Complications from carcinoid syndrome: review of the current evidence

8 Aug 2016
José Mauricio Mota, Luana Guimarães Sousa, Rachel P Riechelmann

Patients with well-differentiated neuroendocrine tumours may develop carcinoid syndrome (CS), which is characterised by flushing, abdominal cramps, diarrhoea, and bronchospasms. In this scenario, long-term secretion of vasoactive substances—serotonin, tachynins, and others, may induce fibrogenic responses in local or distant tissues, leading to complications such as carcinoid heart disease (CHD), mesenteric and/or retroperitoneal fibrosis. Rare cases of lung/pleural fibrosis and scleroderma have also been described. Despite it not being well described yet, current evidence suggests the pathogenesis of such fibrogenic complications relies on signalling through 5-HT2B and TGF-β1. Medical management is still very limited and lacks prospective and randomised studies for definitive recommendations. Surgical procedures remain the best definitive treatment option for CHD and abdominal fibrosis. Recently, cognitive impairment has also been described as a potential consequence of CS. This review critically discusses the literature concerning the epidemiology, pathogenesis, clinical features, diagnosis, and treatment options for CS-related long-term complications.

Article metrics: 7032 views

Related Articles

Nicoletta Biglia, Valentina E Bounous, Francesco De Seta, Stefano Lello, Rossella E Nappi, Anna Maria Paoletti
Sergo Mkhitaryan, Samvel Danielyan, Lilit Sargsyan, Lusine Hakobyan, Samvel Iskanyan, Samvel Bardakchyan, Ruzanna Papyan, Jemma Arakelyan, Karmen Sahakyan, Tatevik Avagyan, Armen Tananyan, Armen Muradyan, Gevorg Tamamyan
Carolina Alves, Marcella Mesquita, Carolina Silva, Maria Soeiro, Ludhmila Hajjar, Rachel P Riechelmann
Benjamin H Mullish, Laura S Osborne, Julian R Marchesi, Julie AK McDonald