Endocrine Abstracts

Ovarian neuroendocrine tumours are rare (< 2% gynaecological tumours) and first described in 1939 by Stewart et al. The occurrence of carcinoid heart disease alongside this is anecdotal. We present a previously fit 66 year old female, with a 6 month history of shortness of breath and ankle oedema. Echocardiogram revealed severe fixed tricuspid regurgitation, pulmonary valve disease, dilated right heart chambers and preserved left ventricular function. Carcinoid heart disease was suspected and she was referred to our neuroendocrine tumour clinic. She gave a seven year history of facial flushing and diarrhoea. Examination revealed a fixed violaceous facial flush, injected red eyes and evidence of right heart failure. The liver was pulsatile and a large pelvic mass was palpable. Investigations revealed chromogranin A of 242 nmol/l (0–6) and 5-hydroxyindolacetic acid (5HIAA) at 857 umol/24 hrs. CT and MRI showed a large solid/cystic 15 cm mass arising from the right ovary, avid on Octreoscan. No lesions were noted in the bowel or liver. Her carcinoid syndrome was controlled with octreotide 50 mg thrice daily subcutaneously with cross over to Lanreotide 120 mg deep sc injection every 28 days. Right heart failure was managed with bumetanide 1 mg daily. After careful review in the Neuroendocrine MDT including gynaecology, cardiology, anaesthetic and HPB surgical review; joint procedure with the neuroendocrine surgical team and gynaecologist was performed. She underwent an uneventful oophorectomy under octreotide cover. Histology revealed an ovarian insular carcinoid tumour with lymphovascular space invasion, ENETS grade 1 (KI 67 <1%). Her 24hr urinary 5HIAA normalized to 17 umol/24 hrs. Ovarian carcinoid presenting with carcinoid heart disease and syndrome is very rare and needs to be considered in elderly females who present with right heart valve disease. This case highlights the need MDT discussion in patients with NET.