Recurrent primary cutaneous mucinous carcinoma with neuroendocrine differentiation: case report and review of the literature

Authors

  • Elodie Miquelestorena-Standley,

    Corresponding author
    1. CHRU de Tours, Laboratoire d'anatomie et cytologie pathologiques, Tours, France
    2. Université François-Rabelais de Tours, PRES Centre-Val de Loire Université, Tours, France
    • Elodie Miquelestorena-Standley

      Service d'anatomie et cytologie pathologiques, CHRU de Tours, Hôpital Trousseau, 37044 Tours Cedex 09, France

      Tel: +33 247478112

      Fax: +33 247474622

      e-mail: elodie.miquelestorena@etu.univ-tours.fr

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  • Fanny Dujardin,

    1. CHRU de Tours, Laboratoire d'anatomie et cytologie pathologiques, Tours, France
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  • Flavie Arbion,

    1. CHRU de Tours, Laboratoire d'anatomie et cytologie pathologiques, Tours, France
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  • Antoine Touzé,

    1. INRA, UR 1282, Infectiologie animale et Santé Publique, Nouzilly, France
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  • Laurent Machet,

    1. Université François-Rabelais de Tours, PRES Centre-Val de Loire Université, Tours, France
    2. CHRU de Tours, Service de dermatologie, Tours, France
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  • Stéphane Velut,

    1. Université François-Rabelais de Tours, PRES Centre-Val de Loire Université, Tours, France
    2. CHRU de Tours, Service de neurochirurgie, Tours, France
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  • Serge Guyétant

    1. CHRU de Tours, Laboratoire d'anatomie et cytologie pathologiques, Tours, France
    2. Université François-Rabelais de Tours, PRES Centre-Val de Loire Université, Tours, France
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Abstract

We report the case of a 60-year-old woman presenting with primary cutaneous mucinous carcinoma (PCMC) with neuroendocrine differentiation, revealed by neuroendocrine tumor lymph node metastasis 7 years before identification of the skin tumor. Only five cases of PCMC with neuroendocrine differentiation have been reported to date. The frequency of this neuroendocrine component may be underestimated, as it can require immunohistochemistry for identification, rather than being obvious on initial histopathologic examination. In the case presented here, the prominent neuroendocrine component displayed the morphological features of a well-differentiated neuroendocrine tumor with expression of common neuroendocrine markers, strong expression of estrogen and progesterone receptors and low Ki67 proliferation index (5%). This case shows that not all primary cutaneous neuroendocrine carcinomas are Merkel cell carcinomas (MCCs). In addition to rare primary cutaneous carcinoid tumors, the diagnosis of PCMC with neuroendocrine differentiation must be considered, particularly when confronted by a mucinous tumor or lymph node metastases of neuroendocrine carcinoma of unknown origin. On the basis of this case, identification of a neuroendocrine component in a PCMC might be an adverse prognostic indicator of recurrence or of lymph node metastasis and should support wider excision margins of the tumor.

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