Primary Ovarian Neuroendocrine Neoplasm With Carcinoid Syndrome and Carcinoid Heart Disease
3
Issued Date
2026-06-01
Resource Type
ISSN
15245012
Scopus ID
2-s2.0-105042289659
Journal Title
Ochsner Journal
Volume
26
Issue
2
Start Page
173
End Page
178
Rights Holder(s)
SCOPUS
Bibliographic Citation
Ochsner Journal Vol.26 No.2 (2026) , 173-178
Suggested Citation
Thammakosol K., Vathesatogkit P., Leelasithorn S., Sriphrapradang C. Primary Ovarian Neuroendocrine Neoplasm With Carcinoid Syndrome and Carcinoid Heart Disease. Ochsner Journal Vol.26 No.2 (2026) , 173-178. 178. doi:10.31486/toj.25.0101 Retrieved from: https://repository.li.mahidol.ac.th/handle/123456789/117527
Title
Primary Ovarian Neuroendocrine Neoplasm With Carcinoid Syndrome and Carcinoid Heart Disease
Author's Affiliation
Corresponding Author(s)
Other Contributor(s)
Abstract
Background: Neuroendocrine neoplasms commonly arise in the midgut, lungs, or pancreas, while primary ovarian neuroendocrine neoplasms are rare and can present with carcinoid syndrome and carcinoid heart disease even in the absence of hepatic metastases. Case Report: A 70-year-old female with coronary artery disease presented with 3 months of dyspnea, leg edema, diarrhea, and facial flushing. Examination suggested right-sided heart failure with a palpable lower abdominal mass. Her 24-hour urinary 5-hydroxyindoleacetic acid level was markedly elevated (106.0 mg), and serum chromogranin A was also elevated (468.6 ng/mL). Echocardiography showed severe tricuspid and pulmonic regurgitation from thickened, immobile leaflets. Imaging identified a DOTATATE-avid left adnexal mass on Gallium-68 DOTATATE positron emission tomography/computed tomography, consistent with primary ovarian neuroendocrine neoplasm. Preoperative management included long-acting octreotide, niacin, and guideline-directed heart failure therapy, followed by tumor resection. Histopathology confirmed insular-type ovarian neuroendocrine neoplasm without invasion or metastasis. Postoperatively, the patient improved clinically, and her biochemical markers normalized. Conclusion: Primary ovarian neuroendocrine neoplasms can cause carcinoid syndrome and right-sided valvulopathy without liver metastasis, likely because ovarian venous drainage bypasses hepatic first-pass metabolism. Multidisciplinary care enables prompt diagnosis, preoperative stabilization, definitive resection, and coordinated surveillance.