Introduction: Carcinoid tumours commonly originate from the gastrointestinal tract. Cardiac manifestations occur in 1041% of patients with carcinoid syndrome, usually associated with liver metastases. We report an atypical case of a carcinoid tumour associated with right sided heart disease without distant metastases.
Case description: A 75-year-old lady with no significant past medical history and a lifelong non-smoker, was admitted with 3 months history of worsening shortness of breath. She had features of right heart failure confirmed on echocardiography and cardiac catheterisation with an estimated RVSP of 53 and tricuspid regurgitation. Basal biochemistry, Chest radiograph and CTPA were normal. On further review, she was noted to have disproportionate facial flushing to the degree of failure, and subsequent carcinoid screening was positive with raised urinary 5HIAA at 258(050) and Chromogranin-A of 379(04). Detailed investigations for pulmonary and gut primary were negative and there was no evidence of liver metastasis. Thin slice CT showed small right ovarian mass, which was confirmed on MRI and subsequent octreoscan was consistent with isolated high uptake on the right ovary. She underwent laproscopic right oophorectomy and histology confirmed carcinoid tumour of the insular type.
Both urinary 5HIAA and Chromogranin-A were normal 4 weeks after surgery and she is currently enjoying normal activity off Somatostatin analogues, and minimum cardiac supportive medications.
Discussion: Primary ovarian carcinoids are uncommon and their association with carcinoid heart disease is very rare; only 21 reported cases in the literature. Cardiac involvement develops without liver metastases, since the vasoactive mediators are released directly into systemic circulation. Absence of distant metastases provides these tumours with good prognosis.
Conclusions: Ovarian carcinoids, diagnosed early, are potentially surgically curable. Our case highlights the importance of considering ovarian carcinoids in patients presenting with right sided failure, features of carcinoid syndrome and unknown primary.