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Neurothekeoma Versus Melanoma

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Pathology of Challenging Melanocytic Neoplasms

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Abstract

The clinicopathologic spectrum of neurothekeoma was elaborated upon relatively recently. As our understanding of its morphologic variability expands, so too does its differential diagnosis. Effective dissection of the differential diagnosis requires recognition of both the classic and variant morphologies, a challenging feat in itself. The remarkable morphologic plasticity exhibited by this peculiar neoplasm lends itself to confusion with various benign and malignant melanocytic and non-melanocytic neoplasms. The collision of neurothekeoma with the notoriously protean melanoma culminates in a perfect storm of diagnostic havoc. Further complicating the diagnosis is a lack of a specific immunoprofile for neurothekeoma, reflecting its uncertain origin and differentiation. Fortunately, the presence or absence of expression of certain markers, such as S100 protein, serves as an invaluable discriminatory tool. As no confirmatory molecular test has surfaced for neurothekeoma, the diagnosis rests on the integration of clinical, histopathologic, and immunohistochemical findings, which are subject to change over time as our understanding of this tumor evolves.

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Notes

  1. 1.

    Other terms that may have been lumped together with “myxoid neurothekeoma” include myxoid tumor of nerve sheath, perineurial myxoma, Pacinian neurofibroma, and bizarre cutaneous neurofibroma [8, 15].

  2. 2.

    The term “cellular neurothekeoma” was introduced by Barnhill and Mihm [15] as a distinctive subtype of neurothekeoma, but currently, it is best understood as a morphologic pattern on a spectrum of cellular ↔ myxoid rather than as an actual subtype.

  3. 3.

    S100A6 must not be confused with S100 protein, with which it is in the same family [16].

  4. 4.

    SMA-positive cells in desmoplastic melanoma are likely non-lesional cells (reactive stromal myofibroblasts) [58].

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Authors and Affiliations

  1. University of Chicago Medicine, 5841 S. Maryland Ave., MC 5067, L502, Chicago, IL, 60637, USA

    Kristen M. Paral & Christopher R. Shea M.D.

  2. CellNEtix Pathology & Laboratories, 1124 Columbia St., Suite 200, Seattle, WA, 98117, USA

    Jon A. Reed

  3. MD Anderson Cancer Center, University of Houston, 1515 Holcombe Blvd., Unit 85, Houston, TX, 77030, USA

    Victor G. Prieto

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  1. Kristen M. Paral
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  2. Jon A. Reed
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  4. Christopher R. Shea M.D.
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Correspondence to Christopher R. Shea M.D. .

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Editors and Affiliations

  1. Section of Dermatology, University of Chicago Medicine, Chicago, Illinois, USA

    Christopher R. Shea

  2. Department of Pathology, Baylor College of Medicine, Houston, Texas, USA

    Jon A. Reed

  3. Department of Pathology, University of Texas, MD Anderson Cancer, Houston, Texas, USA

    Victor G. Prieto

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Paral, K.M., Reed, J.A., Prieto, V.G., Shea, C.R. (2015). Neurothekeoma Versus Melanoma. In: Shea, C., Reed, J., Prieto, V. (eds) Pathology of Challenging Melanocytic Neoplasms. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-1444-9_12

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