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Malignant peripheral nerve sheath tumor: an unusual cause of intussusception

Dana A Telem1, David Pertsemlidis

  • 1The Mount Sinai Hospital, Department of Surgery, One Gustave Levy Plaza Box 1259, New York, NY 10029, USA. dana.telem@mssm.edu

Journal of Gastrointestinal Surgery : Official Journal of the Society for Surgery of the Alimentary Tract
|November 7, 2007
PubMed

Insights

Malignant peripheral nerve sheath tumors (MPNST) are rare in the gastrointestinal tract. This paper details the first US case of a small intestine MPNST presenting as intussusception.

Area of Science:

  • Oncology
  • Gastroenterology
  • Surgical Pathology

Background:

  • Malignant peripheral nerve sheath tumors (MPNST) are aggressive neoplasms originating from peripheral nerves or exhibiting nerve sheath differentiation.
  • MPNSTs predominantly occur in the trunk, extremities, or head and neck, with rare occurrences reported in the gastrointestinal tract.
  • Fewer than 14 cases of gastrointestinal MPNST have been documented, with only two reported in the small intestine, one being recurrent.

Observation:

  • This report describes the first case of MPNST in the United States arising within the small intestine.
  • The presented case involved an MPNST that manifested clinically as intussusception.
  • Intussusception is a condition where one part of the intestine slides into another, often causing obstruction.

Findings:

  • The study identifies a novel presentation of MPNST in the small intestine.
  • This case highlights intussusception as a potential clinical sign of small intestinal MPNST.
  • The tumor's origin and characteristics are detailed, contributing to the understanding of rare MPNST presentations.

Implications:

  • This case expands the known spectrum of MPNST origins and clinical manifestations.
  • Early recognition of intussusception in the context of potential MPNST is crucial for timely diagnosis and treatment.
  • Further research into rare gastrointestinal MPNSTs is warranted to improve diagnostic and therapeutic strategies.

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