Endocrine Abstracts

Pheochromocytomas (PHEOs) and paragangliomas (PGLs) are rare neuroendocrine tumors. About 30-40% of these tumors are due to e germ-line mutation in one of the 13 main susceptibility genes which include: the tumor-suppressor gene VHL; the proto-oncogene RET; the tumor-suppressor gene Nf1; the genes encoding the four subunits of the succinate dehydrogenase (SDH); the SDHAF2/SDH5 gene that is responsible for the flavination of the SDHA subunit;...

Pheochromocytoma (PHEO)/paraganglioma (PGL) are neural crest-derived tumors, and about 25% of the apparently sporadic cases are due to a germ line mutation in one of the susceptibility genes. The group of these genes includes the genes encoding three of the four subunits of the SDH, and the recently discovered gene, SDHAF2/SDH5, responsible for the flavination of the SDHA subunit.Although several hypotheses have been proposed, the molecular events by whi...

Somatostatin (sst) receptors are expressed in many tissues and multiple subtypes are often present in the same cells. Their activation by endogenous sst as well as by sst-analogues leads to inhibition of secretion and growth in some tumor, such as the neuroedocrine ones.Pheochromocytomas (Pheos) and paragangliomas (PGLs) are neural crest-derived tumors which can be sympathetic or parasympathetic in origin and present themselves as sporadic or familial. T...

Paragangliomas (PGLs) and pheochromocytomas (PHEOs) are neural crest-derived tumors (NCD). PGLs can be localized in parasympathetic ganglia (in the head-neck region or in the anterior thorax) or in sympathetic ganglia (in the posterior thorax or in the abdomen). PHEOs can be considered PGLs arising in the adrenal gland.NCD tumors can present as sporadic or familial. The percentage of hereditary forms is supposed to be 25%. The susceptibility genes predis...

Paragangliomas (PGLs) are neuroendocrine tumors arising from the neural crest and localized from the skull base to the pelvic floor. In particular, parasympathetic PGLs arise in the head/neck region (carotid body, vagal nerve, jugulare and tympanic glomus) and more rarely in the anterior thorax, while sympathetic PGLs are localized in the posterior thorax and in the abdomen and secrete catecholamines.Localization of paragangliomas might be performed by a...