Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2023 Aug 31;14(4):1878-1886.
doi: 10.21037/jgo-22-909. Epub 2023 Aug 23.

Metastatic well differentiated serotonin-producing pancreatic neuroendocrine tumor with carcinoid heart disease: a case report

Matthew R Kudelka  1   2 , Ghassan K Abou-Alfa  1   3 , Eileen M O'Reilly  1   3 , Michael B Foote  1   3 , Bhawna Sirohi  4 , Rawad Elias  5 , Ali Shamseddine  6 , Viktoriya Paroder  1   3 , Amgad M Moussa  1   3 , Paul Cohen  1   2 , Karuna Ganesh  1   3
Affiliations
Case Reports

Metastatic well differentiated serotonin-producing pancreatic neuroendocrine tumor with carcinoid heart disease: a case report

Matthew R Kudelka et al. J Gastrointest Oncol. .

Abstract

Background: Less than two percent of pancreatic neuroendocrine tumors (NETs) produce serotonin. Serotonin can cause carcinoid syndrome and less commonly carcinoid heart disease (CHD). CHD is associated with increased mortality and requires a more aggressive approach. Here we present a rare case of a serotonin-producing pancreatic NET complicated by CHD at presentation and discuss timing of systemic therapy, liver-directed therapy, and heart failure management.

Case description: A 36-year-old white man presented with diarrhea, lower extremity edema, and exertional dyspnea. He was found to have a well-differentiated serotonin-producing pancreatic NETs grade three with bilobar liver metastasis complicated by carcinoid syndrome and CHD. His symptoms and disease burden improved with somatostatin analog and liver-directed therapy with bland embolization to control carcinoid symptoms and obtain rapid hormonal control to prevent progression of CHD. He concurrently received diuretics to manage his heart failure and was considered for valvular replacement surgery, which was deferred for optimal hormonal control.

Conclusions: Our case highlights the importance of multidisciplinary care for patients with pancreatic NETs and early identification and management of CHD. Although uncommon, serotonin-producing pancreatic NETs can present with CHD and require combination of somatostatin analogs, liver-directed therapy, and heart failure management.

Keywords: Case report; carcinoid; carcinoid heart disease (CHD); neuroendocrine tumor (NET); pancreatic NET (PaNET).

PubMed Disclaimer

Conflict of interest statement

Conflicts of Interest: The authors have completed the ICMJE uniform disclosure form (available at https://jgo.amegroups.com/article/view/10.21037/jgo-22-909/coif). The series “Educational Case Series of the Memorial Sloan Kettering Cancer Center” was commissioned by the editorial office without any funding or sponsorship. GKA served as the unpaid Guest Editor for the series and he received research support from Arcus, Agios, Astra Zeneca, BioNtech, BMS, Celgene, Flatiron, Genentech/Roche, Genoscience, Incyte, Polaris, Puma, QED, Silenseed, Yiviva, and consulting support for efforts with Adicet, Astra Zeneca, Alnylam, Autem, Bayer, Beigene, Berry Genomics, Cend, Celgene, CytomX, Eisai, Eli Lilly, Exelixis, Flatiron, Genentech/Roche, Genoscience, Helio, Incyte, Ipsen, Legend Biotech, Merck, Nerviano, QED, Redhill, Rafael, Servier, Silenseed, Sillajen, Sobi, Surface Oncology, Therabionics, Vector, and Yiviva. EOR declares research funding to Institution from Genentech/Roche, Celgene/BMS, BioNTech, AstraZeneca, Arcus, Elicio, Parker Institute, American Association of Cancer Research, Imedex and Shanghai Jo’Ann Medical Technology Co., Ltd, consulting fees from Seagen, Boehringer Ingelheim, BioNTech, Ipsen, Merck, IDEAYA, Silenseed, Novartis, AstraZeneca, Noxxon, BioSapien, Cend Therapeutics, Astellas, Thetis, Autem, ZielBio, Tempus, Research To Practice, Agios (spouse), Genentech-Roche (spouse), Eisai (spouse), and she is on Data Safety Monitoring Board of Cytomx Therapeutics (DSMB) and Rafael Therapeutics (DSMB). BS declares unpaid memberships on committees for Lancet Oncology international advisory board, UK global cancer network steering committee, ASCO, Cansupport management committee, EBMT nuclear accident committee and ISMP executive committee. KG reports grants from NIH/NCI, AACR, Burroughs Wellcome Funds, Damon Runyon Cancer Research Foundation, Pershing Square Sohn Foundation, Stand Up to Cancer, Josie Robertson Foundation, Anna fuller foundation, Dalton Family Foundation. The authors have no other conflict of interest to report.

Figures

Figure 1
Figure 1
Timeline of disease course. CTCAP with liver triphasic with and without contrast and MR liver with and without contrast demonstrate area of necrosis in segments 7/8 following second bland embolization where prior tumors were present. CT, computerized tomography; CTCAP, CT chest/abdomen/pelvis; MR, magnetic resonance.
Figure 2
Figure 2
DOTATE PET scan. DOTATE avid hypodense hepatic lesions, for example segment 2, 7.0 cm × 6.5 cm (SUV 20.5) and segment 7, 5.6 cm × 4.8 cm (SUV 25.5); pancreatic tail lesion 5.4 cm × 4.0 cm (SUV 26.6); and upper abdominal nodal metastases. Arrows mark tumor. PET, positron emission tomography; SUV, standardized uptake value.
Figure 3
Figure 3
Systemic serotonin metabolism in gastrointestinal NETs. NETs in the GI tract secrete serotonin that is inactivated by the liver. Tumors that metastasize to the liver bypass hepatic inactivation and secrete serotonin into circulation. Serotonin in the right heart causes valvular fibrosis but is inactivated by pulmonary endothelia prior to transit to the left heart. Therefore, the left heart is spared in carcinoid heart disease unless a cardiac shunt is present (1). GI, gastrointestinal; HT, hydroxytryptamine; NETs, neuroendocrine tumors.
See this image and copyright information in PMC

References

    1. Davar J, Connolly HM, Caplin ME, et al. Diagnosing and Managing Carcinoid Heart Disease in Patients With Neuroendocrine Tumors: An Expert Statement. J Am Coll Cardiol 2017;69:1288-304. 10.1016/j.jacc.2016.12.030 - DOI - PubMed
    1. Cloyd JM, Poultsides GA. Non-functional neuroendocrine tumors of the pancreas: Advances in diagnosis and management. World J Gastroenterol 2015;21:9512-25. 10.3748/wjg.v21.i32.9512 - DOI - PMC - PubMed
    1. Halfdanarson TR, Rabe KG, Rubin J, et al. Pancreatic neuroendocrine tumors (PNETs): incidence, prognosis and recent trend toward improved survival. Ann Oncol 2008;19:1727-33. 10.1093/annonc/mdn351 - DOI - PMC - PubMed
    1. Depoilly T, Leroux R, Andrade D, et al. Immunophenotypic and molecular characterization of pancreatic neuroendocrine tumors producing serotonin. Mod Pathol 2022;35:1713-22. 10.1038/s41379-022-01110-x - DOI - PubMed
    1. Halperin DM, Shen C, Dasari A, et al. Frequency of carcinoid syndrome at neuroendocrine tumour diagnosis: a population-based study. Lancet Oncol 2017;18:525-34. 10.1016/S1470-2045(17)30110-9 - DOI - PMC - PubMed

Publication types