#1078
Somatostatin Responsive ACTH and Precursor Excess in a Midgut Mesentery NET
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
Conference:
12th Annual ENETSConcerence (2015)
Presenting Author:
Authors:
Weickert M,
Khan S,
Shatwell W,
Chambers L,
Tomkins C,
Keywords:
NET,
ACTH precursors,
POMC,
somatostatin,
#48
Preliminary report of the use of everolimus in a patient with progressive medullary thyroid carcinoma
Introduction:
Medullary thyroid carcinoma (MTC) accounts for about 5-10% of all thyroid cancers, and while disease localized to the thyroid can be cured by surgery, there is no effective treatment for disseminated MTC. Constitutive activation of the RET proto-oncogene is implicated in familial and a proportion of sporadic cases, and up-regulates the AKT/PI3-kinase/mTOR signal transduction pathway. Recent studies have shown that the inhibitor of mTOR, everolimus (RAD001, Novartis, Basel), can inhibit the proliferation of the TT cell line and human MTC tumor cells in vitro. Clinically, everolimus has been shown to attenuate the progression of some gastrointestinal neuroendocrine tumors, but its clinical use in MTC has not yet been evaluated.
Conference:
7th Annual ENETSConcerence (2010)
Presenting Author:
Authors:
Druce M,
Maher K,
Grozinsky-Glasberg S,
Gross D,
Chambers J,
Keywords:
medullary thyroid cancer,
calcitonin,
everolimus,
RAD001,
