The large and giant peripheral nerve sheath tumors

INTRODUCTION: the study is devoted to an actual problem of the surgical treatment for patients with extensive and giant peripheral nerve sheath tumors of the extremities. The aim of the work is to improve the results of surgical treatment for patients with peripheral nerve sheath tumors. The article adhered to the WHO classification of 2016. One of the most difficult problems of treatment this pathology is the large and giant peripheral nerve sheath tumors, which make up from 5% to 20% of neoplasms of the peripheral nerves and are mainly divided into neurofibromas and malignant peripheral nerve sheath tumors according to histological characteristics. The defeat of several anatomical regions, the prevalence of the tumor, the gross involvement in the neoplastic process the internal organs and major vessels make it difficult to perform radical surgery with the removal of the affected tissues in a single unit.

METHODS: an analysis of comprehensive examinations and surgical treatments of 320 patients with peripheral nerve sheath tumors of the extremities who were treated at the prof. A.L. Polenov Russian National Research Institute of Physics. The classification of tumor sizes is proposed depending on the diameter of the nerve trunk affected by the neoplastic process.

RESULTS: the statistically significant dependence of the nerve trunk conduction disturbance on the histological structure and tumor size was revealed: tumors with small and medium sizes, predominantly neuromas, conduction disturbances are noted much less frequently than in cases with neurofibromas of medium and large sizes, in 75% of cases with large and giant tumor sizes (malignant peripheral nerve sheath tumors, neurofibromas) there is a complete violation of conductivity (p <0.001).

CONCLUSION: the issues of diagnostics, clinics, tactics of surgical treatment for patients with peripheral nerve tumors and relapse preventions are discussed. In cases of neurofibromas, large tumors predominate (p <0.001), in the group of malignant peripheral nerve sheath tumors, mainly giant tumors are observed (p <0.001). This statement is confirmed by a clear statistical dependence of the size of the tumor on its histological nature (p <0.001). The difficulty in developing adequate surgical tactics for extensive and giant peripheral nerve sheath tumors is due to the localization of the neoplasm on the “border of interests” of various surgical specialties (surgeons, oncologists, etc.), which requires the development of joint researches in this area.

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