Endocrine Abstracts

Oncocytic adrenocortical neoplasm (OAN) represents a rare variant of adrenocortical carcinoma (ACC) with less than 150 cases reported in the literature. OANs account for 1.8% of adrenal masses, predominantly in adults and usually presenting as large tumors featuring hormonal secretion in 20% of cases. Accurate histological characterization of OANs is crucial as this subtype is believed to be more indolent in clinical behavior. There are 3 categories of OANs: pure oncocytic, mi...

Cushing’s syndrome (CS) is a rare and complex condition. Once the diagnosis of CS is confirmed, repetitive morning ACTH measurement is indicated to assess whether CS is ACTH dependent or independent. ACTH under 2,2 pmol/l characterises ACTH-independent CS and above 4,4 pmol/l ACTH-dependent CS. Some groups recommend midnight ACTH measurement rather than the morning. The purpose of our work was to evaluate 12PM ACTH vs 8AM ACTH measurement in the etiological diagnostic of ...

Introduction: The French National Institute of Cancer (INCa) launched supported by the Ministry of Health in 2008 a program for the recognition of national networks for the management of rare cancers. Among the 23 selected networks COMETE-Cancer was recognized by INCa in 2009 for Adrenocortical carcinoma (ACC) and malignant pheochromocytoma/paraganglioma (MPP). At that time 60–120 new ACC/year and 30 new MPP/year were expected at the national level....

Context: Paragangliomas and pheochromocytomas (PPGL) are rare neuroendocrine tumors, characterized by a strong genetic component. Indeed, up to 40% of patients carry a germline mutation in a PPGL susceptibility gene. In accordance with the international recommendations, genotyping of PPGL susceptibility genes is therefore proposed to all patients with PPGL, but it has actually never been shown whether the identification of a germline mutation in one PPGL susceptibility gene ch...