Endocrine Abstracts

A 44 yr old IT systems analyst, presented in 2003 with a six month history of weight gain, diabetes and hypertension. On examination he was floridly Cushingoid. Investigations showed raised 9AM cortisol (1887 nmol/l) with failure to suppress following low and high dose dexamethasone, together with elevated 24 h urinary free cortisol (3300 nmol) and circulating ACTH (130 ng/l) levels. CT scan demonstrated a complex 11.5×9 cm lesion in the left lobe of liver and biopsy indicated a tumour of uncertain origin. Consequently, venous sampling was undertaken, showing a hepatic (198 ng/l) versus peripheral (90 ng/l) gradient, suggesting that this lesion was the source of ectopic ACTH production. Other elevated neuroendocrine tumour (NET) markers included chromogranin A 536 (<60), GAWK 238 (<150), pancreatic polypeptide >500 (<300), and calcitonin 56 (0–4.6). Although the liver lesion was felt unlikely to be the primary tumour, an octreotide scan failed to demonstrate abnormal tracer uptake elsewhere in the liver or extrahepatically. CT chest and abdomen also failed to demonstrate a lesion.

Following preoperative treatment with metyrapone, he underwent successful left hemi-hepatectomy, and enucleation of two small right lobe nodules. Histology confirmed a chromogranin-positive neuroendocrine tumour, but negative staining for ACTH. Postoperatively, his Cushing’s was in biochemical remission, with normal circulating neuroendocrine markers. A further octreotide scan showed no liver or extrahepatic uptake.

He remained in biochemical remission until December 2005, when NET markers were the first to become abnormal with raised pancreatic polypeptide >500, Chr A 639, calcitonin 107. Shortly thereafter, Cushing’s syndrome recurred. An octreotide scan and cross sectional imaging show multiple hepatic lesions, the largest being 7 cm in size. His Cushing’s is again controlled on medical therapy. Current treatment options include chemotherapy with Streptozocin, 5FU and cisplatin or Yttrium 90 DOTA Octreotide therapy as the tumour is somastostatin receptor positive. He is currently being assessed in Royal free Hospital for suitability of this treatment.