Low grade malignant peripheral nerve sheath tumor with smooth muscle differentiation Journal Article


Authors: Rodriguez, F. J.; Scheithauer, B. W.; Abell-Aleff, P. C.; Elamin, E.; Erlandson, R. A.
Article Title: Low grade malignant peripheral nerve sheath tumor with smooth muscle differentiation
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. © Springer-Verlag 2006.
Keywords: immunohistochemistry; adult; human tissue; bone neoplasms; middle aged; clinical feature; histopathology; cancer localization; case report; bone metastasis; cancer grading; microscopy, electron; neurofibromatosis 1; skin neoplasms; differential diagnosis; cell differentiation; biopsy; liver metastasis; lung metastasis; cancer size; malignant neoplastic disease; physical examination; leiomyosarcoma; rectum carcinoma; fatal outcome; peritoneum metastasis; malignant peripheral nerve sheath tumor; nerve sheath tumor; peripheral nerve tumor; nerve sheath neoplasms; neurofibroma; muscle, smooth; low back pain; s100 proteins; paraffin embedding; nerve; forearm; neurofibromatosis type 1; stomach muscle; smooth muscle differentiation
Journal Title: Acta Neuropathologica
Volume: 113
Issue: 6
ISSN: 0001-6322
Publisher: Springer  
Date Published: 2007-06-01
Start Page: 705
End Page: 709
Language: English
DOI: 10.1007/s00401-006-0171-8
PUBMED: 17119986
PROVIDER: scopus
DOI/URL:
Notes: --- - "Cited By (since 1996): 6" - "Export Date: 17 November 2011" - "CODEN: ANPTA" - "Source: Scopus"
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  1. Robert A Erlandson
    125 Erlandson