model offers surgeons distinct advantages. Wide bony resection margins can be drawn on the model, and an individual custom-made prosthesis to re-establish spinopelvic continuity can be designed and tested before the intervention.
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keywords = sacrum
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3/7. Extracorporeal irradiation for malignant bone tumors.

    PURPOSE: Extracorporeal irradiation (ECI) has been used selectively in the management of primary malignant bone tumors since 1996. We report our techniques for ECI and the short-term oncologic and orthopedic outcomes. methods AND MATERIALS: Sixteen patients with primary malignant bone tumors were treated with ECI from 1996 to 2000. The median age was 14 years. The histologic diagnoses were Ewing's sarcoma (11), osteosarcoma (4) and chondrosarcoma (1). The treated sites were femur (7), tibia (4), humerus (2), ilium (2), and sacrum (1). Following induction chemotherapy in Ewing's sarcomas and osteosarcoma, en bloc resection of the tumor and tumor-bearing bone was performed. A single dose of 50 Gy was delivered to the bone extracorporeally using either a linear accelerator (9 cases) or a blood product irradiator (7 cases). The orthopedic outcome was recorded using a standard functional scale. RESULTS: At a median follow-up of 19.5 months, there were no cases of local recurrence or graft failure. One patient required amputation due to chronic osteomyelitis. For the 10 patients with follow-up greater than 18 months, the functional outcomes were graded good to excellent. CONCLUSION: The short-term oncologic and orthopedic results are encouraging and suggest that ECI provides a good alternative for reconstruction in limb conservative surgery in selected patients. This technique should only be used in a multidisciplinary setting, where careful follow-up is available to assess the long-term outcomes.
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keywords = sacrum
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4/7. osteosarcoma of the spine: experience of the Cooperative osteosarcoma Study Group.

    BACKGROUND: Due to the low incidence rate, the optimal strategy for the treatment of patients with spinal osteosarcoma is unknown. methods: Twenty-two patients with osteosarcoma of the spine (15 with tumors of the sacrum and 7 with tumors at other sites) who received chemotherapy according to the Cooperative osteosarcoma Study Group protocol were analyzed. Six patients presented with metastasis, and 16 patients had no evidence of metastasis at the time of entry into the protocol. Of 12 patients who underwent excision of their tumors, 2 patients underwent wide excision, 3 patients underwent marginal excision, and 7 patients underwent intralesional excision. Eight patients received irradiation: Six patients received conventional radiotherapy only, one patient received neutron beam therapy, and one patient received samarium-153-ethylene diamine tetramethylene phosphonate therapy. Follow-up ranged between 24 months and 105 months (median, 47 months). RESULTS: The median survival was 23 months, and three patients have survived without disease for > 6 years. patients with primary metastases (P = 0.004), large tumors (P = 0.010), and sacral tumors (P = 0.048) had lower overall survival compared with patients who had no metastasis, small tumors, and nonsacral tumors, respectively. There was a significant difference in overall survival between 5 patients who underwent either wide or marginal surgery and 17 patients who underwent either intralesional surgery or no surgery (P = 0.033). Among 17 patients who underwent no surgery or intralesional surgery, overall survival tended to be better in 7 patients who received irradiation compared with the overall survival in 10 patients who did not receive irradiation (P = 0.059). CONCLUSIONS: patients with metastases, a large tumors, and sacral tumors had a poor prognosis in the current study with small numbers of patients. Wide or marginal excision of the tumor improved survival. patients with osteosarcoma of the spine should be treated with a combination of chemotherapy and at least marginal excision for those with surgically accessible tumors. Postoperative radiotherapy may be beneficial.
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keywords = sacrum
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5/7. Familial incidence of Paget's disease and secondary osteogenic sarcoma. A report of three cases from a single family.

    This report describes a family of three siblings with a long history of polyostotic Paget's disease. Because of the severe osseous involvement with Paget's disease, all three patients had problems that required orthopedic surgery. Subsequent to these surgeries, two patients developed osteogenic sarcoma at sites unrelated to their past procedures, one in the sacrum and one in the calvarium. Both patients died shortly after their diagnosis because of the aggressive spread of the tumor. Although the etiology of Paget's disease and its complication of osteogenic sarcoma still remain to be clarified, this and other case reports suggest a possible environmental or hereditary contribution to developing osteogenic sarcoma in Paget's disease. patients with a familial clustering of polyostotic Paget's disease may benefit from more thorough screening tests to detect malignant transformation.
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keywords = sacrum
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6/7. radiation induced osteosarcoma of the sacrum following radiation of an undiagnosed bone lesion.

    Years ago a 20-year-old patient presented with low back pain. Radiologically a cystic lesion of the sacrum was found and interpreted as malignant tumor. Without biopsy and histological diagnosis the patient was given radiation therapy with 21 000 rad. One year later a similar lesion was radiated in the left femoral neck. A pathological fracture ensued recessitating several operations. Twenty years after initial radiation therapy the patient developed a rapidly growing tumor of the sacrum, metastasizing to the lungs. One year later he died.
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keywords = sacrum
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7/7. Benign giant cell tumor of bone with osteosarcomatous transformation ("dedifferentiated" primary malignant GCT): report of two cases.

    It is not uncommon for sarcomatous transformation of giant cell tumor (GCT) of bone to occur after radiation, but rarely does malignant transformation occur spontaneously, with less than 15 cases reported up to 1995. Only four of these cases have been documented in detail. We report two additional cases of GCT of bone spontaneously transforming or "dedifferentiating" into osteosarcoma without radiation therapy. The first case is absolutely unique and most interesting in that the dedifferentiation process occurred in one of multiple GCT lung metastases 6 years after successful eradication of a primary tibial tumor. The right lung was resected due to development of a large tumor, and at pathologic examination, demonstrated several small nodules of conventional GCT and a much larger, 14-cm mass composed of a mixture of GCT and high-grade osteosarcoma. The second case involved a physician, who had a large tumor in the sacrum with vague symptoms for 8 years. Open biopsy revealed conventional, benign GCT of bone with a secondary aneurysmal bone cyst. Complete curettage 2 weeks later revealed, in addition to areas of conventional, benign GCT a second component of very high grade osteosarcoma. Both patients died less than 1.5 years from diagnosis. This report of osteosarcomatous transformation of a conventional GCT of bone strengthens the theory that there is a mesenchymal cell line in GCT that may spontaneously transform to sarcoma.
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