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Chap 25 - NEUROECTODERMAL TUMORS: MELANOCYTIC, GLIAL, AND MENINGEAL NEOPLASMS

Published online by Cambridge University Press:  01 March 2011

By
Markku Miettinen
Edited by
Markku Miettinen
Markku Miettinen
Affiliation:
Armed Forces Institute of Pathology, Washington DC
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Summary

Although most neuroectodermal tumors are not soft tissue sarcomas, they are important in the differential diagnosis of primary soft tissue tumors. Cutaneous nevi and ordinary cutaneous melanoma are not discussed here, but the reader is referred to other dermatopathology texts such as the Armed Forces Institute of Pathology (AFIP) atlas for melanocytic tumors of the skin. Pigmented neuroectodermal tumor of infancy, extra-central nervous system gliomas, and meningothelial neoplasms relevant to the soft tissues are also discussed. Primitive neuroectodermal tumors belong to the Ewing sarcoma family of tumors and are discussed in Chapter 31.

CELLULAR BLUE NEVUS

The cellular blue nevus (CBN) is a relatively uncommon melanocytic neoplasm that can form a soft tissue mass. It should be distinguished from malignant melanoma and soft tissue sarcoma, especially clear cell sarcoma and malignant peripheral nerve sheath tumor (MPNST). Atypical and malignant forms occur, however, and the latter especially must be distinguished from the indolent variants.

Clinical Features

CBN typically forms a bluish-black cutaneous nodule of 1 cm to 2 cm that can be larger but rarely >5 cm; on occasion the nodule is ulcerated. This tumor often occurs in young adults, occasionally on a congenital basis, and less often in older adults. The average age in the largest series was 33 years, and there was a >2:1 female predominance. Other series have shown an even gender distribution, also with a median age in the early thirties.

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Modern Soft Tissue Pathology
Tumors and Non-Neoplastic Conditions
 , pp. 724 - 754
Publisher: Cambridge University Press
Print publication year: 2010

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