Incidence of Sarcoma Histotypes and Molecular Subtypes in a Prospective Epidemiological Study with Central Pathology Review and Molecular Testing

Abstract

BackgroundThe exact overall incidence of sarcoma and sarcoma subtypes is not known. The objective of the present population-based study was to determine this incidence in a European region (Rhone-Alpes) of six million inhabitants, based on a central pathological review of the cases.Methodology/Principal FindingsFrom March 2005 to February 2007, pathology reports and tumor blocks were prospectively collected from the 158 pathologists of the Rhone-Alpes region. All diagnosed or suspected cases of sarcoma were collected, reviewed centrally, examined for molecular alterations and classified according to the 2002 World Health Organization classification. Of the 1287 patients screened during the study period, 748 met the criteria for inclusion in the study. The overall crude and world age-standardized incidence rates were respectively 6.2 and 4.8 per 100,000/year. Incidence rates for soft tissue, visceral and bone sarcomas were respectively 3.6, 2.0 and 0.6 per 100,000. The most frequent histological subtypes were gastrointestinal stromal tumor (18%; 1.1/100,000), unclassified sarcoma (16%; 1/100,000), liposarcoma (15%; 0.9/100,000) and leiomyosarcoma (11%; 0.7/100,000).Conclusions/SignificanceThe observed incidence of sarcomas was higher than expected. This study is the first detailed investigation of the crude incidence of histological and molecular subtypes of sarcomas.

Retrospective Analysis of 498 Primary Soft Tissue Sarcomas in a Single Turkish Centre

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“…Sarcomas are a rare and heterogeneous group of malignant tumors of mesenchymal origin that comprise approximately 1 percent of all adult malignancies (Fletcher, 2002). Approximately 80 percent of sarcomas originate from soft tissue and the rest from bone.…”

Pazopanib for metastatic soft-tissue sarcoma (PALETTE): a randomised, double-blind, placebo-controlled phase 3 trial

“…3 In Europe the estimated yearly incidence is fi ve cases per 100 000 people. 4 The development of new systemic treatments for softtissue sarcomas has progressed little in the past few decades, with the exception of treatments for gastrointestinal stromal tumours. Patients with metastatic soft-tissue sarcomas have a median overall survival of about 12 months.…”

Outcome of 449 adult patients with rhabdomyosarcoma: an observational ambispective nationwide study

“…Rhabdomyosarcoma (RMS) accounts for <3% of adult soft tissue sarcoma but is the most frequent soft tissue sarcoma histological subtype before age 10 and the 4th most prevalent cancer during childhood 1, 2, 3, 4. Five‐year overall survival (OS) of children has dramatically improved in the last 30 years based on the results of successive studies of large multinational collaborative trials dedicated to children.…”