Endocrine Abstracts

Introduction: Ovarian metastasis from neuroendocrine tumours is rare with < 80 reported cases. We describe a case of bilateral ovarian metastases from a primary ileal carcinoid tumour managed with somatostatin receptor agonist (SSA) therapy and surgery.

Case report: A 68-year-old lady presented with abdominal pain, distension, and episodic facial flushing. Echocardiography done to investigate left bundle branch block showed severe tricuspid regurgitation (TR) with preserved left ventricular function. Carcinoid heart disease due to carcinoid syndrome was suspected and confirmed with elevated urinary 24 h 5-HIAA excretion (310 umol/l). Chromogranin A was 377 mg/L. A diagnosis of probable primary ovarian carcinoid syndrome was made. Other causes of TR were excluded. Pelvic ultrasound showed a right ovarian mass of 5.6 cm. Serum CA-125 was 67 U/mL. CT scan showed bilateral adnexal masses, ascites, and a calcific small bowel mesenteric deposit without hepatic lesions. A multidisciplinary team involving endocrinology, gynaecologic oncology, cardiology, and anaesthesiology were involved in management. SSA therapy with Octreotide was initiated (100 mg TDS) pre-operatively and an octreotide infusion at 50-100 mg/h was used to minimise the risk of intraoperative carcinoid crisis. Staging laparotomy with bilateral salpingo-oophorectomy, ileal resection and anastomosis and removal of peritoneal disease was performed to complete cytoreduction. There were no enlarged retroperitoneal nodes. Characteristic findings included dense sclerosing fibrosis of peritoneal and retroperitoneal tissues. Histopathology showed a well-differentiated neuroendocrine tumour. Bilateral ovarian tumours, the absence of other teratomatous elements and presence of a well-differentiated NET in the ileal mesentery favoured a primary ileal tumour with ovarian metastases. Facial flushing resolved postoperatively.

Conclusion: A thorough history and clinical evaluation are critical in the diagnosis and management of unusual presentations of neuroendocrine tumours. A multidisciplinary team approach is critical to optimizing patient outcome and in anticipating and managing critical events such as carcinoid crisis.