Pathology of the Gastrointestinal Tract

2017 Edition
| Editors: Fátima Carneiro, Paula Chaves, Arzu Ensari

Neuroendocrine Neoplasms of the Anus

  • Denis Chatelain
  • Jean-François Fléjou
Reference work entry


Neuroendocrine carcinoma; Neuroendocrine tumors; Small cell carcinoma


Neuroendocrine neoplasms very rarely occur in the anus. There is no case of anal carcinoid tumor described in the literature, but some cases have perhaps been assimilated to rectal tumors. There are only rare isolated case reports of anal neuroendocrine carcinoma, small cell neuroendocrine carcinoma, and composite neuroendocrine carcinoma – in situ squamous cell carcinoma or adenocarcinoma published in the literature.

Clinical Features

Patients complain of anal pain, rectal bleeding, and the presence of a perianal mass.
  • Site

    The neuroendocrine tumors probably arise from neuroendocrine cells residing in colorectal type mucosa or in the anal transitional zone mucosa.

  • Incidence, age, sex

    Due to the rarity of these tumors, it is difficult to assess the prevalence of this entity, the sex ratio, and the age of patients.

  • Treatment

    There is no standard treatment for this rare tumor. A few patients have...

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References and Further Reading

  1. Eberhardt, J. M., Brown, K., Lo, S., Nagda, S., & Yong, S. (2012). Extrapulmonary small cell carcinoma of the anal canal: A case report and review of the literature. Case Reports in Medicine, 2012, 341432.CrossRefGoogle Scholar
  2. Shia, J. (2010). An update on tumors of the anal canal. Archives of Pathology & Laboratory Medicine, 134, 1601–1611.Google Scholar

Copyright information

© Springer International Publishing AG 2017

Authors and Affiliations

  • Denis Chatelain
    • 1
  • Jean-François Fléjou
    • 2
  1. 1.Service d’Anatomie PathologiqueCentre Hospitalier et Universitaire du NordAmiensFrance
  2. 2.Faculté de Médecine Pierre et Marie Curie, Service d’Anatomie et Cytologie PathologiquesHôpital Saint-AntoineParisFrance