Periosteal chondrosarcoma: a case report and review of the literature.

Periosteal chondrosarcoma occurs predominantly in the long tubular bones. The long-term survival rate is better and there are fewer local recurrences than with central chondrosarcoma. A case of periosteal chondrosarcoma is reported with a review of the literature. A 13-year-old girl presented with swelling of the distal right thigh of 3 weeks' duration. Radiographs and computed tomographic scan of the lesion showed a soft tissue mass, measuring 6 x 6 cm, with matrix calcification arising from the surface of the bone. An open biopsy followed by en bloc resection of the tumor was performed. The histologic features were those of a chondrosarcoma. An 8-year follow-up period has shown no local recurrence or distant metastases. The differential diagnosis of periosteal chondrosarcoma includes periosteal (chondroblastic) osteosarcoma and periosteal chondroma. Controversy exists as to whether periosteal chondrosarcoma is an entity distinct from periosteal osteosarcoma. The clinicopathologic features in this case and in the cases reported in the literature support the contention that periosteal chondrosarcoma is indeed distinct.