Epidemiology and Presentation
This chapter is dedicated to general concepts in soft tissue tumors’ epidemiology and presentation.
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Epidemiology and Presentation
Soft tissue tumors can arise at any age in both genders, but most of them have a predilection for specific age ranges and some are prevalent in or even specific to males or females. Benign soft tissue tumors are about 100 times more frequent than soft tissue sarcomas: in fact, some benign soft tissue tumors (e.g., hemangiomas, lipomas, dermatofibromas) are very frequent in the population; despite their benign nature, some of these benign neoplasms—though rarely—can be life-threatening (e.g., giant hemangiomas complicated by Kasabach-Merritt syndrome or renal angiomyolipomas complicated by hemorrhage). Soft tissue sarcomas—despite their rarity—are prognostically much more relevant since they are deadly in a significant proportion of cases (→ see section entitled “Prognosis”). In the USA, it is estimated that approximately 13,000 new cases are diagnosed each year. The majority of sarcomas arise in somatic soft tissues (about 70%), the remaining developing in viscera (about 20%, most of which being gastrointestinal stromal tumors, GISTs) and bones (about 10%; bone sarcomas are not covered in this book). Data on sarcoma epidemiology are collected by repositories such as the RARECARE in Europe (http://www.rarecare.eu/rarecancers/rarecancers.asp) and the SEER (Surveillance, Epidemiology, and End Results Program) in the USA (https://seer.cancer.gov/). In adults, the annual incidence of soft tissue sarcomas is about 5 cases per 100,000 individuals, accounting for approximately 1% of all malignancies in this age population; in the light of this incidence, soft tissue sarcomas are classified among rare diseases (as defined by an incidence 40%, including thoracic wall, abdominal wall, mediastinum, retroperitoneum, pelvis, and viscera), or head and neck (
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