Low-grade Schwann cell neoplasms with leptomeningeal dissemination: clinicopathologic and autopsy findings

Erika F. Rodriguez, Jaishri Blakeley, Shannon Langmead, Alessandro Olivi, Anthony Tufaro, Abeer Tabbarah, Gail Berkenblit, Justin M. Sacks, Scott D. Newsome, Elizabeth Montgomery, Fausto J. Rodriguez

Research output: Contribution to journalArticlepeer-review

Abstract

Leptomeningeal dissemination of low-grade Schwann cell neoplasms is an exceptionally rare occurrence and has not been well documented in the literature. We encountered 2 cases of leptomeningeal dissemination of low-grade Schwann cell neoplasms. Patient 1 was a 63-year-old woman with neurofibromatosis type 1 and a progressive low-grade malignant peripheral nerve sheath tumor developing from a diffuse/plexiform orbital neurofibroma that arose in childhood. The neoplasm demonstrated local and leptomeningeal dissemination intracranially leading to the patient's death. There was partial loss of H3K27 tri-methylation, p16 and collagen IV. Patient 2 was a 60-year-old man without neurofibromatosis type 1 who presented with cranial nerve symptoms and a disseminated neoplasm with a Schwann cell phenotype. The neoplasm stabilized after irradiation and chemotherapy, but the patient died of medical complications. Autopsy findings documented disseminated leptomeningeal disease in the intracranial and spinal compartment. H3K27M tri-methylation was preserved. The clinicopathologic and autopsy findings are studied and presented, and the literature is reviewed.

Original languageEnglish (US)
Pages (from-to)121-128
Number of pages8
JournalHuman pathology
Volume60
DOIs
StatePublished - Feb 1 2017

Keywords

  • MPNST
  • NF1
  • Neurofibroma
  • Neurofibromatosis
  • Schwann cell
  • Schwannoma

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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