Extraskeletal myxoid chondrosarcoma of the knee |
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Authors: | Mark C. Gebhardt Selene G. Parekh Andrew E. Rosenberg Daniel I. Rosenthal |
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Affiliation: | (1) Connective Tissue Oncology Center, Boston, Massachusetts, USA, US;(2) Department of Orthopaedic Surgery, Orthopaedic Oncology Service, Gray Building, 6th Floor, 55 Fruit Street, Massachusetts General Hospital, Boston, MA 02114, USA, US;(3) Department of Pathology, Massachusetts General Hospital, Boston, Massachusetts, USA, US;(4) Department of Radiology, Massachusetts General Hospital, Boston, Massachusetts, USA, US |
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Abstract: | Extraskeletal myxoid chondrosarcoma is an uncommon neoplasm, accounting for less than 2% of all soft tissue sarcomas. It affects adult males with a median age in the fifth decade at the time of diagnosis. The tumor usually arises in the deep soft tissues, especially in the lower extremities. Patients present with a gradually enlarging mass that may or may not be associated with pain. This report describes a 25-year-old man who initially presented with a 4- to 5-year history of right knee pain and an enlarging mass in the right knee. Evaluation revealed a cartilaginous neoplasm with no evidence of metastatic disease. The tumor was widely excised and an allograft reconstruction was performed. The patient was closely followed with an eventual above the knee amputation for recurrent myxoid chondrosarcoma. At 34 months, retroperitoneal metastases were noted on abdominal CT. The patient underwent a left radical nephrectomy, renal vein thrombectomy and enucleation of the mass in the right kidney, distal pancreatectomy, and splenectomy. The patient received postoperative chemotherapy. Forty-eight months after initial diagnosis, the patient was found to have recurrent abdominal and retroperitoneal lesions. At 64 months, the patient died from complications of extraskeletal myxoid chondrosarcoma. Received: 6 July 1998 Revision requested: 7 August 1998 Revision received: 29 January 1999 Accepted: 3 February 1999 |
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Keywords: | Extraskeletal myxoid chondrosarcoma Metastasis Kidney Pancreas Chemotherapy Knee |
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