Primary malignant peripheral nerve sheath tumor of the lung in a young child without neurofibromatosis type 1

Pediatr Blood Cancer. 2006 Oct 15;47(5):636-8. doi: 10.1002/pbc.20787.

Abstract

Malignant peripheral nerve sheath tumors (MPNST) are uncommon in children and almost half of the cases occur in patients with neurofibromatosis 1 (NF1). We report a child with a primary MPNST of the lung without NF1. MPNST of the lung has similar clinical and radiologic characteristics as pleuropulmonary blastoma. We suggest to include MPNST of the lung in the differential diagnosis of intrapulmonary masses in children.

Publication types

  • Case Reports

MeSH terms

  • Child
  • Diagnosis, Differential
  • Humans
  • Lung Neoplasms / diagnosis
  • Lung Neoplasms / pathology*
  • Lung Neoplasms / therapy
  • Magnetic Resonance Imaging / methods
  • Male
  • Neoplasms, Multiple Primary / diagnosis
  • Neoplasms, Multiple Primary / pathology*
  • Neoplasms, Multiple Primary / therapy
  • Nerve Sheath Neoplasms / diagnosis
  • Nerve Sheath Neoplasms / pathology*
  • Nerve Sheath Neoplasms / therapy
  • Neurofibromatosis 1 / diagnosis
  • Predictive Value of Tests
  • Pulmonary Blastoma / diagnosis
  • Pulmonary Blastoma / pathology*
  • Pulmonary Blastoma / therapy
  • Tomography, X-Ray Computed / methods