Pheochromocytomas and paragangliomas (PPGLs) are tumors that arise from chromaffin cells and typically produce excess catecholamines. Approximately 40% of patients have a genetic mutation and 10–15% of these tumors are malignant. All patients with PPGLs should be considered for genetic testing. ...
Pheochromocytoma and paraganglioma (PPGL) are rare tumours and at least 30% are part of hereditary syndromes. Approximately 20% of hereditary PPGL are caused by pathogenic germ line variants in genes of the succinate dehydrogenase complex (SDHx), TMEM127 or MAX. Herein we present guidelines ...
Pheochromocytoma/Paragangliomas Chapter© 2016 Pheochromocytoma and Paraganglioma Chapter© 2024 Pheochromocytoma Chapter© 2018 References Webb TA, Sheps SG, Carney JA. Differences between sporadic pheochromocytoma and pheochromocytoma in multiple endocrine neoplasia, type 2. Am J Surg Pathol 1980; 4: ...
Individuals with neurofibromatosis type 1 (NF1) are at an increased risk of developing a pheochromocytoma or paraganglioma (PHEO/PGL). However, the best case detection strategy is unknown. Our objectives were to describe the prevalence, clinical presentation and outcomes of PHEO/PGL associated with ...
Pheochromocytomas and paragangliomas (PPGL) are neuroendocrine tumors which secrete catecholamines, causing cardiovascular compromise. While isolated tumors and locoregional disease can be treated surgically, treatment options for metastatic disease are limited, and no targeted therapies exist. Approximately 25...
To minimise complications during and post surgery the lesion(s) should be carefully localised via imaging studies. Adequate pre- and postoperative medical treatment is important. The history, diagnosis and therapy of two patients鈥攖he one with a paraganglioma of the organ of Zuckerkandl, the other...
The mechanisms triggering metastasis in pheochromocytoma/paraganglioma are unknown, hindering therapeutic options for patients with metastatic tumors (mPPGL). Herein we show by genomic profiling of a large cohort of mPPGLs that high mutational load, micr
Pheochromocytomas and extra-adrenal paragangliomas are rare, and often overlooked, causes of hypertension. The crucial first step is therefore to consider these tumors when thinking of possible diagnoses. Confirming the diagnosis requires biochemical evidence of inappropriate catecholamine production. Measureme...
Pheochromocytomas and paragangliomas are catecholamine-producing neuroendocrine tumors arising from chromaffin cells in the adrenal glands and in the ganglia. The catecholamines secreted by these tumors are usually responsible for signs and symptoms like hypertension, headache, palpitations, excessive sweating...
Guidelines Pheochromocytoma and Paraganglioma :Guidelines Pheochromocytoma and Paraganglioma :Endocrine, AnClinical, SocietyGuideline, PracticeLenders J., Eisenhofer G., Mannelli M., et al. Guidelines on Pheochromocytoma and Paraganglioma // J. Clin. Endocrinol. Metab. - 2014. - Vol. 99. - P.1915...