Malignant transformation in a hybrid schwannoma/perineurioma: addition to the spectrum of a malignant peripheral nerve sheath tumor

Indian J Pathol Microbiol. 2011 Oct-Dec;54(4):825-8. doi: 10.4103/0377-4929.91542.

Abstract

Benign nerve sheath tumors include schwannomas, neurofibromas and perineuriomas. The malignant counterpart of a nerve sheath tumor is designated as a malignant peripheral nerve sheath tumor (MPNST). Lately, benign nerve sheath tumors comprising more than one component have been described, including hybrid schwannomas/perineuriomas. However, malignant transformation in a hybrid schwannoma/perineurioma has not been documented so far. Herein, we present a rare case of a young adult male who presented with a soft tissue mass in his right thigh that was excised elsewhere and submitted to us for histopathological review. One of the tissue sections displayed histopathological features of a hybrid schwannoma/perineurioma, including alternate arrangement of benign schwann and perineurial cells, reinforced with S100-P and epithelial membrane antigen positivity, respectively, along with low MIB1 and negative p53 immunostaining. The other two tissue sections showed a spindly sarcomatous tumor that was immunohistochemically positive for S100-P, CD34, p53 and exhibited high MIB1 (30-40%). Diagnosis of a MPNST arising in a hybrid schwannoma/perineurioma was made. This unusual case forms yet another addition to the spectrum of a MPNST.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Antigens, CD34 / analysis
  • Histocytochemistry
  • Humans
  • Immunohistochemistry
  • Male
  • Microscopy
  • Nerve Sheath Neoplasms / complications*
  • Nerve Sheath Neoplasms / diagnosis*
  • Nerve Sheath Neoplasms / pathology*
  • Nerve Sheath Neoplasms / surgery
  • Neurilemmoma / complications*
  • Neurilemmoma / pathology*
  • Neurilemmoma / surgery
  • S100 Proteins / analysis
  • Thigh / pathology
  • Thigh / surgery
  • Tumor Suppressor Protein p53 / analysis

Substances

  • Antigens, CD34
  • S100 Proteins
  • Tumor Suppressor Protein p53