Endocrine Abstracts

Pheochromocytomas/paragangliomas (PPGLs) are catecholamine secreting tumors that, if missed or not properly treated, can be devastating due to potential lethal cardiovascular complications. The 2014 Endocrine Society clinical practice guideline on PPGL includes recommendations regarding optimalperi-operative care [1]. The main goal of preoperative management is to prevent a patient from developing anesthesia- or surgery-induced catecholamine storm. This warrants the involvemen...

After establishing a biochemical diagnosis, pheochromocytomas and paragangliomas can be localized using different imaging modalities. Appropriate imaging is critical for primary tumor localization, the detection of multiple primary tumors and the detection of metastases. This will guide the optimal choice between curative surgery and palliative treatment options.First line anatomical imaging modalities for pheochromocytoma and paraganglioma imaging inclu...

Paragangliomas (PGLs) derive from either sympathetic tissue in adrenal and extra-adrenal locations, or from parasympathetic tissue of the head and neck. Adrenal PGLs are usually referred to as pheochromocytomas. Most patients with adrenal and extra-adrenal abdominal PGLs have increased plasma and urine concentrations of catecholamines (dopamine, norepinephrine and epinephrine). Typical symptoms and signs of catecholamine excess include headache, palpitations, diaphoresis, and ...

The biochemical diagnosis of pheochromocytomas and extra-adrenal paragangliomas can be established by the measurement of plasma free metanephrines and 24 h urinary excretion of fractionated metanephrines. After establishing the biochemical diagnosis, tumours can be localized using different anatomical and functional imaging modalities. These include computed tomography, magnetic resonance imaging, single-photon emission computed tomography (SPECT) using [123I]-metai...

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 ...

Background: Pheochromocytomas and paragangliomas (mPPGL) are rare neuroendocrine tumors. Therapeutic options in advanced and irresectable mPPGL are limited. Two small retrospective studies demonstrated the effectiveness of temozolomide in patients with mPPGL and suggested that patients with mutation in the succinate dehydrogenase B (SDHB) gene might benefit more than SDHB wildtype cases.Aim: To re-evaluate safety and effectiveness of temozolomide in a la...

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...

Introduction: Pheochromocytoma, a rare catecholamine-secreting adrenal tumor, can cause hypertension and life-threatening complications. Hereditary cases have an increased risk of developing bilateral disease. Standard treatment involves radical adrenalectomy, leading to adrenal insufficiency in bilateral cases. Partial adrenalectomy aims to preserve adrenal function but has higher recurrence rates. This study compares outcomes of partial vs radical adrenalectomy in hereditary...

Background: Hypertension affects more than 30% of the adult population worldwide and is a major cardiovascular risk factor. Identifying secondary causes of hypertension is key to offering targeted treatment and mitigating adverse health outcomes. We tested the performance of urine steroid metabolomics (USM), the computational analysis of 24-hour urine steroid metabolome data by machine learning, for diagnosing endocrine hypertension.<str...

Background: Hypertension affects more than 30% of the adult population worldwide and is a major cardiovascular risk factor. Identifying secondary causes of hypertension is key to offering targeted treatments and mitigating adverse health outcomes. We tested the performance of urine steroid metabolomics (USM), the computational analysis of 24-hour urine steroid metabolome data by machine learning, for diagnosing endocrine forms of hypertension.Methods: 14...