Endocrine Abstracts

Background: Available medical treatments for Broncho-Pulmonary Neuroendocrine Neoplasm (BP-NENs) derived from clinical trials are not specific for the management of this malignancy. Sunitinib, a multi-receptor tyrosine-kinases (RTKs) inhibitor, mainly described as VEGFR inhibitor, has shown its efficacy in Pancreatic NENs but there are not available data about its action in BP-NENs. Our aim was to understand the effects of RTKs inhibition promoted by Sunitinib in order to eval...

Patients with prolactinomas may develop acromegaly during D2-agonists (DA), suggesting the existence of somatomammotroph adenomas with asynchronous secretion of GH and PRL. This may be due to the acquisition of somatotroph characteristics by lactotroph cells or to GH co-secretion by somatommammotroph cells unmasked after PRL inhibition by DA. The prevalence of silent acromegaly in prolactinomas during DA is 4.1%. The purpose of this study was to evaluate the somatotroph axis i...

Peroxisome-proliferator activated receptors (PPARs) are involved in a number of neoplasia. PPARα (PPARα) is a partner of the aryl hydrocarbon receptor interacting protein (AIP), which is involved in the pathogenesis of pituitary adenomas (PA). We wished to investigate the potential expression and biological significance of PPARα in PA, especially in GH/PRL-secreting tumours.Material and methods: A large series of PA was collected (n</e...

Ectopic Cushing syndrome (EAS) is a rare condition characterized by ACTH-dependent hypercortisolism resistant to normal physiologic suppression by glucocorticoids. EAS is due to an extra-pituitary tumor producing bioactive molecules generated by post-translational cleavage of the proopiomelanocortin gene (POMC). EAS is associated with significant morbidity and mortality and although surgical resection of the primary ACTH-producing tumor remains the mainstay of therapy, not all...

Background: Bronchial carcinoids (BCs) are rare neuroendocrine tumors that are still orphan of medical treatment. Human BC primary cultures may display resistance to everolimus, an inhibitor of the mammalian target of rapamycin (mTOR), in terms of cell viability reduction.Aim: To assess whether the novel dual PI3K/mTOR inhibitor, NVP-BEZ235, may be effective in everolimus-resistant human BC tissues and cell lines. In addition, we search for possible mark...

The aryl hydrocarbon receptor interacting protein (AIP) gene is abundantly expressed in normal somatotrophs. Somatotropinomas in patients with germline AIP mutations (AIPmut) are typically more aggressive than non-AIPmut adenomas and associated with higher GH/IGF1 secretion. AIP downregulation may also occur in sporadic somatotropinomas, especially in invasive tumours. We wished to evaluate the impact of somatostatin analogues (SSA) pre-treatment on AIP expression in sporadic ...

Congenital hypothyroidism (CH) is frequently associated with congenital heart defects (CHD). Thyroid defects may have a higher prevalence in children with CHD as embryonic thyroid gland share nuclear transcription factors with heart and great vessels during organogenesis. We investigated thyroid function in 325 children (165 M/160 F, aged 0.2–15.4 years), affected by CHD. Patients with Down syndrome, recent administration of iodinated contrast agents, low T3 sy...

Background: Familial isolated pituitary adenomas (FIPA) is a rare inherited disorder accounting for about 2% of pituitary adenomas. Mutations in the Aryl hydrocarbon receptor Interacting Protein (AIP) gene have been described in about 15% of FIPA families and rarely in early onset sporadic pituitary adenomas. Among the AIP mutations reported so far, the R304X represents, after the Finnish founder mutation Q14X, the second most common one.Me...

IntroductionAIP is a predisposing gene for GH/PRL-secreting PitNETs. Clinically non-functioning PitNETs (NFPT) occasionally occur in the setting of AIP germline mutations, sometimes arising from Pit-1 lineages. However, AIP overexpression has been observed in unselected NFPT and associations with the gonadotroph phenotype and/or tumour aggressiveness were suggested. We wished to evaluate the significance of AIP expression in gonadotroph tumours defined b...

IntroductionAryl hydrocarbon receptor Interacting Protein (AIP), a pituitary tumour suppressing gene located in 11q13, is the most common predisposing gene for early-onset and familial acromegaly. Fifteen years after its identification, there is little evidence of AIP involvement in non-pituitary tumors. We had the opportunity to study AIP status in a cortisol-producing adenoma operated in an AIP mutation carrier.Case-report<p ...