A Cerebral Embolism Caused by a Malignant Peripheral Nerve Sheath Tumor in a Patient with Neurofibromatosis Type 1

Intern Med. 2024 Nov 15;63(22):3087-3091. doi: 10.2169/internalmedicine.2996-23. Epub 2024 Apr 2.

Abstract

A 31-year-old man with neurofibromatosis type 1 (NF-1) had undergone resection of a malignant peripheral nerve sheath tumor (MPNST) on the buttock 3 months previously. He subsequently underwent mechanical thrombectomy for a hyperacute left middle cerebral artery embolism. Histopathologically, the emboli comprised neurofilament-positive pleomorphic tumor cells with geographic necrosis and conspicuous mitosis and were identified as MPNST. The patient died of respiratory failure due to lung MPNST metastasis on day 15 of hospitalization. To our knowledge, this is the first report of a spontaneous cerebral embolism due to MPNST in an NF-1 patient.

Keywords: cerebral embolism; malignant peripheral nerve sheath tumor; mechanical thrombectomy; neurofibromatosis type 1.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Fatal Outcome
  • Humans
  • Intracranial Embolism* / diagnosis
  • Intracranial Embolism* / diagnostic imaging
  • Intracranial Embolism* / etiology
  • Male
  • Nerve Sheath Neoplasms* / complications
  • Nerve Sheath Neoplasms* / diagnosis
  • Neurofibromatosis 1* / complications