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Cancer of soft tissue without mesothelioma

ICD-10 C46-C49

¹ per 100,000 persons, age-standardised according to the old European standard population

* calculated using the period method for 2017 / 2018

Age-standardised incidence rate¹3.44.3
Age-standardised mortality rate¹1.31.5
5-year prevalence6,8177,914
10-year prevalence10,95613,081
Relative 5-year survival rate*51 %61%
Relative 10-year survival rate*45 %55 %

This group of cancers includes the rare malignant tumours of the peripheral nerves, connective tissue and other soft tissue as well as the peritoneum and retroperitoneum. It also includes the rare Kaposi sarcoma that occurs on the skin (4 percent of all cancers in the group). In contrast to carcinomas, which predominantly develop from epithelial or glandular tissue, soft tissue tumours generally form from connective tissue structures. Thus, most soft tissue tumours are sarcomas.

Of the approximately 4,3650 new cases of malignant soft tissue tumours diagnosed annually, about 4 percent are diagnosed as neoplasms of the peripheral nerves and the autonomic nervous system. The largest proportion (about 75 percent) is comprised of tumours of other connective tissue: leiomyosarcomas originating in smooth muscle tissue and liposarcomas (fatty tissue tumours) are two of the most common forms in adulthood. In contrast, rhabdomyosarcomas, which originate in skeletal muscle tissue, occur almost exclusively in children and adolescents. Age-standardised incidence and mortality rates for malignant soft tissue tumours have remained fairly constant in Germany since 1999.

Age-standardised incidence and mortality rates by sex, ICD-10 C46-C49, Germany 1999 – 2016/2017, projection (incidence) through 2020, per 100,000 (old European standard population). Source: © German Centre for Cancer Registry Data, Robert Koch Institute Age-standardised incidence and mortality rates by sex, ICD-10 C46-C49, Germany 1999 – 2016/2017, projection (incidence) through 2020, per 100,000 (old European standard population)

Risk factors usually not clear

In most cases of soft tissue sarcoma, no clear cause can be found. Patients with rare hereditary tumour syndromes can develop sarcomas. The presence of one or more genetic mutations probably also influences the risk of the disease.

After radiotherapy, a sarcoma may develop in the irradiated body region in rare cases. Infection with Human herpesvirus 8 (HHV-8) can cause Kaposi sarcoma. In patients with severe immune deficiency, the Epstein-Barr virus (EBV) may also be involved in the development of soft tissue sarcoma.

Environmental toxins and chemicals can possibly contribute to the development of sarcomas. A connection between vinyl chloride exposure and angiosarcoma of the liver has been established. Chronic inflammatory processes probably also increase the risk of soft tissue sarcomas. In addition, chronic lymphoedema following breast removal can in rare cases lead to the development of angiosarcoma (Stewart-Treves syndrome). An influence of diet or other lifestyle factors, such as smoking or alcohol consumption, is not known.

Date: 24.10.2022

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