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Review
. 2022 Jan 1;9(1):38.
doi: 10.3390/children9010038.

Malignant Peripheral Nerve Sheath Tumors-A Comprehensive Review of Pathophysiology, Diagnosis, and Multidisciplinary Management

Samantha W E Knight  1 , Tristan E Knight  2   3 , Teresa Santiago  4 , Andrew J Murphy  5   6 , Abdelhafeez H Abdelhafeez  5   6
Affiliations
Review

Malignant Peripheral Nerve Sheath Tumors-A Comprehensive Review of Pathophysiology, Diagnosis, and Multidisciplinary Management

Samantha W E Knight et al. Children (Basel). .

Abstract

Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive soft tissue sarcomas (STS) with nerve sheath differentiation and a tendency to metastasize. Although occurring at an incidence of 0.001% in the general population, they are relatively common in individuals with neurofibromatosis type 1 (NF1), for whom the lifetime risk approaches 10%. The staging of MPNSTs is complicated and requires close multi-disciplinary collaboration. Their primary management is most often surgical in nature, with non-surgical modalities playing a supportive, necessary role, particularly in metastatic, invasive, or widespread disease. We, therefore, sought to provide a comprehensive review of the relevant literature describing the characteristics of these tumors, their pathophysiology and risk factors, their diagnosis, and their multi-disciplinary treatment. A close partnership between surgical and medical oncologists is therefore necessary. Advances in the molecular characterization of these tumors have also begun to allow the integration of targeted RAS/RAF/MEK/ERK pathway inhibitors into MPNST management.

Keywords: MEK inhibitor; malignant peripheral nerve sheath tumor; multi-disciplinary management; neurofibroma; neurofibromatosis type 1.

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Conflict of interest statement

The authors declare no conflict of interest. The funders had no role in the design of the study; in the collection, analyses, or interpretation of data; in the writing of the manuscript, or in the decision to publish the results.

Figures

Figure 1
Figure 1
MRI T2 images, intratumor heterogeneity.
Figure 2
Figure 2
18F-FDG PET/CT of a patient with NF1 and an MPNST of the right groin, with a maximal SUV of 7.
Figure 3
Figure 3
Histopathological features of Malignant Peripheral Nerve Sheath Tumors—MPNSTs. (A,B) Low-grade MPNST composed of a spindle cell proliferation with increased cellularity, mild cytological atypia, few mitoses (3 to 9 mitoses per 10 high-power fields), but no evidence of necrosis (Hematoxylin and Eosin, 200×). Diffuse immunoreactivity for S100-protein is noted (200×). (C,D) High-grade MPNST showing marked hypercellularity, nuclear pleomorphism, necrosis, and numerous mitoses (more than 10 mitoses per 10 high-power fields) (Hematoxylin and Eosin, 200×). Only focal S100-protein positivity is observed.
See this image and copyright information in PMC

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