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Abstract
Introduction. The present literature review is devoted to a detailed description of the morphological features, histological diversity, and "mosaic" structure of a neoplasm that is rarely encountered in practice – a malignant peripheral nerve sheath tumor with divergent differentiation. The analysis of the data allowed the identification of prognostic factors and characteristics of the course of this pathology.
Materials and Methods. For the period December 2023 – March 2024, a search and analysis of literature sources was carried out using foreign printed publications, database search engines and electronic libraries PubMed, Medline, Scopus, National Library of Medicine and eLibrary. A total of 96 publications were found. . The review included 60 references. The malignant peripheral nerve sheath tumor (MPNST) is the fifth most common soft tissue sarcoma, after undifferentiated sarcoma, leiomyosarcoma, liposarcoma and synovial sarcoma, and accounts for 3/4 of soft tissue sarcoma incidence. Unlike the vast majority of these malignant neoplasms of mesenchymal origin, MPNSTs are tumors of neuroectodermal nature, characterized by high tissue plasticity, accompanied by multilinear differentiation and manifested by morphological and/or phenotypic heterogeneity. This special feature is realized in a separate subtype of MPNST – MPNST with divergent differentiation, whose uniqueness against the background of a rather large subpopulation of sarcomas with combined morphology lies in the wide range of divergent differentiation, realized by a wide tissue diversity and differences in the biological potential of the divergent component. The review presents clinical cases of MPNST with different, including multiple, divergent components described in the literature. This paper identifies factors associated with the unfavourable course and prognosis of these tumors, shows the variability of the histotypic spectrum of the divergent component, and describes the characteristics of the two most aggressive MPNST with a malignant divergent component – malignant triton tumors and MPNST with divergent angiosarcomatous differentiation.
Conclusion. The problem of tumors with combined morphology, which includes MPNST with divergent differentiation, is largely due to the difficulty of morphological verification, which often leads to diagnostic error. An integrated approach to morphological diagnosis, using immunohistochemistry and, in some cases, molecular genetic methods, is an indispensable rule for the pathologist in daily practice, allowing such a process to be identified in less time and with as much accuracy as possible.
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