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.
Highlights
Sarcomas are a heterogeneous group of rare malignant tumors of connective tissues, capable of differentiation into many different cell types such as connective tissues, visceral tissues and bone
Patient characteristics From March 1, 2005 to February 28, 2006, 1287 suspected sarcoma cases were reported by RA pathologists and 748 (58%) patients were found eligible for inclusion
Many studies have reported on the incidence of primary bone and soft tissue lesions, this is the first exhaustive collection of cases on a regional basis, with centralized pathology review coupled with molecular characterization
Summary
Sarcomas are a heterogeneous group of rare malignant tumors of connective tissues, capable of differentiation into many different cell types such as connective tissues (lipocytes, fibrous supporting structures, muscle, etc.), visceral tissues and bone. These tumors can occur in almost any anatomic site, they are reported to be more frequent in the extremities [1]. The exact overall incidence of sarcomas is unknown and the incidence of the different histological and molecular subtypes has not been determined precisely [7]. 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
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