Very long‐term survivors among patients with metastatic soft tissue sarcoma

Abstract

Background Metastatic soft tissue sarcomas (STS) are a group of rare and heterogeneous mesenchymal tumors with a poor prognosis. The aim of this study was to evaluate the incidence of long‐term survivors and describe their presentation and management in a large cohort of patients with metastatic STS. Methods We collected information of patients with metastatic STS managed in Centre Leon Berard between 1985 and 2015 aiming to compare the group of patients alive 5 years after the diagnosis of metastases vs the others. Prognostic factors of patients and tumors characteristics were investigated by logistic regression analysis. For “long‐term survivors,” we explored therapeutic strategies at metastatic stage. Results Out of 436 patients enrolled, 39 (9%) were still alive 5 years after diagnostic of metastases with a median survival of 146 months (12 years). This “long‐term survivors” group included more female and younger patients, with better performance status, more synovial sarcoma or endometrial stromal sarcoma, more patients with simple genomic sarcomas, lower tumor grade, smaller tumor, and longer disease‐free interval. In multivariate analysis, age below 55 at metastatic stage (P = 0.0002) and grade 1 tumor (P < 0.0001) were significantly associated with the “long‐term survivors.” Their therapeutic management was usually aggressive (intensified or polychemotherapy, repeated local treatment of metastases), leading to 62% of complete response in first‐line setting. Conclusions Very long‐term survivors are observed in metastatic STS. Selection of patients in good condition with less aggressive tumor and administration of intensive treatment may lead to obtain these motivating results in a poor prognosis disease.

Molecular mechanisms underpinning sarcomas and implications for current and future therapy

“…147,148 Chemotherapy is the standard form of treatment for metastatic sarcomas; however, the reported median overall survival is only 12 months and <10% of patients have a 5year overall survival rate. 15,149,150 There is therefore clearly a need for more effective therapies as the traditional approaches have been mostly ineffective. Targeted therapies may overcome the current therapeutic limitations; however, most sarcoma subtypes have alterations in many signaling pathways and therefore effective therapy will probably need to target a range of pathways.…”

“…13 Furthermore, the 5-year survival rate for localized STS is about 50% and <10% for metastatic STS. 14,15 While a proposed molecular targeted approach to treatment has gained traction, the molecular mechanisms that drive the sarcoma cells of origin to a transformed phenotype remain to be elucidated. This review, therefore, focuses on the key molecular mechanisms identified to be associated with sarcomagenesis and their potential as novel targets for sarcoma therapy.…”

A product‐limit estimator of the conditional survival function when cure status is partially known

“…The aim was to estimate the survival time of the patients until death from sarcoma as a function of covariates such as the age at diagnosis, sex, tumor site, cancer spread (metastasis), and the margin status. The variables selected for estimating the survival probabilities were previously reported to be related to long‐term sarcoma survival (Carbonnaux et al., 2019; Daigeler et al., 2014, among others).…”

Sarcomas de partes moles nos membros, mais comuns e tão graves quanto os sarcomas ósseos

“…Dos portadores de metástases, < 20% estarão vivos em dois anos. 15 Além da presença de metástases, as margens cirúrgicas desses sarcomas possuem grande impacto no prognóstico. 16,17 Apesar de menos frequentes no grupo pediátrico, os SPM podem acometer esta faixa etária, e o rabdomiossarcoma é o SPM mais comum.…”