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July 10, 2018; 91 (2 Supplement 1) ArticleOpen Access

Cutaneous neurofibromas

Current clinical and pathologic issues

Nicolas Ortonne, Pierre Wolkenstein, Jaishri O. Blakeley, Bruce Korf, Scott R. Plotkin, Vincent M. Riccardi, Douglas C. Miller, Susan Huson, Juha Peltonen, Andrew Rosenberg, Steven L. Carroll, Sharad K. Verma, Victor Mautner, Meena Upadhyaya, Anat Stemmer-Rachamimov
First published July 9, 2018, DOI: https://doi.org/10.1212/WNL.0000000000005792
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Figures

  • Figure 1
    Figure 1 Polymorphism of cutaneous neurofibromas (cNF) in a single patient

    Many different aspects of cNF can be seen in this patient, including sessile cNF (arrows), globular cNF (arrowheads), and pedunculated cNF (asterisks). NF = neurofibromas.

  • Figure 2
    Figure 2 Proposed categories of clinically observed cutaneous neurofibromas (cNF) to be considered in the prospective validation study

    (A) Nascent/latent cNF. (B) Flat cNF. (C) Sessile cNF. (D) Globular cNF. (E) Pedunculated cNF.

  • Figure 3
    Figure 3 Cutaneous neurofibromas (cNF), morphologic aspect

    The lesion is made of scattered spindle cells (arrowheads) that correspond to Schwann cells and fibroblasts, admixed with numerous capillaries and veins (asterisks) and mast cells (arrows) (hematoxylin and eosin, ×400, and inset ×25).

  • Figure 4
    Figure 4 Histopathologic features of cutaneous neurofibromas (cNF)

    cNF in which various cell types are evidenced using immunostainings. (A, B) Hematoxylin & eosin at 25 and 200 magnification show a cNF presenting as a self-limiting tumor in the skin with no peripheral capsule and in which numerous spindle cells are seen. (C–G) Immunostainings developed with DAB with antibodies to CD117 (C, ×200), S100 protein (D ×25 and E ×100), and CD34 (F ×25 and G ×100). These stains demonstrate numerous mast cells showing strong membrane expression of CD117 (C), Schwann cells displaying heterogeneous cytoplasmic and nuclear expression of S100 protein (D, E), and CD34+ fibroblasts (F,G).

  • Figure 5
    Figure 5 Unified clinicopathologic aspects of neurofibromas

    (A) Clinical pictures show the various subtypes of cutaneous neurofibromas (cNF). This includes digital photographs of nascent (visible by ultrasound, but not the naked eye), flat, sessile, globular, and pedunculated cNF. The ultrasound image is courtesy of Dominique Pichard. The red arrow tip indicates the cNF involving the epidermis and dermis. (B) Gross and histologic pictures show the various NF subtypes involving skin and subcutaneous tissue (diffuse, with or without atypia). The upper panels show a cutaneous and subcutaneous NF with diffuse growth pattern replacing the dermis (upper right picture) and the subcutaneous fat tissue (hematoxylin & eosin, ×25 and ×100). The middle panels show a neurofibroma involving the dermis and subcutaneous tissue with 2 distinct growth patterns including plexiform (arrowheads) and diffuse (hematoxylin & eosin, ×25). The lower panels show a neurofibroma located entirely in the subcutaneous tissue with a plexiform neurofibroma with intraneural growth pattern (hematoxylin & eosin, ×100).

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