Overall and net survival of patients with sarcoma between 2005 and 2010: Results from the French Network of Cancer Registries (FRANCIM)

Abstract

BACKGROUND: Sarcomas are rare, heterogeneous, ubiquitously localized malignancies with many histologic subtypes and genomic patterns. The survival of patients with sarcoma has rarely been described based on this heterogeneity; therefore, the authors' objective was to estimate survival outcomes in patients who had sarcomas using the 2020 version of the World Health Organization classification of soft tissue and bone tumors. METHODS: Patients older than 15 years who had incident sarcoma diagnosed between 2005 and 2010 were extracted from 14 French population-based cancer registries covering 18% of the French metropolitan population. Vital status for each patient was actively followed up to June 30, 2013. Net survival (NS) was estimated using the unbiased Pohar-Perme method. RESULTS: Overall, 4202 patients were included. NS declined with increasing age at diagnosis. According to topographic groups, large 5-year NS disparities were observed, ranging from 47% among women with gynecologic sarcomas to 89% among patients with skin sarcomas. Patients with soft tissue, bone, and gastrointestinal sarcomas had 5-year NS rates of 53%, 61%, and 70%, respectively. Similar heterogeneity was observed according to histologic subtypes, with 5-year NS ranging from 19% for patients with angiosarcomas to 96% for patients with dermatofibrosarcomas. Patients with sarcoma who displayed missense mutations had a better 5-year NS (74%); those with MDM2-amplified sarcomas had the worst NS (45%). CONCLUSIONS: NS rates in patients with sarcoma are presented here for the first time based on the 2020 World Health Organization classification applied to population-based registry data. Large prognostic heterogeneity was observed based on age, topographic and histologic groups, and genomic alteration profiles, constituting a benchmark for future studies and clinical trials.

Improving Diagnosis and Care for Patients With Sarcoma: Do Real-World General Practitioners Data and Prospective Data Collections Have a Place Next to Clinical Trials?

“…This linkage provides unique insights into the physical and psychosocial issues sarcoma survivor experience, and this input can be used to create more personalized rehabilitation services in sarcoma survivors as this presents a heterogeneous group in which a one-follow-upstrategy-fits-all would prove to be insufficient. 2,6,59,[63][64][65][66][67] This linkage facilitated the comparison of not only patient and tumor characteristics but also attributes between individuals who responded to the questionnaires and those who did not. The knowledge gained, namely, that nonresponders were somewhat younger and had a lower socioeconomic status than responders, could be used to improve response rates in these patient groups in future sarcoma research.…”

PAX–FOXO1 fusion status in children and adolescents with alveolar rhabdomyosarcoma: Impact on clinical, pathological, and survival features

“…Rhabdomyosarcoma (RMS) is the most common soft tissue pediatric malignancy, accounting in France for about 4% and 1% of all cancers in children and in adolescents, respectively 1,2 . Considering the latest French reports, the 5‐year overall survivals (OS) of patients with RMS were worse in adults and in adolescents than in children (26% [18–36], 58.8% [32.5–77.8], and 72.9% [69.9–75.6], respectively) 1,3,4 . But, according to the most recent World Health Organization (WHO) classification scheme, RMS are subdivided into four subtypes, each with differing histological, genetic, clinical, and survival features: embryonal, alveolar (ARMS), spindle cell/sclerosing, and pleomorphic RMS 5 …”

“…1,2 Considering the latest French reports, the 5-year overall survivals (OS) of patients with RMS were worse in adults and in adolescents than in children (26% [18][19][20][21][22][23][24][25][26][27][28][29][30][31][32][33][34][35][36], 58.8% [32.5-77.8], and 72.9% [69.9-75.6], respectively). 1,3,4 But, according to the most recent World Health Organization (WHO) classification scheme, RMS are subdivided into four subtypes, each with differing histological, genetic, clinical, and survival features: embryonal, alveolar (ARMS), spindle cell/sclerosing, and pleomorphic RMS. 5 ARMS, representing 25% of all RMS, preferentially affects adolescents and is notoriously aggressive.…”