Mesenchymal chondrosarcoma of bone and soft tissue. A review of 111 cases

Abstract

A series of 111 mesenchymal chondrosarcomas was reviewed. The ages of the patients ranged from 5 to 74 years, and approximately 60% of them were in the second and third decades of life. There was no significant sex predilection. Seventy-two tumors, including 5 that involved multiple skeletal sites, arose in bone. Thirty-eight tumors were found in extraskeletal sites. At initial diagnosis, multifocal involvement, both in bone and in soft tissue, was observed in one case. Roentgenographically, the lesions in bone frequently resembled ordinary chondrosarcomas, showing osteolytic and destructive appearances with stippled calcification. Tumors in extraskeletal sites were almost always identified as calcified masses. Histologically, a combination of cellular zones composed of undifferentiated small cells and chondroid zones typically presented a bimorphic appearance that was virtually pathognomonic in most cases. Ablative surgical treatment seemed to be the procedure of choice. The value of irradiation or chemotherapy (or both) was difficult to assess in the current study. Prognosis for patients with mesenchymal chondrosarcoma is usually poor, and long-term follow-up is necessary. In a group of 23 patients from the Mayo Clinic, the 5-year survival rate was 54.6% and the 10-year survival rate was 27.3%.

Survival in Mesenchymal Chondrosarcoma Varies Based on Age and Tumor Location: A Survival Analysis of the SEER Database

“…Although general trends and features of the clinical behavior of mesenchymal chondrosarcoma have been suggested in prior studies [1,14,15,17,18], no large series have been available to verify these descriptions as a result of the rarity of this disease. To date, the largest study on mesenchymal chondrosarcoma was performed by Nakashima et al [18], which presented 111 new patients with mesenchymal chondrosarcoma and reviewed 132 previously reported patients.…”

“…To date, the largest study on mesenchymal chondrosarcoma was performed by Nakashima et al [18], which presented 111 new patients with mesenchymal chondrosarcoma and reviewed 132 previously reported patients. However, that investigation included survival analysis for only 23 patients and contained limited subgroup analysis.…”

“…In comparison to conventional chondrosarcoma, which has a 10-year overall survival rate of 60% to 70% [6,7,9], the mesenchymal subtype is believed to portend a worse prognosis [2,14,17]. The 10-year survivorship of mesenchymal chondrosarcoma has been previously reported as ranging from 20% to 67% [3,8,11,14,15,17,18,21] with cohorts comprised of head and neck tumors or of pediatric patients representing the upper end of this spectrum [4,16,23]. The disease recently was reported to have a median event-free survival of 57 months [25].…”

“…Additionally when compared with the \ 1% occurrence of extraskeletal classic chondrosarcoma, mesenchymal chondrosarcoma is thought to have a higher proportion of tumors arising in extraskeletal locations [4,15]. Extraskeletal mesenchymal chondrosarcoma has been reported to occur in 14% to 73% of patients [4,8,14,15] with the largest series reporting that 39% had extraskeletal tumors [18].…”

Chondrogene Tumoren des Skeletts

“…Abb. 8b, d) [17]. Im Gegensatz zum Ewing-Sarkom lassen sich nach eigenen Erfahrungen immer Retikulinfasern nachweisen, die einzelne oder kleine Gruppen von Tumorzellen umgeben (.…”