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


Authors: Kudelka, M. R.; Abou-Alfa, G. K.; O’Reilly, E. M.; Foote, M. B.; Sirohi, B.; Elias, R.; Shamseddine, A.; Paroder, V.; Moussa, A. M.; Cohen, P.; Ganesh, K.
Article Title: Metastatic well differentiated serotonin-producing pancreatic neuroendocrine tumor with carcinoid heart disease: A case report
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. © Journal of Gastrointestinal Oncology. All rights reserved.
Keywords: case report; carcinoid; neuroendocrine tumor (net); carcinoid heart disease (chd); pancreatic net (panet)
Journal Title: Journal of Gastrointestinal Oncology
Volume: 14
Issue: 4
ISSN: 2078-6891
Publisher: Pioneer Bioscience Publishing Company  
Date Published: 2023-08-31
Start Page: 1878
End Page: 1886
Language: English
DOI: 10.21037/jgo-22-909
PROVIDER: scopus
PMCID: PMC10502540
PUBMED: 37720425
DOI/URL:
Notes: Article -- Source: Scopus
Altmetric
Citation Impact
BMJ Impact Analytics
MSK Authors
  1. Ghassan Abou-Alfa
    568 Abou-Alfa
  2. Eileen O'Reilly
    780 O'Reilly
  3. Karuna   Ganesh
    68 Ganesh
  4. Viktoriya Paroder
    60 Paroder
  5. Paul Cohen
    3 Cohen
  6. Amgad Mohamed Abdelhady Moussa
    34 Moussa
  7. Michael Bonner Foote
    41 Foote