Abstract
BackgroundThe exhaustive collection of new sarcoma cases and their second histologic review offer a unique opportunity to study their incidence and time trends in France according to the major subtypes.MethodsData were collected from population-based cancer registries covering 22% of the French population. Crude and world age-standardized incidence rates (ASR) were estimated according to anatomic, histological and genetic groups, age and sex over the 2010–2013 period.ResultsTime trends in incidence were calculated by the annual percent change over the 2000–2013 period. During the most recent period (2010–2013), 3942 patients with sarcoma were included. The ASR of soft-tissue and bone sarcomas, and gastro-intestinal stromal tumors (GIST) were 2.1, 1.0 and 0.6, respectively. For the four most frequent histological subtypes (unclassified, leiomyosarcoma, GIST and liposarcoma), the ASR ranged from 0.4 to 0.7. ASRs were 1.9 for complex genomic and 1.3 for recurrent translocation sarcomas. The time-trend analysis showed a significant increase of sarcoma incidence rate between 2000 and 2005, which stabilized thereafter. Incidence rates increased for four histological subtypes (GIST, chondrosarcoma, myxofibrosarcoma, solitary fibrous tumors) and decreased for three (leiomyosarcomas, Kaposi sarcoma and fibrosarcoma).ConclusionTo our knowledge, this study is the first to investigate sarcoma incidence based on a systematic pathological review of these cancers and on the updated sarcoma classifications. Due to the paucity of literature on sarcomas, future studies using data from population-based cancer registries should consider a standardized inclusion criterion presented in our study to better describe and compare data between countries.
Highlights
The exhaustive collection of new sarcoma cases and their second histologic review offer a unique opportunity to study their incidence and time trends in France according to the major subtypes
Over the 2010–13 period, sarcomas accounted for 1.3% (3942/307,862) of all malignant tumors diagnosed over the French registry area
Sarcomas accounted for 1.3% of all malignant tumors (1.1% for soft tissue -including skin and viscera- and 0.2% for bone) and had an age-standardized incidence rates (ASR)-E of 6.1 per 100,000 person-years over the 2010–2013 period (European population standard)
Summary
The exhaustive collection of new sarcoma cases and their second histologic review offer a unique opportunity to study their incidence and time trends in France according to the major subtypes. Sarcomas are a heterogeneous group of rare malignant tumors derived from primitive mesenchymal cells. These tumors arise from muscle, connective tissue, supportive tissue and vascular tissue, and more than 80 histologic subtypes are included in the 2013 World Health Organization (WHO) Classification of Tumors of Soft Tissue and Bone [1]. In addition to having a multiple and complex histology, these tumors can occur in almost any anatomic site. In spite of these facts, sarcomas account for less than 1% of all adult cancers and for about 20% of all malignant solid tumors in children, adolescents and young adults [2]. Innovation in immune-histochemistry and molecular biology techniques in the last three decades has led to major changes in the diagnosis and classification of sarcoma subtypes
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