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WHO Classification of Tumours
Enterochromaffin cell (EC), serotonin-producing neuroendocrine tumour (NET)
Pancreas


Definition

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
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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

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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.

Commentary: Neuroendocrine tumours of the digestive system