Low grade malignant peripheral nerve sheath tumor with smooth muscle differentiation

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...

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Veröffentlicht in:Acta neuropathologica 2007-06, Vol.113 (6), p.705-709
Hauptverfasser: Rodriguez, Fausto J, Scheithauer, Bernd W, Abell-Aleff, Patrice C, Elamin, Elshami, Erlandson, Robert A
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container_issue 6
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container_title Acta neuropathologica
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creator Rodriguez, Fausto J
Scheithauer, Bernd W
Abell-Aleff, Patrice C
Elamin, Elshami
Erlandson, Robert A
description 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.
doi_str_mv 10.1007/s00401-006-0171-8
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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. 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subjects 2-Methylisoborneol
Actin
Biopsy
Bone Neoplasms - secondary
CD57 antigen
Cell differentiation
Cell Differentiation - physiology
Desmin
Electron microscopy
Fatal Outcome
Forearm
Forearm - pathology
Genetic disorders
Glial fibrillary acidic protein
Humans
Immunohistochemistry
Low Back Pain - etiology
Male
Membrane proteins
Mesenchyme
Microfilaments
Microscopy, Electron
Middle Aged
Muscle, Smooth - pathology
Myogenin
Nerve Sheath Neoplasms - pathology
Neurofibromatosis
Neurofibromatosis 1 - complications
Neurofibromatosis 1 - pathology
Neurofilaments
Neurological disorders
Neuronal-glial interactions
Paraffin Embedding
Peripheral nerves
Proteins
S100 protein
S100 Proteins - metabolism
Skin Neoplasms - pathology
Smooth muscle
Tumors
title Low grade malignant peripheral nerve sheath tumor with smooth muscle differentiation
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