title: Radiation-induced intradural malignant peripheral nerve sheath tumor of the cauda equina with diffuse leptomeningeal metastasis.
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.
authors.affiliation: Department of Neurological Surgery, University of California, San Francisco, California;
authors.forename: Darryl
authors.initials: D
authors.lastname: Lau
authors.forename: Dominic H
authors.initials: DH
authors.lastname: Moon
authors.forename: Paul
authors.initials: P
authors.lastname: Park
authors.forename: Shawn
authors.initials: S
authors.lastname: Hervey-Jumper
authors.forename: Paul E
authors.initials: PE
authors.lastname: McKeever
authors.forename: Daniel A
authors.initials: DA
authors.lastname: Orringer
mesh_terms: D000328:Adult; D001706:Biopsy; D001932:Brain Neoplasms / Q000556:secondary* / Q000601:surgery*; D002420:Cauda Equina / Q000528:radiation effects*; D003131:Combined Modality Therapy; D003287:Contrast Media; D017679:Cryotherapy; D017809:Fatal Outcome; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008577:Meningeal Neoplasms / Q000556:secondary* / Q000601:surgery*; D009373:Neoplasms, Germ Cell and Embryonal / Q000532:radiotherapy; D009381:Neoplasms, Radiation-Induced / Q000473:pathology / Q000628:therapy*; D018317:Nerve Sheath Neoplasms / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D019635:Neurosurgical Procedures; D012447:Sacrum; D013736:Testicular Neoplasms / Q000532:radiotherapy; D014057:Tomography, X-Ray Computed
chemical_list: D003287:Contrast Media
keywords: MPNST = malignant peripheral nerve sheath tumor; RT = radiation therapy; cauda equina; malignant peripheral nerve sheath tumor; oncology; radiation therapy