Insights into Bilateral Pheochromocytoma- A Clinical Case Series from South India
Keywords:
Adrenalectomy, Bilateral Pheochromocytoma, Hereditary Pheochromocytoma, VHL Syndrome *Author for correspondenceAbstract
Bilateral Pheochromocytoma (PCC) is a rare condition characterized by the presence of catecholamine-secreting tumours in both adrenal glands. Hereditary Pheochromocytomas occur at a younger age and tend to be multifocal and/or bilateral at presentation. This case series presents three patients diagnosed with bilateral Pheochromocytoma, highlighting their clinical presentation, diagnostic approach, surgical management, and outcomes. All patients had sustained severe hypertension associated with the classic symptoms of Pheochromocytoma. Two patients had synchronous tumours whereas one patient had a metachronous presentation. Imaging studies including CECT abdomen and Ga-68 DOTANOC PET/CT showed bilateral adrenal lesions in all cases along with renal hilar paraganglioma in one case. Biochemical phenotype of the tumours was predominantly normetanephrine secreting type. Genetic testing done in two patients showed missense mutation (p.Arg167Gln in exon 3 and Gly93Ser in exon 1) of VHL tumour suppressor gene. All three patients underwent open bilateral adrenalectomy with resolution of symptoms and normalization of catecholamine levels. Pathology confirmed the diagnosis of Pheochromocytoma in both adrenal glands. None of them developed symptoms of adrenal insufficiency during the follow-up period. Two patients became normotensive after surgery, while one patient continues to have persistent hypertension, although with a reduced need for antihypertensive medications compared to the preoperative period. Remarkable remission of diabetes mellitus was noticed in two patients after tumour removal. This case series highlights the importance of recognizing bilateral Pheochromocytoma, the role of genetic evaluation, and the necessity for life-long follow up to optimize outcomes.
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