Internistische Therapie von Weichteilsarkomen

Abstract

Soft tissue sarcomas are a diagnostically and therapeutically complex disease. This is due to the pronounced heterogeneity, characterized by biologically very different histological subtypes and the resulting clinical progressions. The term encompasses more than 80 different pathologically defined tumors of the soft tissue. Every year, nearly 4000 patients are affected in Germany. The diversity and rarity of the disease make progress difficult. The average 5-year mortality is around 40 %. Therapy is stage-adapted based on size, grading, localization, involvement of lymph nodes or distant metastasis. In the localized situation, surgery is the basis of therapy. Standard is the wide resection. Depending on grading, degree of resection and localization, adjuvant radiotherapy follows in the case of so-called high-risk tumors. Certain very aggressive types (so-called small, blue, round cell sarcomas such as embryonal rhabdomyosarcomas, Ewing tumors, PNET and desmoplastic soft tissue sarcomas (desmoplastic small round cell tumors) are primarily treated with systemic treatment in a multimodality setting. Metastasis surgery is established in isolated pulmonary metastases based on retrospective studies. This article focuses on recent development in treatment of adult type soft tissue sarcoma and GIST.