Endocrine Abstracts

The occurrence of a pheochromocytoma (Pheo) or a paraganglioma (PGL) during pregnancy is extremely rare with a frequency of 0.002% of all pregnancies.Because of the rarity of this association and the extremely variable clinical picture of Pheo/PGL, the diagnosis constitutes a real challenge for the clinicians. In fact, the differential diagnosis between Pheo/PGL and the more frequent gestational hypertension or (pre)eclampsia is very difficult. Paroxysma...

Pheochromocytoma (Pheo) is a chromaffin tumor of the adrenal gland. When extra-adrenal, the tumor is called paraganglioma (PGL). At present, the only objective criterion for malignancy is the presence of metastases (i.e. spreading of the tumor in bones, liver, lungs or lymph-nodes where chromaffin tissue is normally absent). Extensive invasion of adjacent tissues can be considered only an indicator of malignant potential as well as high cellularity, necrosis, vascular/capsular...

Definition: High blood pressure due to excessive activation of mineralocorticoid receptors (MR).Consequences of an excess in MR activation are an excess in sodium and water retention, an expansion of plasma volume and a depletion of exchangeable body potassium.Laboratory hallmarks of mineralocorticoid hypertension are a suppressed renin secretion and a tendency to develop hypokalemia.Causes: The most frequent...

Context: Adrenocortical carcinoma (ACC) is an aggressive malignancy with high recurrence rates. Regular post-operative follow-up imaging is essential, but associated with high radiation exposure and frequent diagnostic ambiguity. Urine steroid metabolomics has been described as a novel diagnostic tool for the detection of adrenocortical malignancy. Here we present the first clinical study assessing the performance of this innovative approach in the follow-up of patients with c...

Adrenocortical carcinoma (ACC) is a rare, heterogeneous malignancy with aggressive behavior and poor prognosis, particularly when metastatic at diagnosis. To date, radical surgery, possibly associated to mitotane adjuvant therapy, is considered the best option for ACC treatment. However, the mean 5-year survival rate diminishes dramatically in metastatic ACC and chemo-resistance often develops. Thus, more specific and effective drugs for ACC treatment are urgently required. Th...

Solid tumours are very complex tissues comprising not only cancer cells, but also non-malignant stromal cells such as endothelial cells, fibroblasts, immune cells and extracellular matrix, forming the so called tumour microenvironment. In the last few years, it has become more and more evident the pivotal role of the tumour microenvironment in modulating cancer progression and metastasis. Tumour microenvironment has thus become a potential therapeutic target. To obtain an expe...

Adrenocortical carcinoma (ACC) is a rare, heterogeneous malignancy with a poor prognosis, particularly when metastatic at diagnosis. To date, radical surgery, possibly associated to mitotane adjuvant therapy, is the only available treatment. However, the mean 5-year survival rate drops under 10% in metastatic ACC and chemo-resistance often develops. Thus, more specific and effective drugs for ACC treatment are urgently required. The antidiabetic drug metformin, used in type 2 ...

This study examined whether different forms of hereditary pheochromocytoma are characterized by different catecholamine phenotypes and whether this is reflected by differences in plasma concentrations of normetanephrine, metanephrine and methoxytyramine – the respective O-methylated metabolites of norepinephrine, epinephrine and dopamine. Subjects included 154 patients with hereditary pheochromocytoma, 72 with tumors associated with von Hippel–Lindau (VHL) syndrome, ...

The von Hippel-Lindau (VHL) syndrome is an inherited multi-tumor disorder characterized by clinical heterogeneity and high penetrance. Pheochromocytoma (Pheo) is present in 10-15% of cases. It can be isolated or associated with other lesions such as hemangioblastomas, kidney cysts or cancer, and pancreatic lesions. Pheos secrete norepinephrine and are generally located in the adrenals. While performing genetic testing in patients affected by apparently sporadic pheos or PGLs, ...

Pheochromocytoma/paraganglioma (Pheo/PGL) are rare neuroendocrine tumors generally benign. About 30–40% of Pheo/PGL are due to germ-line mutations in one of the susceptibility genes, including those encoding the succinate dehydrogenase subunits A-D (SDHA-D). Up to 80% of patients affected by SDHB mutated Pheo/PGL develop metastatic disease with no successful cure at present. In this study, we evaluated the different effect of tumor microenvironment on tu...