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Related Experiment Video

Updated: Jun 6, 2026

Genetic Profiling and Genome-Scale Dropout Screening to Identify Therapeutic Targets in Mouse Models of Malignant Peripheral Nerve Sheath Tumor

Genetic Profiling and Genome-Scale Dropout Screening to Identify Therapeutic Targets in Mouse Models of Malignant Peripheral Nerve Sheath Tumor

Published on: August 25, 2023

Segmental neurofibromatosis and malignancy.

Julie D Dang¹, Philip R Cohen

¹Department of Internal Medicine-Pediatrics, University of Texas-Houston Medical School, Houston, Texas, USA.

|December 9, 2010

Summary

This summary is machine-generated.

Segmental neurofibromatosis, a localized form of neurofibromatosis type I, rarely involves cancer. Malignancies in these patients, often neural crest-derived, may occur at rates similar to general neurofibromatosis type I.

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Genetic Profiling and Genome-Scale Dropout Screening to Identify Therapeutic Targets in Mouse Models of Malignant Peripheral Nerve Sheath Tumor

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An Orthotopic Sciatic Nerve Xenograft for Neurofibromatosis Type 1 Neurofibromas

An Orthotopic Sciatic Nerve Xenograft for Neurofibromatosis Type 1 Neurofibromas

Published on: October 10, 2025

Area of Science:

Oncology
Genetics
Dermatology

Background:

Segmental neurofibromatosis (SNF) is a rare variant of neurofibromatosis type I (NF1).
SNF presents with localized neurofibromas and café-au-lait macules in one body segment.
While NF1 increases malignancy risk, cancer is infrequent in SNF.

Purpose of the Study:

To review and summarize reported cases of malignancy in patients with SNF.
To characterize the types of cancers and patient demographics in SNF with malignancy.

Main Methods:

Systematic review of published literature on SNF and associated malignancies.
Data extraction and synthesis of patient characteristics and cancer types.

Main Results:

Ten cases of SNF with malignancy were identified (6 women, 4 men).
Cancers included malignant peripheral nerve sheath tumor (3), malignant melanoma (2), breast (1), colon (1), gastric (1), lung (1), and Hodgkin lymphoma (1).
Malignant peripheral nerve sheath tumors and malignant melanomas, both neural crest-derived, were most common.

Conclusions:

Malignancy in SNF is uncommon but occurs.
The spectrum of cancers in SNF includes neural crest-derived tumors.
The cancer incidence in SNF may approach that seen in NF1.