Low grade malignant peripheral nerve sheath tumor with smooth muscle differentiation

Fausto J Rodriguez, Bernd W. Scheithauer, Patrice C. Abell-Aleff, Elshami Elamin, Robert A. Erlandson

Research output: Contribution to journalArticle

Abstract

Malignant peripheral nerve sheath tumors are usually Schwann cell derived. Differentiation along mesenchymal lines is uncommon. Herein we present an example with smooth muscle differentiation occurring in the left distal forearm of a 62-year-old male with neurofibromatosis type I. Incisional biopsy of the slowly growing mass demonstrated a low-grade malignant peripheral nerve sheath tumor with strong S-100 protein immunoexpression as well as focal smooth muscle actin staining in atypical neoplastic cells. Immunostains for epithelial membrane antigen, neurofilament protein, glial fibrillary acidic protein, CD57, desmin, and myogenin were negative. The MIB-1 labeling index was 5.4%. Electron microscopy revealed arrays of microfilaments (actin myofilaments) variably associated with fusiform dense bodies, plasmalemmal micropinocytotic vesicles, and foci of external lamina (basement membrane) consistent with smooth muscle differentiation. Smooth muscle should be added to the spectrum of differentiation exhibited by malignant peripheral nerve sheath tumors. Its recognition requires exclusion of the alternative diagnosis of leiomyosarcoma.

Original languageEnglish (US)
Pages (from-to)705-709
Number of pages5
JournalActa Neuropathologica
Volume113
Issue number6
DOIs
StatePublished - Jun 2007
Externally publishedYes

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Neurilemmoma
Smooth Muscle
Myogenin
Neurofilament Proteins
Mucin-1
Neurofibromatosis 1
Desmin
Leiomyosarcoma
S100 Proteins
Myofibrils
Schwann Cells
Glial Fibrillary Acidic Protein
Actin Cytoskeleton
Basement Membrane
Forearm
Actins
Electron Microscopy
Staining and Labeling
Biopsy

Keywords

  • Immunohistochemistry
  • Malignant peripheral nerve sheath tumor
  • Nerve
  • Neurofibromatosis type 1
  • Smooth muscle differentiation

ASJC Scopus subject areas

  • Clinical Neurology
  • Pathology and Forensic Medicine
  • Neuroscience(all)

Cite this

Low grade malignant peripheral nerve sheath tumor with smooth muscle differentiation. / Rodriguez, Fausto J; Scheithauer, Bernd W.; Abell-Aleff, Patrice C.; Elamin, Elshami; Erlandson, Robert A.

In: Acta Neuropathologica, Vol. 113, No. 6, 06.2007, p. 705-709.

Research output: Contribution to journalArticle

Rodriguez, Fausto J ; Scheithauer, Bernd W. ; Abell-Aleff, Patrice C. ; Elamin, Elshami ; Erlandson, Robert A. / Low grade malignant peripheral nerve sheath tumor with smooth muscle differentiation. In: Acta Neuropathologica. 2007 ; Vol. 113, No. 6. pp. 705-709.
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