Endocrine Abstracts (2019) 65 P291 | DOI: 10.1530/endoabs.65.P291

ACTH producing pancreatic NET

Muhammad Masood Ashraf1, Christine May1, Lia Anguelova1, Mike Tadman1, Shahab Khan2, Radu Mihai2 & Bahram Jafar-Mohammadi1

1Oxford Centre for Diabetes Endocrinology & Metabolism, Churchill Hospital, Oxford, UK; 2Department of Endocrine Surgery, Churchill Hospital, Oxford, UK

We present the case of a 64 year old woman who presented with one month history of tiredness and 8 kg weight loss. Severe hypokalemia (2.2 mmol/l) was identified by the GP. Clinically she appeared mildly Cushingoid. Biochemical investigations showed a random cortisol significantly elevated at 2170 nmol/l, 24-hour urinary cortisol was 15 700 nmol/l(0−135). ACTH level was elevated at 7400 ng/l(0−40). The low dose dexamethasone suppression test demonstrated failure to suppress, cortisol value > 1700 nmol/l. The clinical presentation was felt to one of a malignant process and CT chest abdomen and pelvis confirmed liver metastases, occupying approximately 75% of the liver, with no identifiable primary alesion and bilateral adrenal hyperplasia. The liver biopsy confirmed metastatic well differentiated (G1-Ki 67 of <2%) Neuroendocrine Tumour with Likely GI tract primary origin based on immunohistochenistry. A gallium Dotatate PET scan demonstrated Dotatae avid tumour in tail of pancreas, with multiple Dotatate avid liver metastasis. Considering her symptoms, initial blockade with metyrapone was attempted but was ultimately inadequate for symptom control. Somatostatin Analogues (SSA) was commenced given the DOTATATE scan results. She suffered several collapse fractures of her spine (no evidence of a malignant process were seen and this was felt to be osteoporotic in nature. Unfortunatelt the patient’s symptoms and cortisol levels were not well controlled on Metyrapone and SSA, therefore bilateral adrenalectomy was felt to be necessary. As there are two surgeons locally who are able to perform adrenalectmy through a posterior approach using retroperitoneal technique she underwent a simultaneous retroperitoneal bilateral adrenalectomy. Post operatively she has good clinical improvement in her symptoms and further imaging is planned to consider if PRRT is required for disease progression. Our case demonstrates the need to review and modify treatments depending on patient’s response. We believe this is the first simultaneous bilateral adrenalectomy in the UK.

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