Objective: The clinical features and the management of alveolar soft part sarcoma (ASPS) are not well known. The efficacy of chemotherapy for soft tissue sarcoma, including high-dose ifosfamide and cisplatin, has not been established yet. Some reports suggest ASPS may occur primarily in bone. Methods: We report on a series of 57 patients with ASPS over 20 years. Their ages ranged from 7 to 75 years (mean 25). Results: There were 37 females and 20 males. Thirteen lesions (23%) showed bone involvement at the primary site, and 6 of them were diagnosed as bone tumors at presentation. Thirty-seven patients had distant metastases at presentation. Tumor size, bone involvement at the primary site and the presence of metastases at presentation were prognostic indicators (p < 0.05). Marginal excision with radiotherapy or wide excision without radiotherapy achieved good local control. Chemotherapy was performed in 47 patients with different regimens. Two patients treated with intra-arterial chemotherapy regimens responded partially, but intravenous chemotherapy with high-dose ifosfamide or cisplatin failed. Conclusions: ASPS can present primarily as a bone tumor. No advantage of chemotherapy with high-dose ifosfamide or cisplatin could be demonstrated.
Absence of severe motor weakness and a central enhancement pattern strongly suggest a benign nature, while severe rest pain and invasive tumor margin suggest malignant lesions in peripheral nerve tumors.
Background: In musculoskeletal sarcomas, brainmetastases are rare, butseverely affectquality of life. Methods: All patients with musculoskeletal sarcomas who were treated at our institutions from 1975 to 1997were reviewed for examples of brain metastasis. Results: Of 480 sarcoma patients, 179 had distant metastases, including 20 patients with brain metastases (4.2%). Alveolar soft part sarcoma (3/4), extraskeletal Ewing's sarcoma (2/8), rhabdomyosarcoma (2/13) and bone Ewing's sarcoma (2/18) tended to metastasize to the brain. All 20 patients had distantor local relapses and 16 of the 20 patients had pulmonary metastases. Three patients underwent surgical treatment and two of them survived over 1 year. Meansurvival after diagnosis of brain metastasis was 5.1 months. Conclusions: Patients with alveolar soft part sarcoma, Ewing's sarcoma, rhabdomyosarcoma and pulmonary metastases have a high risk of brain metastasis.
Despite extensive experience with prosthetic replacement for the reconstruction of limbs following juxta-articular resection of tumours, there are few reports of prosthetic replacement of the distal radius. We present two cases of massive bone defects of the distal radius in which alumina ceramic prosthetic replacements were used. We evaluated the patients more than ten years after the procedure. Both patients had degenerative changes to the wrist. This, however, was not associated with pain or decreased function, and both had returned to their previous occupation after surgery. When a patient has a massive defect of the distal radius, reconstruction using a ceramic prosthesis is a reasonable alternative to using autograft. This method of treatment results in little pain, a moderate range of movement and satisfactory function.
Central enhancement pattern on imaging studies strongly suggests a benign tumor; in contrast, severe motor weakness suggests malignant lesions. Core needle biopsy was reliable with respect to preoperative diagnosis.
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