Soft tissue sarcoma in Italy: From epidemiological data to clinical networking to improve patient care and outcomes

Abstract

Sarcomas are rare malignant neoplasms that develop from mesenchymal cells and include a heterogeneous and large group of histological subtypes that may occur at any anatomical site. Soft tissue sarcomas (STS), the focus of this review, account for ≈70‒80% of sarcomas and represent <1% of all cancers. The heterogeneity of STS applies to both their topography and morphology, and 5-year survival can vary widely depending on disease stage and the complex interplay between anatomical site and histology for different STS subtypes. The rarity and heterogeneity of STS, together with other factors, such as the lack of clinical expertise often lead to difficulties and delays in making an accurate diagnosis and to the inappropriate management of each STS subtype. Therefore, this group of cancers requires special attention and approaches to diagnosis and treatment. Epidemiological data on STS are limited, and concerns have been raised regarding accurate registration of STS in cancer registries, including issues related to details of the histotypes. This review provides an overview of the epidemiology of STS in Italy, focusing on data from the Italian Association of Cancer Registries (AIRTUM), and compares findings with those from other European countries. Based on these data, and considering that STS is among the most common group of rare cancers, the relevance of multidisciplinary care for STS patients through reference centres, clinical networks and collaborative disease-specific groups is discussed.

Keywords: Cancer registries; Epidemiology; European reference network; Histology; Incidence; Italian rare cancer network; Prevalence; Reference centres; Regional variability; Soft tissue sarcoma.