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Updated: Feb 8, 2026

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Primary Cutaneous Carcinomas with Neuroendocrine Differentiation.

Thibault Kervarrec1, Nicolas Macagno2, Serge Guyétant3

  • 1Department of Pathology, Université de Tours, Centre Hospitalier Universitaire de Tours, Tours, France; Caraderm Network, France; Biologie des infections à polyomavirus" team, UMR INRA ISP 1282, Université de Tours, Tours, France.

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Summary

This review covers skin tumors with a neuroendocrine phenotype, including Merkel cell carcinoma. It details their clinical, morphologic, immunohistochemical, and genetic aspects for diagnosis and management.

Keywords:
BetacateninMerkelNeuroendocrineRosetteSkinSweat glandWnt

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Area of Science:

  • Dermatology
  • Oncology
  • Pathology

Background:

  • Neuroendocrine neoplasms occur in various organs, categorized by differentiation.
  • Skin neuroendocrine tumors present unique diagnostic challenges.

Purpose of the Study:

  • To summarize key features of skin neuroendocrine tumors.
  • To focus on Merkel cell carcinoma and related entities.
  • To aid in diagnosis, management, and differential diagnosis.

Main Methods:

  • Review of clinical, morphologic, immunohistochemical, and genetic data.
  • Focus on specific entities: Merkel cell carcinoma, Wnt/betacatenin rosette-forming carcinoma, endocrine mucin-producing sweat gland carcinoma, and sweat gland carcinoma with neuroendocrine differentiation.

Main Results:

  • Detailed characterization of selected skin neuroendocrine tumors.
  • Emphasis on diagnostic criteria and management strategies.
  • Discussion of differential diagnoses for accurate identification.

Conclusions:

  • Accurate diagnosis of skin neuroendocrine tumors requires integrating multiple features.
  • Understanding these entities is crucial for effective patient management.
  • Further research may refine diagnostic and therapeutic approaches.