Abstract

PHEOCHROMOCYTOMA AND PARAGANGLIOMA IN PAEDIATRICS – AN OVERVIEW

Dr. Kannan Sreevasumathi*

ABSTRACT

Pheochromocytoma (PCC) and paraganglioma (PGL) are rare chromaffin cell tumors which secrete catecholamines and type a part of the family of system tumors. Although a rare reason behind hypertension in medicine, the presentation of hypertension in these patients is characteristic, and treatment is definitive. The gold standard for diagnosing is via measuring of plasma free metanephrines, with imaging studies performed for localization, identification of pathological process lesions and for surgical resection. surgical medical aid with alpha-blocking agents, beta blockers, and probably tyrosine hydroxylase inhibitors aid in an exceedingly safe pre-, intra- and surgical course. PCC and PGL are inheritable in the maximum amount as eightieth of medicine cases, and every one patients with mutations ought to be followed closely given the danger of repetition and malignancy. While the presentation of chromaffin cell tumors has been well represented with multiple endocrine pathologic process, NF1, and Von Hippel–Lindau syndromes, the identification of new sequence mutations resulting in chromaffin cell tumors at a young age is dynamical the landscape of however clinicians approach such cases. The paraganglioma–pheochromocytoma syndromes (SDHx) comprise familial sequence mutations, of that the SDHB gene mutation carries a high rate of malignancy. Since the inheritance rate of such tumors is over antecedently represented, screening is suggested in all patients, and long follow-up for continual tumors could be a should. A multidisciplinary team approach permits for optimum health-care delivery in such kids. This review serves to supply an summary of medicine PCC and PGL, as well as updates on the preferred strategies of imaging, pointers on sequence testing yet as management of hypertension in such patients.

Keywords: pheochromocytoma, paraganglioma, paediatrics, SDHx hereditary paraganglioma–pheochromocytoma syndromes, phenoxybenzamine, Metyrosine, long follow-up.