Ivan Chebib1, Andrew E Rosenberg2, Christopher D M Fletcher3, Daniel I Rosenthal4, Francis J Hornicek5, G Petur Nielsen6. 1. James Homer Wright Pathology Laboratories, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA. ichebib@partners.org. 2. Department of Pathology, University of Miami Hospital, Miami, FL, USA. 3. Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA. 4. Department of Radiology, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA. 5. Center for Sarcoma and Connective Tissue Oncology, Department of Orthopaedic Surgery, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA. 6. James Homer Wright Pathology Laboratories, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA.
Abstract
AIMS: To assess the clinical and histopathological spectrum of primary intra-articular sarcomas. METHODS AND RESULTS: Fifteen patients were identified as having primary intra-articular sarcomas and the clinical and pathological features were evaluated. There were nine males and six females who ranged in age from 16 to 84 (mean 44) years. All tumours originated in the knee joint. The pathological diagnoses included: five synovial sarcoma, three extraskeletal myxoid chondrosarcomas, two high-grade myxofibrosarcoma (one conventional, one epithelioid), two undifferentiated pleomorphic sarcoma (one with giant cells) and one each myxoinflammatory fibroblastic sarcoma, conventional hyaline chondrosarcoma, and high-grade myofibroblastic sarcoma. All tumours were treated by segmental resection or amputation. Adjuvant therapy was given in selected cases. Follow-up ranged from 11 to 150 months. Of patients with follow-up, two died of disease; one developed pulmonary metastases after 6 years and was then lost to follow-up. Nine patients were alive and free of disease 12-150 months after diagnosis. CONCLUSIONS: Primary sarcomas of the joints are very rare and most frequently affect the knee. Our experience indicates that synovial sarcoma and extraskeletal myxoid chondrosarcoma are the most common intra-articular sarcomas.
AIMS: To assess the clinical and histopathological spectrum of primary intra-articular sarcomas. METHODS AND RESULTS: Fifteen patients were identified as having primary intra-articular sarcomas and the clinical and pathological features were evaluated. There were nine males and six females who ranged in age from 16 to 84 (mean 44) years. All tumours originated in the knee joint. The pathological diagnoses included: five synovial sarcoma, three extraskeletal myxoid chondrosarcomas, two high-grade myxofibrosarcoma (one conventional, one epithelioid), two undifferentiated pleomorphic sarcoma (one with giant cells) and one each myxoinflammatory fibroblastic sarcoma, conventional hyaline chondrosarcoma, and high-grade myofibroblastic sarcoma. All tumours were treated by segmental resection or amputation. Adjuvant therapy was given in selected cases. Follow-up ranged from 11 to 150 months. Of patients with follow-up, two died of disease; one developed pulmonary metastases after 6 years and was then lost to follow-up. Nine patients were alive and free of disease 12-150 months after diagnosis. CONCLUSIONS:Primary sarcomas of the joints are very rare and most frequently affect the knee. Our experience indicates that synovial sarcoma and extraskeletal myxoid chondrosarcoma are the most common intra-articular sarcomas.