Proceedings of the tumor board of the childrens hospital of los angeles. Malignant fibrous histiocytoma of bone following primitive neuroectodermal tumor of bone
β Scribed by Hays, Daniel M. ;D'Angio, Giulio J. ;Evans, Audrey E. ;Siegel, Stuart E. ;Stanley, Philip ;Shimada, Hiroyuki ;Bernstein, Saul ;Soni, Daljit ;Hays, Daniel M.
- Publisher
- John Wiley and Sons
- Year
- 1994
- Tongue
- English
- Weight
- 637 KB
- Volume
- 22
- Category
- Article
- ISSN
- 0098-1532
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β¦ Synopsis
The patient is a 22-year-old Hispanic male who presented at this hospital in September 1977 at age 8, with a 3-month history of pain just below the left knee following a fall, accompanied by a limp, and a 2-week history of swelling of the left lower leg just distal to the knee. Examination revealed a 7 X 8 cm non-tender firm mass located lateral and distal to the left knee. There was no warmth or discoloration overlying the mass, and there was full range of motion in the left knee and ankle joints. X-ray films of the leg revealed a large destructive lesion of the proximal left fibula.
An incisional biopsy of the mass was performed and light and electron microscopic examination of the tumor led to a diagnosis of Ewing's sarcoma. A metastatic workup consisting of radiographs of the chest and pelvic bones, liverispleen, bone, and gallium scans, and bone marrow aspirate and biopsy revealed no evidence of disease. The patient was placed on the Intergroup Ewing's Sarcoma study and randomized to therapeutic group 3. This consisted of systemic treatment with vincristine, cyclophosphamide, and dactinomycin, accompanied by 5,000 cGy to a field including the shaft of the fibula, and an additional 1,000 cGy to the local tumor area. "Prophylactic" irradiation (1,800 cGy) to both lung fields also was administered. He remained on systemic chemotherapy for 24 months with no evidence of progressive local or metastatic disease. A nondisplaced pathologic fracture of the left tibia occurred during the 9th month of therapy, which was treated with a weight-bearing cast and healed over a 3-month period. At the time of completion of the chemotherapy a biopsy of the left proximal fibula was performed; it showed necrotic bone with calcification.
The patient did reasonably well over the next 1 1 years, returning to normal daily activities including some sports
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