Carcinoid tumours are the commonest neuroendocrine tumours (50%), with a prevalence of 35/100,000. 10% present with carcinoid syndrome due to release of kinins and serotonin, released directly from the tumour in to the blood or due to liver metastases. Carcinoid heart disease due to ovarian carcinoid is extremely rare. Here we present such a case. A 69 year old female presented to surgeons with increased bowel frequency and weight loss. Blood tests, colonoscopy and biopsies were normal. One year later she presented with shortness of breath and signs of right heart failure prompting referral to cardiology. Echocardiogram showed thickened tricuspid and pulmonary valves with severe tricuspid regurgitation raising suspicion of carcinoid heart disease. She was referred to the endocrine team, who uncovered symptoms of intermittent flushing, loose stools and generalised pruritis. Biochemical screening confirmed elevated urinary 5-HIAA at 665 umol/24 hrs (050). CT scan confirmed a 15 cm mass in the right ovary with no metastases. Octreotide scanning showed the medial part of the tumour as functionally active and so octreotide therapy was started. Consequently, 5-HIAA levels reduced by fifty percent within weeks. After extensive discussion involving endocrinologists, obstetricians, cardiothoracic surgeons and anaesthetists it was decided the patient should undergo tumour resection followed by valve replacement surgery. Tumour resection was performed under intravenous octreotide infusion and histology showed mature cystic teratoma. Following surgery, 5-HIAA levels normalised without octreotide therapy. Subsequent tricuspid and pulmonary valve replacements were successfully carried out. Carcinoid heart disease without liver metastases is only known to occur with ovarian carcinoid or retroperitoneal disease. This was a very complex case requiring meticulous planning and coordination between various specialities to deal with both the tumour and heart disease, leading to an excellent outcome.
Declaration of interest: There is no conflict of interest that could be perceived as prejudicing the impartiality of the research reported.
Funding: No specific grant from any funding agency in the public, commercial or not-for-profit sector.
CT Scan: In the pelvis the uterus looks normal but there is a sinister looking 15 cm cystic/solid mass anteriorly extending from the midline into the right side.