TriangleHEALTH PROFESSIONAL · SOURCE READING
Risk factors
Source: Childhood Soft Tissue Sarcoma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.
Source updated: April 21, 2025 · Captured 2026-09-09.
Selected source text with whitespace normalised. This Triangle page is not an NCI PDQ summary.
Context: Treatment of Peripheral Nerve Sheath Tumors / Malignant Peripheral Nerve Sheath Tumor (MPNST) NOS
MPNST can arise sporadically and in children with neurofibromatosis type 1 (NF1).[1] Among patients with NF1, a family history of MPNST is associated with an increased risk of developing early-onset MPNST.[2]
A rare case of a child with documented neurofibromatosis type 2 (NF2) and a benign neurofibroma had five recurrences of disease. During this time, the lesions progressively lost markers (such as S-100) and acquired clear-cut signs of malignant transformation to MPNST, documented by multiple markers, including the first example of NOTCH2 in this disease.[3]
Source links and citations
Publication references
Read the original reference and check its publication notices.
Preserved source evidence · Not medical advice