Malignant neuroectodermal tumor with melanocytic and rhabdomyoblastic differentiation

Munir R Tanas; Brian P Rubin

doi:10.4081/rt.2009.e26

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Malignant neuroectodermal tumor with melanocytic and rhabdomyoblastic differentiation

Journal article

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Malignant neuroectodermal tumor with melanocytic and rhabdomyoblastic differentiation

Munir R Tanas and Brian P Rubin

Rare tumors, Vol.1(2), pp.e26-81

12/28/2009

DOI: 10.4081/rt.2009.e26

PMCID: PMC2994463

PMID: 21139905

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Abstract

Malignant melanoma can metastasize widely and vary significantly in its histological appearance; it rarely presents as a deep-seated mass without an obvious primary site elsewhere. Malignant peripheral nerve sheath tumor (MPNST) is a high-grade sarcoma characterized by conventional and epithelioid subtypes. MPNST can demonstrate heterologous differentiation, usually in the form of osteosarcomatous, chondrosarcomatous, or rhabdomyosarcomatous differentiation. MPNST does not harbor true melanocytic differentiation, although epithelioid MPNST typically is diffusely S-100 protein positive and superficially can resemble malignant melanoma. An unusual intra-abdominal mass was recently encountered with features of both melanoma and conventional or epithelioid MPNST containing a fascicular spindle cell component, an epithelioid component with melanocytic differentiation, as well as a rhabdomyosarcomatous component. The terminology “malignant neuroectodermal tumor with melanocytic and rhabdomyoblastic differentiation” is proposed to describe this neoplasm, reflecting the unusual concomittant lines of differentiation as well as offering a possible rationale for nosologically challenging aspects of this neoplasm.

melanocytic differentiation

rhabdomyoblastic differentiation

malignant melanoma

malignant peripheral nerve sheath tumor

malignant neuroectodermal tumor

Case Report

Details

Title: Subtitle: Malignant neuroectodermal tumor with melanocytic and rhabdomyoblastic differentiation
Creators: Munir R Tanas - Department of Anatomic Pathology, Pathology and Laboratory Medicine Institute, The Cleveland Clinic and The Cleveland Clinic Lerner College of Medicine, Case Western Reserve University, Cleveland, OH, USA
Brian P Rubin - Department of Anatomic Pathology, Pathology and Laboratory Medicine Institute, The Cleveland Clinic and The Cleveland Clinic Lerner College of Medicine, Case Western Reserve University, Cleveland, OH, USA
Resource Type: Journal article
Publication Details: Rare tumors, Vol.1(2), pp.e26-81
DOI: 10.4081/rt.2009.e26
PMID: 21139905
PMCID: PMC2994463
NLM abbreviation: Rare Tumors
ISSN: 2036-3605
eISSN: 2036-3613
Publisher: PAGEPress Publications; Pavia, Italy
Language: English
Date published: 12/28/2009
Academic Unit: Pathology
Record Identifier: 9984046921702771

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