Revista da Sociedade Portuguesa de Endocrinologia Diabetes e Metabolismo

Online first

Revista Portuguesa de Endocrinologia, Diabetes e Metabolismo - Online first: 2022-08-18
Review article

Carcinoid syndrome: a review

Diana Borges Duarte, Raquel G. Martins

Abstract

Abstract Carcinoid syndrome (CS) is a debilitating disease caused by the production of a variety of biologically active substances by functional neuroendocrine neoplasms (NENs). While the reported frequency of CS among NEN patients has been inconsistent, the negative impact on patient quality of life is clearly established. The hallmark symptoms of CS are flushing, diarrhoea, abdominal pain and valvular heart disease; other signs and symptoms can include wheezing, telangiectasias, pellagra, and the complications of mesenteric fibrosis, including bowel obstruction and ischemia. These symptoms are mediated by the release of serotonin, histamine, kallikrein, prostaglandins and tachykinins. There have been advances in many aspects of the carcinoid-syndrome all of which are reviewed in this article, including new methods to establish diagnosis, an increased understanding of natural history and pathogenesis and important new approaches to its treatment.

Portuguese abstract

A síndrome carcinóide (SC) é uma doença debilitante causada pela produção de múltiplas substâncias biológicas ativas por neoplasias neuroendócrinas (NENs). Apesar da variabilidade na frequência reportada, o impacto negativo na qualidade de vida dos pacientes com NENs está bem estabelecido. Os sintomas clássicos da SC são o flushing, a diarreia, a dor abdominal e a doença valvular cardíaca; outros sinais e sintomas incluem o broncoespasmo, telangiectasias, pelagra e as complicações da fibrose mesentérica como obstrução e isquemia intestinal. Estes sintomas são mediados pela libertação de serotonina, histamina, calicreína, prostaglandinas e taquicininas. A síndrome carcinóide tem sido alvo de vários avanços, incluindo novos métodos de diagnóstico, um aperfeiçoamento na compreensão da sua história natural e patogénese, e novas abordagens terapêuticas, que serão abordados neste artigo.


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