8241/3 Carcinoid tumor, argentaffin, malignant



Definitions

Colon and rectum
ICD-O-3 topography code: C18-C20
ICD10: C18-C20
           

This is a neuroendocrine tumour (NET) characterized by the secretion of serotonin by enterochromaffin-like (ECL) cells.





Small intestine
ICD-O-3 topography code: C17
ICD10: C17
           

This is a neuroendocrine tumour (NET) characterized by the secretion of serotonin by enterochromaffin-like (ECL) cells. Serotonin-producing carcinoids are unusual in the upper small intestine. In the duodenum they only exceptionally give rise to a clinical carcinoid syndrome 1
 
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



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Stomach
ICD-O-3 topography code: C16
ICD10: C16
           

Most neuroendocrine neoplasms of the stomach are NETs ? well-differentiated, nonfunctioning enterochromaffin-like (ECL) cell carcinoids (ECL cell NETs) that mainly produce histamine and histidine decarboxylase. However, minor cell subpopulations expressing serotonin, ghrelin, gastrin, somatostatin, pancreatic polypeptide (PP), or α-human chorionic gonadotrophin (α-hCG) have been detected
2
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Papotti M, Cassoni P, Volante M, Deghenghi R, Muccioli G, Ghigo E (2001)
Ghrelin-producing endocrine tumors of the stomach and intestine.
J Clin Endocrinol Metab 86: 5052-9



3
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Rindi G, Luinetti O, Cornaggia M, Capella C, Solcia E (1993)
Three subtypes of gastric argyrophil carcinoid and the gastric neuroendocrine carcinoma: a clinicopathologic study.
Gastroenterology 104: 994-1006



4
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Rindi G, Savio A, Torsello A, Zoli M, Locatelli V, Cocchi D, Paolotti D, Solcia E (2002)
Ghrelin expression in gut endocrine growths.
Histochem Cell Biol 117: 521-5



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EC cell, serotonin-producing NETs of the stomach is rare. This tumour may arise in both the corpus?fundus and antrum of the stomach. It is occasionally found in association with a carcinoid syndrome
5
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CHRISTODOULOPOULOS JB, KLOTZ AP (1961)
Carcinoid syndrome with primary carcinoid tumor of the stomach.
Gastroenterology 40: 429-40



. Its histopathology is similar to that of the ileal NETs. The tumour cells are argentaffin, intensely argyrophilic and immunoreactive for chromogranin A, synaptophysin and anti-serotonin antibodies. The presence of EC cells is confirmed via electron microscopic detection of characteristic pleomorphic, intensely osmiophilic granules similar to those of normal gastric EC cells6
 
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



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Pancreas
ICD-O-3 topography code: C25
ICD10: C25
           

A serotonin-secreting tumour is a usually malignant neoplasm of the pancreas that may become functionally active (syndromic) only after metastasizing to the liver. It produces the clinical symptoms of the carcinoid syndrome
7
 
Ronald A. DeLellis, Ricardo V. Lloyd, Philipp U. Heitz, Charis Eng
World Health Organization Classification of Tumours. Pathology and Genetics of Tumors of Endocrine Organs
IARC Press: Lyon 2004



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The actual incidence of serotonin secreting tumours of the pancreas is not known. The tumours are frequently malignant and metastasize to lymph nodes and liver. When a patient develops liver or retroperitoneal metastases, the carcinoid syndrome may occur with typical flushing, diarrhoea and bronchoconstriction, accompanied by elevated plasma 5-HT levels and urinary 5-HIAA excretion
8
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Feldman JM (1987)
Carcinoid tumors and syndrome.
Semin Oncol 14: 237-46



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All syndromic examples have liver metastases and therefore the ultimate prognosis is poor; however, the disease may progress very slowly.





Region of the ampulla of Vater
ICD-O-3 topography code: C24.1
ICD10: C24.1
           

This is a neuroendocrine tumour (NET) characterized by the secretion of serotonin by enterochromaffin-like (ECL) cells.