Mesenchymal chondrosarcoma of soft tissues and bone in children, adolescents, and young adults

Abstract

BACKGROUND. Mesenchymal chondrosarcoma (MCS) is a rare tumor with a strong tendency toward late recurrences leading to reported 10‐year survival rates below 50%. The recommended treatment is resection with wide margins; the effectiveness of chemo‐ and radiotherapy remain poorly defined. As reports about MCS in young patients are scarce, treatment and outcomes of children/adolescents/young adults in the CWS and COSS studies were investigated. METHODS. Since 1977, 15 of >7000 CWS and COSS patients ≤25 years had a confirmed diagnosis of MCS. RESULTS. The median age was 16.6 (range, 1–25) and median follow‐up 9.6 years (range, 1–22). Four MCS were osseous and 11 extraosseous. All but 1 patient had nonmetastatic disease. Tumor sites were head/neck (n = 6), paravertebral (n = 3), pelvis (n = 3), limbs (n = 2), and kidney (n = 1). All tumors were resected, but only 8 completely. Thirteen individuals received chemotherapy, 6 were irradiated. Actuarial 10‐year event‐free and overall survival rates were 53% and 67%, respectively. Four recurrences occurred, all within 4 years from diagnosis (3 local, 1 combined; 2 of these in irradiated patients). One of these patients survived after surgery and radiation for local recurrence. Seven of 8 patients whose tumors were completely resected during primary treatment, but only 4 of 7 patients with incomplete surgery survived disease‐free. CONCLUSIONS. This series of 15 MCS differs from others: the median age was young, most tumors were extraosseous, most patients received chemotherapy, and the outcome was better than published. Despite long‐term follow‐up, characteristic late metastatic recurrences were not observed. Treating MCS according to standard multimodal soft tissue/bone sarcoma regimens is proposed. Cancer 2008. © 2008 American Cancer Society.

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

“…Consistent with previous data, patients with tumors affecting craniofacial sites were found to have a more favorable prognosis [4,16,23]. Although this finding may seem counterintuitive, we hypothesize this could be related to easier earlier detection of masses in these locations or potentially a uniformly different biologic behavior of tumors affecting these locations.…”

“…Although we were able to identify the presence of metastatic disease for the vast majority of the patients in our cohort, we were unable to analyze the timing of metastases or recurrence over the course of followup because these time variables were not coded for within SEER. The absence of documentation on timing of local or distant recurrence is a major limitation because this disease has a documented high rate of late distant and local recurrence [4,8,14,18]. The OS of this disease has varied widely ranging from 20% to 67% [2-4, 8, 11, 14, 16-18, 22, 23].…”

“…The OS of this disease has varied widely ranging from 20% to 67% [2-4, 8, 11, 14, 16-18, 22, 23]. Recent studies examining mesenchymal chondrosarcoma in pediatric patients or limited to craniofacial sites represent the more favorable end of this spectrum [4,16,21,23]. A study from 2014 of 37 patients by Kawaguchi et al [15] is the largest survivorship series performed on this disease, reporting 5-year survival of 51% and 10-year survival of 37%.…”

“…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].…”

“…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].…”