PRIMITIVE NEUROECTODERMAL TUMORS IN THE STRUCTURE OF SOFT TISSUE SARCOMAS

Trofimenko Yury Gennadievich, Zavyalov Alexander Alexandrovich, Stepanyants Nikolay Georgievich, Panasenko Valeriy Alexandrovich

Primitive neuroectodermal tumors are characterized by a low frequency of spread, a high degree of malignancy, rapid progression, a high frequency of relapses and metastasis. Impaired development, differentiation and migration of neural crest cells are the cause of a whole class of syndromes and lead to oncogenesis in various organs and tissues, mainly in male children and adolescents. According to the World Health Organization, the peripheral type of PNEO (pPNEO), classical Ewing's sarcoma and extra-skeletal Ewing's sarcoma make up the family of Ewing's sarcoma tumors. These tumors are determined by specific chromosomal abnormalities, primarily translocation t(11,22) (q24,12) and are associated with 85% of tumors and leads to the fusion of the EWS-FLI-1 gene. The actual prevalence of PNEO is difficult to assess due to the rarity of the tumor and the difficulties of morphological assessment. Most of the publications of the PEO study are clinical descriptions of single tumors of different localization and among patients of different age groups, and clear diagnostic criteria have been established only recently. These types of tumors can be distinguished based on morphological examination and expression of neural markers. The strategies of combined treatment, consisting of surgery, chemotherapy and radiation, remain controversial, which determines the future prospects of research.

Keywords:Ewing's sarcoma, oncogenesis, biopsy, children, adolescents, peripheral primitive neuroectodermal tumor