Soft tissue sarcomas associated with neurofibromatosis: treatment experience

Abstract

Soft tissue sarcomas are a rare and heterogeneous group of solid tumors originating from the mesenchyme and accounting for only 1 % of all malignant neoplasms in adults. The modern clinical and morphological World Health Organization of soft tissue sarcomas classification includes more than 100 forms of these tumors. Neurofibromatosis is a group of hereditary, autosomal dominant diseases characterized by the development of multiple tumor formations, often benign. Currently, there are 3 types: neutrophibromatosis type I, neurofibromatosis type II and schwannomatosis. The estimated risk of developing sarcoma in patients with neurofibromatosis type 1 at age 30 years is 25.1 %, and by age 50 years it is 38.8 %. Treatment of soft tissue sarcomas associated with neurofibromatosis fully complies with the standard recommendations of National Comprehensive Cancer Network, NCCN (NCCN), American Society of Clinical Oncology (ASCO), European Society for Medical Oncology (ESMO) and RUSSCO and does not have its own peculiarities. Over the past 15 years, at the A.F. Tsyb Medical Radiological Research Center – branch of the National Medical Research Radiological Center, more than 190 patients with locally advanced, high-grade soft tissue sarcomas have been treated, including five patients with sarcomas associated with neurofibromatosis. The article presents clinical cases of treatment of soft tissue sarcomas associated with neurofibromatosis.