Mesenchymal Chondrosarcoma. An Analysis of Patients Treated at a Single Institution

Cesari, Marilena; Bertoni, Franco; Bacchini, Patrizia; Mercuri, Mario; Palmerini, Emanuela; Ferrari, Stefano

doi:10.1177/030089160709300503

Cited by 120 publications

(104 citation statements)

References 18 publications

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“…Information regarding use of chemotherapy is not available within the database and the period of inclusion (1973-2011) includes a period of many years before the use of chemotherapy for treatment of musculoskeletal neoplasms. Although this information would have undoubtedly provided meaningful data to analyze as part of this investigation, it is not felt to be a glaring deficit because the use of chemotherapy for treatment of this disease remains controversial, even by current standards [2,8]. Nevertheless, it is impossible to make any statement regarding the efficacy of chemotherapy on survival.…”

Section: Discussionmentioning

confidence: 99%

“…Few studies have analyzed survival by skeletal and extraskeletal locations. In an analysis of 13 patients with extraskeletal tumors, Hashimoto et al found a 42.8% survival rate at 10 years [11], yet in a separate analysis of 26 tumors, Cesari et al [2] suggested mesenchymal chondrosarcoma of the soft tissues may be a more aggressive disease than its skeletal counterpart with a 10-year survival rate of 0% in such tumors compared with 29% for skeletal tumors. Although the current investigation found extraskeletal tumors to be more common than previously reported, we found no difference in survival between skeletal and extraskeletal tumors.…”

Section: Discussionmentioning

confidence: 99%

“…It has been associated with late recurrence and metastasis with reports of tumors recurring over 20 years from initiation of primary treatment. 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].…”

Section: Introductionmentioning

confidence: 99%

“…Tumor grade, extent of surgical margins, and the presence of distant disease have all been associated with worse outcomes in conventional chondrosarcoma [9]. However, analysis of the effects of these variables on the prognosis of mesenchymal chondrosarcoma has been limited, and differences in survival between skeletal and extraskeletal mesenchymal chondrosarcoma have not been well defined [2,8,11]. Identification of the prognostic implications of disease location has not been completed.…”

Section: Introductionmentioning

confidence: 99%

“…(2) What is the effect of demographic and tumor characteristics such as skeletal origin (skeletal or extraskeletal), primary location of disease (cranial, axial, or appendicular), distant metastasis, tumor size, American Joint Committee on Cancer (AJCC) stage [5], grade, age, and gender on survival in patients with mesenchymal chondrosarcoma?…”

Section: Introductionmentioning

confidence: 99%

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

Schneiderman

Kliethermes

Nystrom

2017

Clinical Orthopaedics &Amp; Related Research

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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 KaplanMeier 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. KaplanMeier 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 Each author certifies that he or she, or a member of his or her immediate family, has no funding or commercial associations (eg, consultancies, stock ownership, equity interest, paten...

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

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

Schneiderman

Kliethermes

Nystrom

2017

Clinical Orthopaedics &Amp; Related Research

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Sarcomas of Bone in Adults

Gounder

Yamada

Fabbri

2018

The American Cancer Society's Oncology in Practice

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Mesenchymal chondrosarcoma: An Australian multi‐centre cohort study

et al. 2022

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Background Mesenchymal chondrosarcoma (MCS) is an ultra‐rare sarcoma that follows a more aggressive course than conventional chondrosarcoma. This study evaluates prognostic factors, treatments (surgery, chemotherapy, and radiation), and outcomes in an Australian setting. Methods We collected demographics, clinicopathological variables, treatment characteristics, and survival status from patients with MCS registered on the national ACCORD sarcoma database. Outcomes include overall survival (OS) and progression‐free survival (PFS). Results We identified 22 patients with MCS between 2001–2022. Median age was 28 (range 10–59) years, 19 (86%) had localised disease at diagnosis of whom 16 had surgery (84%), 11 received radiation (58%), and 10 chemotherapy (53%). Ten (52%) developed recurrence and/or metastases on follow‐up and three patients with initial metastatic disease received surgery, radiation, and chemotherapy. At a median follow‐up of 50.9 (range 0.4–210) months nine patients had died. The median OS was 104.1 months (95% CI 25.8–182.3). There was improved OS for patients with localised disease who had surgical resection of the primary ( p = 0.003) and those with ECOG 0–1 compared to 2–3 ( p = 0.023) on univariate analysis. Conclusions This study demonstrates contemporary Australian treatment patterns of MCS. The role of chemotherapy for localised disease remains uncertain. Understanding treatment patterns and outcomes help support treatment decisions and design of trials for novel therapeutic strategies.

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Mesenchymal Chondrosarcoma. An Analysis of Patients Treated at a Single Institution

Abstract: Our experience confirmed the importance of complete surgical remission in MC treatment and suggests that the addition of chemotherapy should offer a benefit in terms of DFS. Due to the rarity of MC, multicentrer studies are needed to identify the most active chemotherapy regimen.

Cited by 120 publications

References 18 publications

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

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

Sarcomas of Bone in Adults

Mesenchymal chondrosarcoma: An Australian multi‐centre cohort study

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