Somatostatin Responsive ACTH and Precursor Excess in a Midgut Mesentery NET

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Introduction: ACTH production from a midgut mesentery NET is extremely rare (1). A 62y old woman presented with hypokalaemia (2mmol/L) and hyperpigmentation, 11y after surgery of a pT3N1Mx non-functional G1 NET with SRS positive, non-resectable but stable, residual mass encasing mesenteric vessels. Serum cortisol (3261 nmol/L), ACTH (796 ng/L), CgA (530 pmol/L) and urine total cortisol metabolites (33920 µg/24h) including 14 sub-products indicated change of biological behaviour into a functioning NET. Pituitary or pulmonary sources were excluded

Aim(s): To test somatostatin (SS) responsiveness of ACTH excess

Materials and methods: A test dose of SS 100µg sc was applied

Conference: 12th Annual ENETSConcerence (2015)

Presenting Author:

Authors: Weickert M, Khan S, Shatwell W, Chambers L, Tomkins C,

Keywords: NET, ACTH precursors, POMC, somatostatin,