Management of blood pressure in children with pheochromocytoma and other catecholamine-secreting tumors (CSTs) is unique and challenging. The authors report a single-center experience using sequential α-adrenergic blockade (phenoxybenzamine), increased fluid intake, and β-blockade for presurgical management of 10 CSTs in children. In this retrospective review, mean duration for blood pressure control in preparation for surgery was 4.5±2.6 weeks. Intraoperative hypertension was noted transiently (<2 hours) in eight patients (80%) and was treated with continuous infusion of short-acting antihypertensive agents. Two (20%) patients required vasopressor medication infusion to manage intraoperative hypotension. Only two (20%) patients developed postoperative hypotension and required vasopressor medication infusion for <24 hours. All antihypertensive medications were discontinued in the immediate (≤4 days) postoperative period in 80% of patients. In conclusion, a systematic and multidisciplinary approach utilizing adrenergic blockade is effective in treating children with CSTs.
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http://dx.doi.org/10.1111/jch.12571 | DOI Listing |
Cureus
December 2024
Internal Medicine, University of Florida College of Medicine, Pensacola, USA.
Extra-adrenal pheochromocytomas are rare neuroendocrine tumors originating outside the adrenal glands and can pose significant diagnostic challenges due to their variable presentations. This report highlights a case of an extra-adrenal pheochromocytoma masquerading as a pancreatic head malignancy. We underscore the importance of considering extra-adrenal pheochromocytoma in the differential diagnosis of pancreatic masses, particularly when biochemical or clinical features suggest catecholamine excess.
View Article and Find Full Text PDFCureus
December 2024
Pediatric Radiology, Detroit Medical Center, Wayne State University, Detroit, USA.
Pheochromocytoma is a catecholamine-secreting tumor that arises from the medullary chromaffin cells but can rarely be extra-adrenal in origin. We present a case of a 16-year-old female patient with uncontrolled hypertension, despite being on lisinopril and metoprolol, and associated left-sided chest pain, recurrent headaches, and an unintentional weight loss of 10 pounds in one month. Laboratory work-up showed a markedly elevated plasma metanephrine level of 4463.
View Article and Find Full Text PDFBMJ Case Rep
December 2024
Cardiology, Université de Fribourg Faculté de médecine, Fribourg, Switzerland
Phaeochromocytomas are rare catecholamine-secreting tumours, usually benign, originating from chromaffin cells of the adrenal glands. Their typical presentation includes the triad of headaches, sweating and tachycardia due to excessive catecholamine release. However, many patients do not exhibit all three symptoms, making diagnosis challenging.
View Article and Find Full Text PDFKidney Blood Press Res
January 2025
Department of Child Health, Faculty of Medicine, Universitas Sriwijaya, Palembang, Indonesia.
Background: Pheochromocytomas and paragangliomas are rare chromaffin cell-derived tumors characterized by catecholamine-secreting activity. Pheochromocytomas account for 1.7% of pediatric hypertension cases.
View Article and Find Full Text PDFJ ASEAN Fed Endocr Soc
December 2024
Department of Endocrinology, Gauhati Medical College and Hospital, Guwahati, India.
Introduction: Pheochromocytomas are catecholamine-secreting tumors arising from chromaffin cells of the adrenal gland. Surgery is the only curative treatment with a high biochemical cure rate, low mortality and high risk of perioperative complications.
Objectives: To study the demographic characteristics of patients with pheochromocytoma and to identify the risk factors for perioperative complications, treatment outcomes, and aggressive behavior of the tumor.
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