Mota José Mauricio, Sousa Luana Guimarães, Riechelmann Rachel P
Instituto do Câncer do Estado de São Paulo, University of São Paulo, 01246-000 Brazil.
Ecancermedicalscience. 2016 Aug 8;10:662. doi: 10.3332/ecancer.2016.662. eCollection 2016.
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.
高分化神经内分泌肿瘤患者可能会出现类癌综合征（CS），其特征为潮红、腹部绞痛、腹泻和支气管痉挛。在这种情况下，血管活性物质（血清素、速激肽等）的长期分泌可能会在局部或远处组织中诱导纤维化反应，导致类癌性心脏病（CHD）、肠系膜和/或腹膜后纤维化等并发症。也有罕见的肺/胸膜纤维化和硬皮病病例报道。尽管目前对此描述不多，但现有证据表明，此类纤维化并发症的发病机制依赖于5-HT2B和TGF-β1信号通路。药物治疗仍然非常有限，且缺乏前瞻性和随机研究以给出明确建议。手术仍然是治疗CHD和腹部纤维化的最佳确定性治疗选择。最近，认知障碍也被描述为CS的一个潜在后果。本综述批判性地讨论了有关CS相关长期并发症的流行病学、发病机制、临床特征、诊断和治疗选择的文献。
