Searchable abstracts of presentations at key conferences in endocrinology

ea0032s14.2 | Clinical care of the pheochromocytoma patient | ECE2013

Pheochromocytoma imaging

Pacak Karel

Pheochromocytomas (PHEO) and paragangliomas (PGL) are neuroendocrine tumors that were born to be imaged using specific radiopharmaceuticals. This is based on the expression of the cell membrane norepinephrine transporter system and somatostatin receptors, as well as GLUT and amino acid transporters found on PHEO/PGL cells.Anatomical imaging modalities (e.g. CT or MRI) may define the size, shape, structure and enhancement of tumors well, but they lack the...

ea0022pl7 | New trends in diagnosis and localisation of (neuro) endocrine tumours | ECE2010

New trends in diagnosis and localization of (neuro)endocrine tumors

Pacak Karel

Endocrine tumors differ from other tumors in that that they use specific pathways to synthesize, store, and release hormones, their metabolites, or precursors; these tumors may differentially express specific receptors or transporters located in either the cell membrane or intracellular space. Additionally, they can uptake released hormones back into their cells. These characteristics allow for the existence of several (neuro)endocrine, tumor-specific markers that can be used ...

ea0032p534 | Endocrine tumours and neoplasia | ECE2013

Mitochondrial ultrastructure in pseudohypoxic succinate dehydrogenase B and von Hippel–Lindau gene mutation derived pheochromocytomas and paragangliomas

Tsokos Maria , Fliedner Stephanie , Prodanov Tamara , Abu-Asab Mones , Osman Jailan , Lehnert Hendrik , Pacak Karel

Mutations in the mitochondrial succinate dehydrogenase (SDH) subunits A, B, C, and D have been shown to hamper oxidative phosphorylation and predispose to pheochromocytomas (PHEOs) and paragangliomas (PGLs). These tumors are characterized by a glycolytic and pseudohypoxic phenotype, which is also seen in most PHEOs/PGLs occurring as part of von Hippel–Lindau (VHL) syndrome, due to VHL gene mutations. The rate of extra-adrenal tumor origin and malignancy however is particu...

ea0016p306 | Endocrine tumours | ECE2008

Distinct catecholamine phenotypes in hereditary pheochromocytoma

Eisenhofer Graeme , Lenders Jacques WM , Mannelli Massimo M , Bornstein Stefan R , Pacak Karel

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, ...

ea0049gp25 | Adrenal 3 | ECE2017

Tumor microenvironment increases migration/invasion of murine pheochromocytoma SDHB silenced spheroids

D'Antongiovanni Vanessa , Martinelli Serena , Richter Susan , Canu Letizia , Guasti Daniele , Romagnoli Paolo , Pacak Karel , Eisenhofer Graeme , Mannelli Massimo , Rapizzi Elena

Pheochromocytomas (PHEOs) and paragangliomas (PGLs) are rare neuroendocrine tumors. About 30-40% of Pheo/PGLs are due to a germ-line mutation in one of the 13 main susceptibility genes which include the genes encoding the four subunits of the succinate dehydrogenase (SDH - mitochondrial complex II). In PHEO/PGL due to SDHB mutations up to 80% of affected patients develop metastatic disease and no successful cure is at present available. To obtain an experimental model resembli...

ea0049ep92 | Adrenal medulla | ECE2017

Role of microenvironment on metabolic control of murine pheochromocytoma SDHB silenced cells

D'Antongiovanni Vanessa , Richter Susan , Martinelli Serena , Canu Letizia , Ercolino Tonino , Eisenhofer Graeme , Pacak Karel , Rapizzi Elena , Mannelli Massimo

Pheochromocytomas (PHEOs) and paragangliomas (PGLs) are rare neuroendocrine tumors. About 30–40% of Pheo/PGLs are due to a germ-line mutation in one of the 13 main susceptibility genes which include the genes encoding the four subunits of the succinate dehydrogenase (SDH - mitochondrial complex II). In PHEO/PGL due to SDHB mutations up to 80% of affected patients develop metastatic disease and no successful cure is at present available. Tumor microenvironment plays a pivo...

ea0049ep93 | Adrenal medulla | ECE2017

Role of microenvironment on proliferation and migration of an SDHB silenced murine Pheochromocytoma cell line

Martinelli Serena , D'Antongiovanni Vanessa , Richter Susan , Canu Letizia , Ercolino Tonino , Eisenhofer Graeme , Pacak Karel , Rapizzi Elena , Mannelli Massimo

Paragangliomas are rare neuroendocrine tumors derived from neural crest cells: if localized in the adrenal medulla they are called Pheocromocytomas (Pheo).The 30–40% of Pheo are mutated in one of the susceptibility genes among which there are genes encoding for the four subunits of the succinate dehydrogenase (SDH). Germ line mutations of SDHB are metastatic in about 80% of the cases. Surgery is the current therapy, but in presence of metastasis there is no effective trea...

ea0041ep602 | Endocrine tumours and neoplasia | ECE2016

Synergistic anti-tumour effects of 13-cis retinoic acid and lovastatin in pancreatic neuroendocrine tumour (BON1) cells through enhanced EGFR inhibition

Nolting Svenja , Prada Elke Tatjana Aristizabal , Lauseker Michael , Maurer Julian , Spottl Gerald , Goke Burkhard , Pacak Karel , Grossman Ashley

Introduction: In our previous studies we found that the combination of 13-cis retinoic acid (13cRA) and lovastatin significantly reduced tumour growth in a mouse phaeochromocytoma allograft model, with the lowest microvessel density in the combination-treated tumours. We have now investigated the effect of 13cRA plus lovastatin on neuroendocrine (BON1, H727) and non-endocrine tumour (HepG2, Huh7) cell viability and signalling pathways (EGFR, AKT, ERK, p70S6K) to eluci...

ea0081oc6.1 | Oral Communications 6: Endocrine-Related Cancer | ECE2022

Recurrent disease in patients with sporadic pheochromocytoma and paraganglioma

Li Minghao , Prodanov Tamara , Meuter Leah , Kerstens Michiel , Bechmann Nicole , Prejbisz Aleksander , Fassnacht Martin , Timmers Henri , Beuschlein Felix , Fliedner Stephanie , Robledo Mercedes , Lenders Jacques , Pacak Karel , Eisenhofer Graeme , Pamporaki Christina

Background: It is well established that life-long follow-up is required for patients with hereditary pheochromocytomas and paragangliomas (PPGLs), due to the potential of developing recurrent disease. However, whether follow-up of patients with sporadic PPGLs is necessary, remains unclear.Aims: To examine the prevalence and predictors of recurrent disease in patients with sporadic PPGLs.Materials and method: This multicenter study included retrospective ...

ea0049oc7.3 | Cardiovascular endocrinology (1) | ECE2017

High Total 68Ga-DOTATATE-Avid Tumor Volume (TV) is associated with low progression-free survival and high disease-specific mortality rate in patients with neuroendocrine tumors

Tirosh Amit , Papadakis Georgios Z. , Millo Corina , Sadowski Samira M. , Herscovitch Peter , Pacak Karel , Marx Stephen J. , Yang Lily , Nockel Pavel , Shell Jasmine , Green Patience , Keutgen Xavier M. , Patel Dhaval , Nilubol Naris , Kebebew Electron

Background: Patients with neuroendocrine tumors (NETs) have divergent survival, even when having the same site of primary tumor, and tumor stage and grade. 68Gallium (68Ga)-DOTATATE positron emission tomography (PET)/computed tomography (CT) is a sensitive imaging modality for detecting NETs. The purpose of this study was to determine whether 68Ga-DOTATATE PET/CT imaging has any prognostic utility in patients with NETs.Methods: One hundred and eighty-fou...