The Differences Between Intracranial Mesenchymal Chondrosarcoma and Conventional Chondrosarcoma in Clinical Features and Outcomes.

OBJECTIVE: To report differences in clinical features and outcomes between intracranial mesenchymal chondrosarcoma (MCS) and conventional chondrosarcoma (CCS). METHODS: Clinical data of patients with primary intracranial MCS and CCS were retrospectively extracted and analyzed to compare differences between MCS and CCS. RESULTS: Seventy-four patients with intracranial chondrosarcoma (61 cases with MCS and 13 cases with CCS) were included. Compared with patients with CCS, patients with MCS presented at a younger mean age (21.1 years vs. 34.5 years, P < 0.001) and had a poor mean preoperative Karnofsky performance scale score (64.6 vs. 77.8, P = 0.014). Compared with CCS, MCS was less often located in the skull base (38.5% vs. 96.7%, P < 0.001) and had a larger tumor volume (87.8 cm3 vs. 26.7 cm3, P < 0.001). Rates of gross total resection in MCS and CCS subgroups were 41.1% (n = 25) and 46.2% (n = 6), respectively; rates of adjuvant radiotherapy postoperatively were 44.2% (n = 27) and 46.2% (n = 6), respectively. After mean follow-up of 41.7 months, 1-year, 3-year, and 5-year overall survival and progression-free survival of MCS were significantly shorter than overall survival and progression-free survival of CCS. Multivariate analysis revealed that tumor pathology and extent of surgery were independent predictors for tumor recurrence. CONCLUSIONS: Clinical features of MCS are quite different from CCS. Treatment strategies used for CCS do not yield satisfactory outcomes for MCS. Treatment of MCS should be aggressive and individualized.

Intracranial Mesenchymal Chondrosarcoma: Report of 16 Cases.

OBJECTIVES: Limited data regarding intracranial mesenchymal chondrosarcoma (MCS) are available. The goal of this study was to report the clinical characteristics, challenges in management, and poor outcomes of intracranial MCS. METHODS: Clinical data for 16 patients with MCS were reviewed retrospectively to evaluate their clinical characteristics, management, and outcomes. RESULTS: This study included 11 male and 5 female patients with a mean age of 22.9 ± 14.4 years. The most common presentations were headache (n = 10; 62.5%), followed by cranial deficits (n = 7; 43.6%). The radiologic spectrum for MCS was broad, and only 18.8% (3/16) of MCSs were correctly diagnosed preoperatively. Aggressive resection (including subtotal resection and gross total resection) and partial resection was performed in 62.5% (10/16) and 37.50% (6/16) of patients. With a median follow-up of 34 months (range, 10-78 months), 5 patients (31.3%) died and 8 patients (50%) developed tumor recurrence. The 1-, 3-, and 5-year rates of progression-free survival and overall survival were 86%, 53%, and 42% and 93%, 70%, and 56%, respectively. Although the differences were not significantly different, aggressive resection and the use of radiotherapy tended to improve the prognosis of the patients. CONCLUSIONS: Clinical characteristics of MCS are variable. The current management of intracranial MCS referring to conventional chondrosarcoma could not yield satisfactory outcomes. Further study is needed to identify the optimal treatments.

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

BACKGROUND: Studies suggest that mesenchymal chondrosarcoma is associated with a poorer prognosis and a higher proportion of extraskeletal tumors than conventional chondrosarcoma. However, these investigations have been small heterogeneous cohorts, limiting analysis of prognostic factors. QUESTIONS/PURPOSES: (1) What is the 5- and 10-year survival rate of patients diagnosed with mesenchymal chondrosarcoma? (2) What is the effect of demographic and tumor characteristics on survival in patients with mesenchymal chondrosarcoma? METHODS: The Surveillance, Epidemiology, and End Results (SEER) database was used to identify all patients diagnosed with mesenchymal chondrosarcoma from 1973 to 2011. SEER reports survival data on over 8.2 million patients with cancer and has attained 98% completeness in reporting. Using variables within the database, this study designated each patient's tumor as skeletal or extraskeletal and cranial, axial, or appendicular, respectively. Overall survival (OS) was determined for the entire series as well as each group. Median survival was calculated using Kaplan-Meier methods. Cox proportional hazards regression was used to determine whether demographic and tumor variables affected survival. Two hundred five patients with mesenchymal chondrosarcoma were identified, including 82 (40%) skeletal and 123 (60%) extraskeletal. RESULTS: OS for the entire series was 51% (95% confidence interval [CI], 43%-58%) and 43% (95% CI, 35%-51%) at 5 and 10 years, respectively. No difference in OS was detected between extraskeletal and skeletal tumors. Kaplan-Meier analyses showed OS was worse for tumors in axial locations compared with appendicular and cranial locations. Appendicular tumors demonstrated an OS of 50% (95% CI, 36%-63%) at 5 years and 39% (95% CI, 26%-52%) at 10 years. OS for axial tumors was 37% (95% CI, 25%-49%) and 31% (95% CI, 20%-43%), whereas it was 74% (95% CI, 59%-84%) and 67% (95% CI, 50%-79%) for cranial tumors at 5 and 10 years, respectively. When controlling for age, sex, tumor origin, and tumor location, the presence of metastasis (hazard ratio [HR], 12.38; 95% CI, 5.75-26.65; p < 0.001) and 1-cm size increase (HR, 1.16; 95% CI, 1.09-1.23; p < 0.001) were both independently associated with an increased risk of death. Tumor location showed different behaviors depending on patient age. In comparison to cranial tumors at age 20 years, the HR was 5.56 (95% CI, 1.47-21.05; p = 0.01) for axial tumors and 6.26 (95% CI, 1.54-25.42; p = 0.01) for appendicular tumors. At age 60 years, those ratios were 0.10 (95% CI, 0.02-0.55; p = 0.01) and 0.14 (95% CI, 0.04-0.58; p = 0.01), respectively. CONCLUSIONS: Our data suggest that extraskeletal tumors are more common than previously reported; however, this factor does not have clear prognostic value. Presence of metastatic disease and increased tumor size are the main predictors of poor survival outcome. Cranial tumors appear to have a different clinical behavior with our data suggesting better overall survival in young patients (compared with axial and appendicular locations) and a worse survival outcome in older patients. LEVEL OF EVIDENCE: Level IV, prognostic study.

Clinical outcomes for patients after surgery and radiation therapy for mesenchymal chondrosarcomas.

INTRODUCTION: We report the outcome of 23 patients with mesenchymal chondrosarcomas treated with surgery and radiation therapy +/- chemotherapy. The intent of the project was to review the impact of patient and treatment variables on treatment outcome, in particular with regard to extent of surgery and radiation dose. PATIENTS AND METHODS: Twenty-three patients with mesenchymal chondrosarcomas were treated with surgery and radiation therapy (min. dose 44 Gy; max. dose 78 Gy; median dose 60 Gy; mean dose 61 Gy). RESULTS: The median survival for the entire cohort of patients was 21.65 years (95% confidence interval ± 4.25). The 5- and 10-year OS was 78.6%. Median disease-free survival for the 23 patients was 7.2 years. Disease-free survival (DFS) at 3 and 5 years was 70.7% and 57.8%, respectively. The local control rate at 5 and 10 years was 89.5% (95%CI 64.1-97.3%). Only three patients experienced local failure, three patients had regional failure, and eight developed distant metastases. CONCLUSIONS: In this cohort of patients local tumor control was high when using a combination of surgery and radiation. There was not a clear relationship between radiation dose and local tumor control. J. Surg. Oncol. 2016;114:982-986. © 2016 Wiley Periodicals, Inc.

Results of sub-analysis of a phase 2 study on trabectedin treatment for extraskeletal myxoid chondrosarcoma and mesenchymal chondrosarcoma.

BACKGROUND: Trabectedin is reported to be particularly effective against translocation-related sarcoma. Recently, a randomized phase 2 study in patients with translocation-related sarcomas unresponsive or intolerable to standard chemotherapy was conducted, which showed clinical benefit of trabectedin compared with best supportive care (BSC). Extraskeletal myxoid chondrosarcoma (EMCS) and Mesenchymal chondrosarcoma (MCS) are very rare malignant soft tissue sarcomas, and are associated with translocations resulting in fusion genes. In addition, the previous in vivo data showed that trabectedin affect tumor necrosis and reduction in vascularization in a xenograft model of a human high-grade chondrosarcoma. The aim of the present analysis was to clarify the efficacy of trabectedin for EMCS and MCS subjects in the randomized phase 2 study. METHODS: Five subjects with EMCS and MCS received trabectedin treatment in the randomized phase 2 study. Three MCS subjects were allocated to the BSC group. Objective response and progression-free survival (PFS) were assessed according to the Response Evaluation Criteria in Solid Tumors (RECIST) version 1.1 by central radiology imaging review. RESULTS: The median follow-up time of the randomized phase 2 study was 22.7 months, and one subject with MCS was still receiving trabectedin treatment at the final data cutoff. The median PFS was 12.5 months (95 % CI: 7.4-not reached) in the trabectedin group, while 1.0 months (95 % CI: 0.3-1.0 months) in MCS subjects of the BSC group. The six-month progression-free rate was 100 % in the trabectedin group. One subject with MCS showed partial response, and the others in the trabectedin group showed stable disease. Overall survival of EMCS and MCS subjects was 26.4 months (range, 10.4-26.4 months) in the trabectedin group. At the final data cutoff, two of five subjects were still alive. CONCLUSIONS: This sub-analysis shows that trabectedin is effective for patients with EMCS and MCS compared with BSC. The efficacy results were better than previously reported data of TRS. These facts suggest that trabectedin become an important choice of treatment for patients with advanced EMCS or MCS who failed or were intolerable to standard chemotherapy. TRIAL REGISTRATION: The randomized phase 2 study is registered with the Japan Pharmaceutical Information Center, number JapicCTI-121850 (May 31, 2012).

Radiation therapy is associated with fewer recurrences in mesenchymal chondrosarcoma.

BACKGROUND: Mesenchymal chondrosarcoma (MSC) is a rare variant of chondrosarcoma. Because of the rarity of the disease, most studies only contain a small number of patients and thus the prognostic variables and role of adjuvant therapies remain controversial. QUESTIONS/PURPOSES: We therefore asked (1) what the overall and disease-free survival were for patients with this diagnosis at 5 and 10 years; (2) whether there were significant prognostic factors associated with survival; and (3) whether use of adjuvant chemotherapy or radiotherapy was associated with survival in patients with MSC. METHODS: We retrospectively reviewed the cases of MSC diagnosed from 1979 to 2010 at one referral center. Forty-three cases were identified. Thirty-seven cases were analyzed for demographics, treatments, and outcomes. Thirty patients with localized disease were analyzed for prognostic factors. The minimum followup was 1 month (mean, 6 years; range, 1 month to 17 years). There were 17 females and 20 males. The mean age at diagnosis was 33 years (range, 11-65 years). Nineteen cases were skeletal and 18 cases were extraskeletal. Seventy-six percent of the tumors were located in the trunk. RESULTS: Five- and 10-year overall survival was 51% and 37%, respectively. Five- and 10-year disease-free survival was 23% and 5%, respectively. Age (< 30 years) and male sex were associated with poorer overall and disease-free survival in patients presenting with a localized tumor, respectively. Patients who did not receive radiotherapy were more likely to have a local recurrence. Adjuvant chemotherapy failed to show a significant association with overall, disease-free, metastasis-free, or local recurrence-free survival. CONCLUSIONS: The present study reinforced the role of adjuvant radiotherapy for local tumor control. LEVEL OF EVIDENCE: Level IV, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

Central nervous system mesenchymal chondrosarcoma.

Central nervous system mesenchymal chondrosarcomas are rare malignant tumors that constitute a separate entity from the classical chondrosarcoma and myxoid variant. Clinical behaviour of central nervous system chondrosarcomas is still unknown. We describe two rare examples of intracranial mesenchymal chondrosarcoma with a review of the literature, in an attempt to clarify the clinical characteristics, prognosis and treatment of choice of these unusual tumors. Among the 55 reported cases, 23 had postoperative radiotherapy. Although there is no statistical significance according to the Log-Rank test (p=0.7), the patients treated with radiation therapy seem to have a better chance of survival. Patients who had adjuvant chemotherapy (only 5) showed survival times similar to those patients who had none. Although clinical behaviour of central nervous system chondrosarcomas remains to be defined, data from our series as well as literature show that radical removal is the best therapeutic choice. In addition, patients treated with postoperative radiotherapy seem to show a trend toward increased survival.

Primary thoracic wall tumours of mesenchymal origin in dogs: a retrospective study of 46 cases.

Forty-three of the chest wall tumours in 46 dogs were malignant; five had metastases apparent at the time of presentation, five more had metastases discovered intraoperatively. Surgical resection of the tumours was associated with a significantly better outcome than conservative management. The median survival times after surgery for dogs with osteosarcoma was 17 weeks, for dogs with fibrosarcoma it was 26 weeks and for dogs with chondrosarcoma it was 250 weeks. En bloc excision of primary tumours affecting the chest wall was associated with minimal morbidity, but long-term survival was limited by distant metastases, primarily to the lungs. The tumours recurred in only three dogs. Early, radical surgical excision is recommended in the management of tumours of the chest wall. The prognosis depends on the histologic type of tumour and a histological diagnosis is mandatory before excision.

Chondrosarcoma of bone: an oncological and functional follow-up study.

We retrospectively analysed the course of 42 out of 45 patients suffering from different chondrosarcomas which were treated surgically. We found a prospective 5- and 10-year survival of 64% for both time intervals. Follow-up examination was possible in 21 of 45 patients. Most of them were staged as NED, one as AWD, and 8 of 45 were lost to follow-up. From 16 dead patients 12 died of the disease and 4 of unknown but not to tumor related reasons. Survival was depending significantly on the histopathological grade, and the stage according to Enneking's surgical staging system. Eleven out of forty-five patients developed metastases within a mean period of twelve months after surgery. The survival of these patients was significantly reduced. Patients with centrally located tumors exhibited a distinct but non-significant worse survival than those suffering from peripheral tumors. In 18% (8 of 45) recurrence of the tumor was evident within a mean period of 24 months (5-85). Functional evaluation was performed in 21 out of 28 alive patients. After a mean follow-up time of 72 months the mean score was 64% (23-100). Females exhibited a distinct but non-significant better result, the same was observed for peripheral locations compared to centrally located tumors. Regarding age, grade, and line of resection no tendency of any dependence was detected, but worst results were seen in those with a stage III tumor and dedifferentiated CS.