Martins Fernandes Andreia, Reis Sara, Neves Catarina, Prieto David, Aldinhas Paulo, G Martins Raquel
Department of Endocrinology, Instituto Português de Oncologia de Coimbra Francisco Gentil, E.P.E., Coimbra 3000-075, Portugal.
Department of Pathology, Instituto Português de Oncologia de Coimbra Francisco Gentil, E.P.E., Coimbra 3000-075, Portugal.
JCEM Case Rep. 2025 Apr 17;3(5):luaf073. doi: 10.1210/jcemcr/luaf073. eCollection 2025 May.
Ovarian carcinoid tumors (OCTs) are rare and may cause carcinoid syndrome (CS) even in the absence of liver metastases. Carcinoid heart disease (CHD), which develops in up to 50% of patients with CS, substantially affects morbidity and mortality. While prognosis is generally favorable, maintaining clinical suspicion and early diagnosis is crucial to prevent the development of advanced heart failure or metastases. We present a case of a woman exhibiting asthenia, diarrhea, and de novo severe hypertension. Echocardiography revealed typical features of CHD. Elevated urinary levels of 5-hydroxyindoleacetic acid (5-HIAA) corroborated the diagnosis of CS. Ga-DOTANOC positron emission tomography computed tomography identified a suspicious left pelvic mass, which was subsequently confirmed by magnetic resonance imaging. Surgical resection of the tumor was performed, followed by tricuspid valve replacement surgery, confirming the diagnosis of OCT associated with CS and CHD. Postoperative follow-up revealed considerable clinical improvement, and the patient has remained free of recurrence. This case underscores the complex cardiovascular involvement in CS, with secondary hypertension as the initial symptomatic manifestation, which improved following resection of OCT. Additionally, it highlights the role of CS in the pathogenesis of severe tricuspid valve dysfunction, which ultimately required cardiac surgery.
卵巢类癌肿瘤（OCTs）较为罕见，即便没有肝转移也可能引发类癌综合征（CS）。类癌性心脏病（CHD）在高达50%的CS患者中出现，严重影响发病率和死亡率。虽然总体预后良好，但保持临床怀疑和早期诊断对于预防晚期心力衰竭或转移的发生至关重要。我们报告一例表现为乏力、腹泻和新发严重高血压的女性病例。超声心动图显示出CHD的典型特征。尿中5-羟吲哚乙酸（5-HIAA）水平升高证实了CS的诊断。镓-多他胺（Ga-DOTANOC）正电子发射断层扫描计算机断层扫描发现左盆腔有一个可疑肿块，随后磁共振成像予以证实。对肿瘤进行了手术切除，随后进行了三尖瓣置换手术，确诊为与CS和CHD相关的OCT。术后随访显示临床有显著改善，患者未再复发。该病例强调了CS中复杂的心血管受累情况，继发性高血压作为初始症状表现，在OCT切除后有所改善。此外，它突出了CS在严重三尖瓣功能障碍发病机制中的作用，最终需要进行心脏手术。
