Endocrine Abstracts

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...

Introduction: Currently, it is unclear whether plasma free or 24-hour urinary metanephrines are preferable for diagnosis of pheochromocytoma/paraganglioma (PPGL) in children.Objectives: To investigate whether measurements of plasma free or 24-hour urinary fractionated metanephrines is a reliable test for screening for PPGL in children.Methods: This retrospective study included data from 60 children with and 78 without PPGL. Data in...

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 ...

Introduction: Pheochromocytomas and paragangliomas (PPGLs) exhibit an up to 20% malignancy rate. Various clinical, genetic, and pathological features have been proposed as predictors of malignancy. However, until present there are no robust indices to reliably predict metastatic PPGLs.Aim: The aim of the present study was to prospectively validate the value of methoxytyramine as risk marker of metastatic disease and establish a machine learning (ML) mode...