Endocrine Abstracts

Introduction: Approximately 20% of neuroendocrine tumor (NET) patients experience carcinoid syndrome (CS), primarily associated with metastatic ileal NETs. CS manifests with symptoms such as diarrhea, flushes, and cardiac complications. Carcinoid heart disease (CHD) is a negative prognostic factor in NETs. However, the considerable variability in reported diagnoses and treatment approaches in the literature creates uncertainty about optimal management in this specific population.

Methods: A cross-sectional, single-center retrospective study was designed. Patients with CS followed during 2023 in our hospital were included, focusing on those with associated carcinoid heart disease. Epidemiological, clinical, biochemical, therapeutic, and evolutionary variables were recorded.

Results: Fifteen patients (9 females and 6 males) with a mean age of 65 years (45-77 years) were included. The primary tumor location was intestinal (jejuno-ileal) in 14 cases, with only 1 pancreatic case. Tumor grade was G2 in 60%, G1 in 33.3%, and G3 in 6.7%. Diarrhea was present in 80% of cases, and flushing in 60%. Biochemically, the mean chromogranin A (CgA) was 356 units (range 3.2 - 3515), and mean 5-hydroxyindoleacetic acid (5HIAA) was 77 mg/24h (range 12.4 - 437). Regarding CHD, this comorbidity was present in 20% of patients (3/15). The time from oncologic diagnosis to carcinoid heart disease diagnosis was less than 4 months (1-120 days). Two patients underwent cardiac surgery (one with a biological prosthesis and the other with a mechanical prosthesis). After surgery, their dyspnea improved according to NyHA from II-III to I-II, respectively. One patient underwent a second surgery and died due to complications. All three patients were being treated with somatostatin analogues. Only the patient with the mildest heart disease (NyHA I) received PRRT treatment. There were no statistically significant differences in CgA and 5HIAA values at diagnosis between patients with and without CHD.

Conclusions: Significant variability exists in the treatment approaches for patients with carcinoid heart disease, and mortality after valve replacement remains high. Chromogranin A and 5HIAA were not predictive markers for CHD presence in our CS population.