Radiation-induced intradural malignant peripheral nerve sheath tumor of the cauda equina with diffuse leptomeningeal metastasis: Case report

Darryl Lau, Dominic H. Moon, Paul Park, Shawn Hervey-Jumper, Paul E. McKeever, Daniel A. Orringer

Research output: Contribution to journalArticle

11 Scopus citations

Abstract

Malignant peripheral nerve sheath tumors (MPNSTs) are rare, affecting only a small portion of the general population. In many cases, MPNSTs occur in association with neurofibromatosis Type 1 and at times arise secondary to previous radiation therapy (RT). These tumors can be found essentially anywhere a peripheral nerve is present, but they rarely originate primarily from the spinal nerve or cauda equina and cause leptomeningeal spread. This report describes the treatment course of a 43-year-old man with a history of testicular seminoma treated with RT a decade before, who was found to have a large sacral MPNST. The patient underwent complete sacrectomy for gross-total resection. Despite this effort, he was eventually found to have metastatic lesions throughout the spine and brain, ultimately resulting in acute hydrocephalus and death. Biopsy results of these metastatic lesions proved to be characteristic of his original MPNST. The literature is also reviewed and the diagnostic modalities, management strategies, and prognosis of MPNST are discussed.

Original languageEnglish (US)
Pages (from-to)719-726
Number of pages8
JournalJournal of Neurosurgery: Spine
Volume21
Issue number5
DOIs
StatePublished - Nov 1 2014

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Keywords

  • Cauda equina
  • Malignant peripheral nerve sheath tumor
  • Oncology
  • Radiation therapy

ASJC Scopus subject areas

  • Surgery
  • Neurology
  • Clinical Neurology

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