Abstract
Sarcomas are a heterogeneous group of rare cancers of mesenchymal origin. In this study, we provide updated, world age-standardised incidence rate (ASR) and European age-standardised incidence rate for malignant soft tissue sarcoma (ICD-O-3 topographic code C47–C49) and bone sarcoma (C40, C41) in Italy, by area (north, centre, and south) and by cancer registry. We also assess morphology in relation to site and area and assess metastases at diagnosis. We analysed 1,112 cases, with incidence 2009–2012, provided by 15 cancer registries (CRs) affiliated to the Association of Italian Cancer Registries (AIRTUM). Overall, ASR was 1.7/100,000/year for soft tissue sarcoma and 0.7 for bone sarcoma. Central Italy had the highest (2.4) ASR and south Italy had the lowest (1.6) ASR for soft tissue sarcoma. Central Italy had the highest (1.1) ASR and north Italy had the lowest (0.7) ASR for bone sarcoma. By CR, ASRs ranged from 1.1 to 2.6 for soft tissue sarcoma and from 0 to 1.4 for bone sarcoma. The most frequent soft tissue sarcomas were sarcoma not otherwise specified (NOS) (29.4%) and liposarcoma (22.2%); the most common bone sarcoma was chondrosarcoma (37.6%). Soft tissue sarcomas occurred most frequently (35.6%) in lower limb connective tissue; bone sarcomas arose mainly (68.8%) in long bones. The frequencies of morphologies arising at different sites varied considerably by Italian area; for example, 20% of hemangiosarcomas occurred in the head and neck in south Italy with 17% at this site in the centre and 6% in the north. For soft tissue sarcoma, the highest ASRs of 2.6 and 2.4 contrast with the lowest ASRs 1.1 and 1.3, suggesting high-risk hot spots that deserve further investigation. The marked variations in morphology distribution with site and geography suggest geographic variation in risk factors that may also repay further investigation particularly since sarcoma etiology is poorly understood.
Highlights
Sarcomas are a heterogeneous group of malignant neoplasms of mesenchymal origin. ey are one of the major groups of rare cancers in Europe [1] accounting for around 1% of all cancers diagnosed [2]. e World Health Organization [3] recognises approximately 70 histotypes grouped into two main categories: soft tissue sarcoma and bone sarcoma
Single institution reports of incidence, survival, and prevalence are unlikely to be reliable, and pooled data from population-based cancer registries are required to obtain representative data. e aim of the present study is to provide updated estimates of the incidence of malignant soft tissue and bone sarcomas in Italy overall, by area and by cancer registry, using data from 15 population-based cancer registries affiliated to the Association of Italian Cancer Registries (AIRTUM) [12]. e presence of metastases at diagnosis and morphology in relation to site—which appears to not have been previously investigated in sarcomas diagnosed in Italy—were assessed
A total of 1,112 sarcoma cases were diagnosed in 2009–2012 in the areas covered by the 15 registries. ere were 859 soft tissue sarcomas and 253 bone sarcomas; 55% of total cases were in males and 45% in females
Summary
Sarcomas are a heterogeneous group of malignant neoplasms of mesenchymal origin. ey are one of the major groups of rare cancers in Europe [1] accounting for around 1% of all cancers diagnosed [2]. e World Health Organization [3] recognises approximately 70 histotypes grouped into two main categories: soft tissue sarcoma and bone sarcoma. E World Health Organization [3] recognises approximately 70 histotypes grouped into two main categories: soft tissue sarcoma and bone sarcoma. In Europe, soft tissue sarcomas are more frequent than bone sarcomas, with world age-standardised incidence rates (ASRs) of 1.5–3.0/100,000/year and 0.5–2.0/100,000/ year, respectively. Both types of sarcoma are moderately more frequent males than females [4]. Soft tissue sarcomas constitute about 84% of the sarcomas diagnosed in Europe [5]. Accurate diagnosis of these entities is challenging in relation to their rarity and morphologic heterogeneity [6]. Liposarcomas arise mainly in the thigh of young adults, and myxofibrosarcomas often arise in the superficial soft tissue of elderly patients [7]. e most common soft tissue sarcoma histotypes in Europe have been reported as leiomyosarcoma (19%), liposarcoma (16%), and sarcoma not otherwise specified (NOS, 14%) [8]
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