TY - JOUR

T1 - Злокачественные опухоли из оболочек периферических нервов в практике детского онколога: клинический разбор

AU - Dinikina, Y.V.

N1 - Export Date: 19 February 2026; Cited By: 0; Correspondence Address: Y.V. Dinikina; St. Petersburg State University, Saint Petersburg The V.A. Almazov National Medical Research Center, Saint Petersburg, Russian Federation; email: dinikina_yuv@almazovcentre.ru

PY - 2025

Y1 - 2025

N2 - Malignant peripheral nerve sheath tumors (MPNSTs) are highly aggressive neoplasms that may develop sporadically as a result of prior irradiation or in patients with neurofibromatosis type 1 de novo or following a malignant transformation of plexiform neurofibroma. MPNSTs are rare in children, and their pathogenesis still remains unclear. However, molecular genetic tests in adult patients demonstrated a clear association between MPNSTs and pathogenic mutations in the NF1, CDKN2A/CDKN2B, PTEN, EED or SUZ12 genes. Due to the rare occurrence of these tumors, their various clinical manifestations depending on the location as well as characteristic rapid growth, they are often definitively diagnosed already at an advanced stage of the disease, which greatly increases the probability of an unfavorable outcome. Total tumor resection remains the mainstay of treatment for MPNSTs but due to the above-mentioned factors tumors are considered unresectable in 17–53% of cases. Both systemic anticancer therapy and local radiotherapy demonstrate low effectiveness, yet the inclusion of anthracyclines and ifosfamide in treatment regimens results in better outcomes. The effectiveness of targeted therapy for MPNSTs is extremely limited. Still, clinical studies on protein kinase (pazopanib), MEK (trametinib, selumetinib) and mTOR (sirolimus) inhibitors as well as on immunotherapy are under way. According to international studies, unfavorable prognostic factors include large tumor size, non-extremity site, neurofibromatosis type 1 and immunohistochemical markers of an aggressive biological behavior of the tumor. Survival rates in children with MPNSTs range from 34.6 to 65%, with an ongoing trend towards better survival since 2005. This article presents up-to-date literature data on MPNSTs in children as well as clinical cases with special attention to patients’ medical history, diagnostic approaches and anticancer therapy options. © 2025, D. Rogachev NMRCPHOI. All rights reserved.

AB - Malignant peripheral nerve sheath tumors (MPNSTs) are highly aggressive neoplasms that may develop sporadically as a result of prior irradiation or in patients with neurofibromatosis type 1 de novo or following a malignant transformation of plexiform neurofibroma. MPNSTs are rare in children, and their pathogenesis still remains unclear. However, molecular genetic tests in adult patients demonstrated a clear association between MPNSTs and pathogenic mutations in the NF1, CDKN2A/CDKN2B, PTEN, EED or SUZ12 genes. Due to the rare occurrence of these tumors, their various clinical manifestations depending on the location as well as characteristic rapid growth, they are often definitively diagnosed already at an advanced stage of the disease, which greatly increases the probability of an unfavorable outcome. Total tumor resection remains the mainstay of treatment for MPNSTs but due to the above-mentioned factors tumors are considered unresectable in 17–53% of cases. Both systemic anticancer therapy and local radiotherapy demonstrate low effectiveness, yet the inclusion of anthracyclines and ifosfamide in treatment regimens results in better outcomes. The effectiveness of targeted therapy for MPNSTs is extremely limited. Still, clinical studies on protein kinase (pazopanib), MEK (trametinib, selumetinib) and mTOR (sirolimus) inhibitors as well as on immunotherapy are under way. According to international studies, unfavorable prognostic factors include large tumor size, non-extremity site, neurofibromatosis type 1 and immunohistochemical markers of an aggressive biological behavior of the tumor. Survival rates in children with MPNSTs range from 34.6 to 65%, with an ongoing trend towards better survival since 2005. This article presents up-to-date literature data on MPNSTs in children as well as clinical cases with special attention to patients’ medical history, diagnostic approaches and anticancer therapy options. © 2025, D. Rogachev NMRCPHOI. All rights reserved.

KW - children

KW - malignant peripheral nerve sheath tumors

KW - malignant transformation

KW - neurofibromatosis type 1

KW - plexiform neurofibromas

KW - etoposide

KW - irinotecan

KW - pazopanib

KW - selumetinib

KW - sirolimus

KW - trametinib

KW - Article

KW - child

KW - childhood cancer

KW - computer assisted tomography

KW - follow up

KW - gene mutation

KW - high throughput sequencing

KW - histopathology

KW - human

KW - immunohistochemistry

KW - malignant peripheral nerve sheath tumor

KW - nuclear magnetic resonance imaging

KW - positron emission tomography-computed tomography

KW - radiation dose

KW - sarcoma

UR - https://elibrary.ru/PRLXWD

U2 - 10.24287/j.962

DO - 10.24287/j.962

M3 - статья

VL - 24

SP - 96

EP - 105

JO - ВОПРОСЫ ГЕМАТОЛОГИИ, ОНКОЛОГИИ И ИММУНОПАТОЛОГИИ В ПЕДИАТРИИ

JF - ВОПРОСЫ ГЕМАТОЛОГИИ, ОНКОЛОГИИ И ИММУНОПАТОЛОГИИ В ПЕДИАТРИИ

SN - 1726-1708

IS - 3

ER -