Grfoma

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Retrieved

2021-01-23

Source

Orphanet

Trials

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Genes

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Drugs

(R)-1-[1-(4-acetoxy-3,3-dimethyl-2-oxo-butyl)-2-oxo-5-(pyridin-2-yl)-2,3-dihydro-1H-benzo[e][1,4]diazepin-3-yl]-3-(3-methylamino-phenyl)-urea, (S)-ethyl 2-amino-3-(4-(2-amino-6((R)-1-(4-chloro-2-(3-methyl-1H-pyrazol-1-yl)phenyl)- 2,2,2-trifluoroethoxy)pyrimidin-4-yl)phenyl)propanoate, Combretastatin A4 phosphate (CA4P), Everolimus ( AFINITOR, VOTUBIA ), Gallium (68Ga)-edotreotide ( GALLIUM DOTATOC GA 68, SomaKit TOC ), Gallium (68Ga)-pasireotide tetraxetan, Gallium [Ga-68]-N-[(4,7,10-tricarboxymethyl-1,4,7,10-tetraazacyclododec-1-yl)acetyl]-D-phenylalanyl-L-cysteinyl-L-tyrosyl-D-tryptophanyl-L-lysyl-L-threoninyl-Lcysteinyl-L-threonine-cyclic(2-7)disulfide ( NETSPOT ), Humanised IgG4 monoclonal antibody to the human toll-like receptor type 2, Lutetium (177Lu) edotreotide, Pasireotide ( SIGNIFOR, SIGNIFOR LAR ), Pegylated recombinant human interleukin-10, Relacorilant, Satoreotide trizoxetan, Yttrium (90Y) edotreotide, [Lu-177]-DOTA-Tyr3-Octreotate ( LUTATHERA ), lutetium-177(3+), S2,S7-cyclo[N-{4,7,10-tricarboxymethyl-1,4,7,10-tetraaza-cyclododecan-1-yl-acetyl}-4-chloro-L-phenylalanyl-D-cysteinyl-4-[(4S)-2,6-dioxo-1,3-diazinane-4-carboxamido]-L-phenylalanyl-4-(carbamoylamino)-D-phenylalanyl-L-lysyl-L-threonyl-L-cysteinyl-D-tyrosinamide], motixafortide

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GRFoma is a type of pancreatic endocrine tumor (see this term) that hypersecretes growth hormone-releasing factor (GRF or GHRH) and that clinically resembles a pituitary adenoma (see this term) as patients present with acromegaly. In addition to the pancreas, this tumor can also occur in the lungs or small intestine, are usually large > 6cm and approximately 1/3 have metastasized at the time of diagnosis. It often co-occurs with Zollinger-Ellison syndrome or multiple endocrine neoplasia type 1 (MEN 1; see these terms).