Ovarian carcinoid tumor with carcinoid heart syndrome: A case report and literature review.

BACKGROUND

Primary ovarian carcinoid tumors are rare neuroendocrine neoplasms comprising <0.1% of ovarian tumors and 1% of all carcinoid tumors. Up to 30% present with carcinoid syndrome, and approximately one-quarter of these develop carcinoid heart syndrome. Unlike gastrointestinal carcinoid tumors, ovarian lesions can cause carcinoid syndrome without hepatic metastases due to direct venous drainage into the systemic circulation.

CASE: A 46-year-old woman presented with abdominal discomfort, new onset hypertension, and lower extremity edema. Imaging revealed a 16.8 cm right adnexal mass with mixed solid and cystic components. Echocardiography demonstrated torrential tricuspid regurgitation related to thickened, immobile leaflets, consistent with carcinoid heart syndrome. Serum serotonin and chromogranin A were markedly elevated. She underwent exploratory laparotomy, total abdominal hysterectomy, bilateral salpingo-oophorectomy, right pelvic and para-aortic lymphadenectomy, and omental biopsy. Pathology showed a carcinoid tumor arising in a mature cystic teratoma, confined to the right ovary (FIGO stage IA). Immunohistochemistry confirmed neuroendocrine differentiation (synaptophysin, chromogranin positive). Postoperatively, chromogranin A normalized, serotonin decreased, and proBNP improved. Three months later, she underwent successful bioprosthetic tricuspid valve replacement with ongoing Cardiology follow up.

DISCUSSION: This case illustrates that ovarian carcinoid tumors may present with minimal symptoms despite significant cardiac involvement. Early cardiac evaluation is warranted in suspected carcinoid tumors to identify carcinoid heart syndrome. Overall survival is generally favorable for early stage ovarian carcinoid tumors. Long-term cardiac prognosis is determined by right ventricular function and presence of residual tumor after resection. Management requires individualized and multidisciplinary surgical planning, balancing tumor resection with timely cardiac intervention.