Simões-Pereira Joana, Wang Lai Mun, Kardos Attila, Grossman Ashley
Serviço de Endocrinologia. Instituto Português de Oncologia de Lisboa Francisco Gentil. Lisboa. Portugal.
Department of Pathology. John Radcliffe Hospital. Oxford. United Kingdom. United Kingdom.
Acta Med Port. 2017 May 31;30(5):421-425. doi: 10.20344/amp.7713.
The carcinoid syndrome is rare but it is associated with carcinoid heart disease in more than a half of the cases. Carcinoid heart disease is typically characterised by morphological and functional modifications of right-sided valves. Its aetiology is probable multifactorial but serotonin appears to play a key role in the development of this valvular disease. Unlike gastrointestinal neuroendocrine tumours, ovarian neuroendocrine tumours can present with carcinoid syndrome and carcinoid heart disease in the absence of liver metastases; such ovarian neuroendocrine tumours are a unique clinical entity. The additional burden of cardiac impairment in these patients represents a significant reduction in survival. Early recognition and surgical valve replacement before advanced heart failure is established may improve the clinical outcome. We report the case of a woman with an ovarian neuroendocrine tumour and highly symptomatic carcinoid heart disease who was submitted to tumour resection followed by valvuloplasty. She demonstrated an outstanding clinical improvement and has remained free of tumour and symptomatology.
类癌综合征罕见，但在超过半数的病例中与类癌性心脏病相关。类癌性心脏病的典型特征是右侧瓣膜的形态和功能改变。其病因可能是多因素的，但血清素似乎在这种瓣膜疾病的发展中起关键作用。与胃肠道神经内分泌肿瘤不同，卵巢神经内分泌肿瘤在无肝转移的情况下可出现类癌综合征和类癌性心脏病；此类卵巢神经内分泌肿瘤是一种独特的临床实体。这些患者心脏损害带来的额外负担导致生存率显著降低。在晚期心力衰竭形成之前尽早识别并进行外科瓣膜置换可能会改善临床结局。我们报告一例患有卵巢神经内分泌肿瘤和高度症状性类癌性心脏病的女性病例，该患者接受了肿瘤切除，随后进行了瓣膜成形术。她表现出显著的临床改善，且一直无肿瘤及症状。
