Secondary chondrosarcoma in osteochondroma: report of 107 patients.

Secondary chondrosarcomas are rare; recognition and diagnosis are difficult. Slow growth and late recurrence require long-term followup to understand the clinical course. In the current study, 107 patients had secondary chondrosarcoma arising in a solitary osteochondroma (61 patients) or multiple exostoses (46 patients). All histologic slides were reviewed without knowledge of the outcome, and radiologic studies were available for review in 71 cases. Patients with secondary chondrosarcoma were one to two decades younger than those with primary chondrosarcoma. Male preponderance and a predilection for flat bones were observed. The radiologic signs of sarcomatous degeneration included irregularity of the margin, inhomogeneous mineralization, and an associated soft tissue mass. The tumors generally were well-differentiated. Only 10 tumors were classified as Grade 2. Five-year and 10-year local recurrence rates were 15.9% and 17.5%, respectively, and 5- and 10-year mortality rates were 1.6% and 4.8% for patients having initial treatment at the authors' institution. Metastasis developed in five patients: in the lung in four patients and in the groin region in one patient. Most patients who died of tumor died of local recurrence. Wide excision had the lowest local recurrence rate. With successful surgical treatment, patients may have long-term disease-free survival.