Success with Lutetium-177 DOTATATE Therapy in Cushing’s Syndrome Caused by Functional Pancreatic Neuroendocrine Tumor (pNET) with Ectopic Adrenocorticotropic Hormone (ACTH) Secretion Refractory to Concomitant Everolimus, Pasireotide and Metyrapone

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Introduction: We report a case of a 59-year-old female presenting with florid ACTH-dependent Cushing’s syndrome complicated by pulmonary cryptococcal infection.

Aim(s):

Materials and methods: Following a normal pituitary MRI, a CT demonstrated an inoperable pancreatic mass with regional lymphadenopathy. Para-aortic lymph node biopsy demonstrated low to intermediate grade pNET with positive immunohistochemical staining for ACTH and Ki-67 <5%. Indium-111 octreotide scintigraphy confirmed intense octreotide-avidity with moderate avidity on Fluorine-18 FDG PET (SUV max 3.8). Adrenalectomy was not technically feasible due to splenic vein thrombosis and abdominal varices. Everolimus and pasireotide were commenced along with escalating doses of metyrapone and spironolactone. She improved both clinically and biochemically then remained stable on CT scans for eleven months. Upon clinical progression, CT scans showed no change but Gallium-68 DOTATATE PET scan revealed widespread progressive disease including new extensive liver and distant nodal metastases.

Conference: 11th Annual ENETSConcerence (2014)

Presenting Author:

Authors: Chan B, Wyld D, Burge M, Cuneo R, Macfarlane D,

Keywords: pancreatic, neuroendocrine, tumor, ACTH, everolimus, pasireotide, lutetium,