Work

Transformation of a gluteal type 1 plexiform neurofibroma into a high-grade malignant peripheral nerve sheath tumor: a rare case report

Year: 2026

Type: article

Abstract: Neurofibromatosis type 1 is a rare autosomal dominant disorder with heterogeneous clinical manifestations. Under certain conditions, plexiform neurofibromas may undergo malignant transformation into the more aggressive malignant peripheral nerve sheath tumor (MPNST). This case report describes a 43-year-old male with neurofibromatosis type 1 who underwent preoperative interventional vascular therapy followed by resection of a giant gluteal mass. Postoperative histopathological examination confirmed malignant transformation to MPNST. The key features of this case include a giant and rapidly progressive tumor, diagnostic challenges related to a history of trauma, tumor-related cachexia, and a multidisciplinary treatment strategy involving preoperative embolization. This case highlights the importance of regular follow-up in patients with neurofibromatosis type 1, accurate differential diagnosis, and early recognition of malignant transformation. In addition, it demonstrates the value of preoperative embolization in the management of highly vascular tumors.

Source: Frontiers in Oncology

DOI: 10.3389/fonc.2026.1786227

Authors: Hui Huang, Yang Xiao, Wei Li, Wu Wu, Yange Zhang

Institution: West China Hospital of Sichuan University

Language: English

Topic: Neurofibromatosis and Schwannoma Cases

Subfield: Neurology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $3,801