{"id":5268,"date":"2024-12-11T17:29:08","date_gmt":"2024-12-11T16:29:08","guid":{"rendered":"https:\/\/irccs.com\/patologie\/sarcomas-and-rare-tumors\/"},"modified":"2026-07-17T16:16:27","modified_gmt":"2026-07-17T14:16:27","slug":"sarcomas-and-rare-tumors","status":"publish","type":"pathology","link":"https:\/\/inoc.it\/en\/pathology\/sarcomas-and-rare-tumors\/","title":{"rendered":"Sarcomas and Rare Tumors"},"content":{"rendered":"\n

<\/span>Pathology<\/h2>\n\n\n\n

Soft tissue sarcomas<\/strong> are rare tumors arising from the malignant transformation of cells within the so called soft tissues<\/strong>, including muscle, connective tissue, blood and lymphatic vessels, nerves, ligaments, and adipose tissue<\/strong>. More than 50 histological subtypes<\/strong> have been identified, each with distinct biological and clinical features.<\/p>\n\n\n\n

These tumors can develop in virtually any anatomical site. In more than 50% of cases<\/strong>, they occur in the limbs or superficial trunk<\/strong>, while the remainder may arise in the head and neck, internal organs (such as the uterus), or the retroperitoneum<\/strong>, a deep abdominal region rich in fat and connective tissue.<\/p>\n\n\n\n

Some sarcoma subtypes are more typical of pediatric age<\/strong>, such as rhabdomyosarcoma<\/strong>, which affects approximately 4\u20137 children per million under 15 years of age annually<\/strong>. Overall, sarcomas show three incidence peaks: childhood, young adulthood (18\u201330 years), and over 65 years<\/strong>.<\/p>\n\n\n\n

The Numbers in Italy<\/h3>\n\n\n\n

Soft tissue sarcomas are rare diseases<\/strong>, with an incidence of approximately 5 cases per 100,000 people per year<\/strong>, representing about 1% of all adult cancers<\/strong>. According to the Italian Association of Cancer Registries <\/strong>(AIRTUM)<\/a>, approximately 2,230 new diagnoses<\/strong> of sarcomas and rare cancers were recorded in Italy in 2024, including 1,289 in men and 941 in women<\/strong>.
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<\/span>Symptoms<\/h2>\n\n\n\n

When sarcomas affect the limbs, they typically present as a mass or swelling that progressively increases in size over weeks or months<\/strong>. They are usually painless, except when in close contact with nerves or sensitive structures.<\/p>\n\n\n\n

When located in the abdomen or retroperitoneum<\/strong>, symptoms are often non-specific and may include weight loss, reduced appetite, fatigue, or abdominal swelling<\/strong>. In some cases, the tumor may cause intestinal obstruction, gastrointestinal bleeding, or lower limb edema due to vascular compression<\/strong>.<\/p>\n\n\n\n

The lack of specific symptoms often leads to delayed diagnosis<\/strong> in abdominal sarcomas compared with limb localized disease.<\/p>\n\n\n\n

<\/span>Risk Factors<\/h2>\n\n\n\n

Given the heterogeneity of soft tissue sarcomas, there are no universal risk factors. However, several conditions may increase risk:<\/p>\n\n\n\n