Keywords
Pheochromocytoma
Child
How to Cite
Abstract
Arterial hypertension (AH) is an increasing trend in pediatrics. Timely diagnosis and treatment can help identify underlying causes and prevent both acute and long-term complications. Pheochromocytomas and paragangliomas are rare causes of AH in children.
The objective is to report a case of AH secondary to a genetically determined pheochromocytoma, associated with ipsilateral renal atrophy.
We present the case of a 9-year-old asymptomatic school-age girl with severe chronic AH, normal kidney function, and no target organ damage. Ultrasound and angiography revealed a nodule in the right adrenal topography. A DMSA scan showed 12% uptake in the right kidney. Urinary metanephrines and renin levels were markedly elevated. PET confirmed the presence of a pheochromocytoma without dissemination.
After a gradual decrease in blood pressure, surgical resection was performed. Histopathological analysis confirmed the diagnosis and its benign nature. Genetic testing identified the pathogenic variant c.72+1G>A in the SDHB gene. Postoperatively, AH persisted, but metanephrine levels normalized. Doppler ultrasound showed hypovascularization in the upper half of the kidney, and DMSA uptake decreased to 9%. A nephrectomy was performed. Blood pressure normalized in the office setting, but ambulatory blood pressure monitoring remained abnormal. Multidisciplinary follow-up is essential for this monorenal patient, who presents with masked AH and remains at risk for tumor recurrence and malignancy.
References
Flynn J, Kaelber D, Baker C, Blowey D, Carroll A, Daniels S, et al; Subcommittee on Screening and Management of High Blood Pressure in Children. Clinical practice guideline for screening and management of high blood pressure in children and adolescents. Pediatrics 2017; 140(3):e20171904. doi: 10.1542/peds.2017-1904. Erratum in: Pediatrics 2017; 140(6):e20173035. doi: 10.1542/peds.2017-3035. Erratum in: Pediatrics 2018; 142(3):e20181739. doi: 10.1542/peds.2018-1739.
Lurbe E, Agabiti E, Cruickshank J, Dominiczak A, Erdine S, Hirth A, et al. 2016 European Society of Hypertension guidelines for the management of high blood pressure in children and adolescents. J Hypertens 2016; 34(10):1887-920. doi: 10.1097/HJH.0000000000001039.
Caggiani M, Farre Y. 3er Consenso uruguayo de hipertensión arterial en el niño y el adolescente. Arch Pediatr Urug 2006; 77(3):300-5.
Boggia J, Lujambio I, Noboa A. Hipertensión arterial. En: Boggia J, Malacrida L, Luzardo L. Fisopatología: mecanismos de las disfunciones orgánicas. Montevideo: BiblioMédica, 2017:137-58.
Pamporaki C, Hamplova B, Peitzsch M, Prejbisz A, Beuschlein F, Timmers H, et al. Characteristics of pediatric vs adult pheochromocytomas and paragangliomas. J Clin Endocrinol Metab 2017; 102(4):1122-32. doi: 10.1210/jc.2016-3829.
Neumann H, Young WJr, Eng C. Pheochromocytoma and Paraganglioma. N Engl J Med 2019; 381(6):552-65. doi: 10.1056/NEJMra1806651.
Kota S, Kota S, Meher L, Tripathy P, Sruti J, Modi K. Pheochromocytoma with renal artery stenosis: a case-based review of literature. J Cardiovasc Dis Res 2012; 3(1):36-9. doi: 10.4103/0975-3583.91601.
Chandra V, Thompson G, Bower T, Taler S. Renal artery stenosis and a functioning hilar paraganglioma: a rare cause of renovascular hypertension-a case report. Vasc Endovascular Surg 2004; 38(4):385-90. doi: 10.1177/153857440403800413.
Bholah R, Bunchman T. Review of pediatric pheochromocytoma and paraganglioma. Front Pediatr 2017; 5:155. doi: 10.3389/fped.2017.00155.
Sarathi V, Bandgar T, Lila A, Deshpande A, Dalvi A, Patwardhan S, et al. Coexistence of pheochromocytoma/praganglioma and renal artery stenosis. Indian J Endocrinol Metab 2012; 16(6):1009-11. doi: 10.4103/2230-8210.103022.
Rednam S, Erez A, Druker H, Janeway K, Kamihara J, Kohlmann W, et al. Von Hippel-Lindau and hereditary pheochromocytoma/paraganglioma syndromes: clinical features, genetics, and surveillance recommendations in childhood. Clin Cancer Res 2017; 23(12):e68-e75. doi: 10.1158/1078-0432.CCR-17-0547.
Instituto Nacional del Cáncer. Feocromocitoma y paragangliom: versión para profesionales de salud. Bethesda, MD: INC, 2023. Disponible en: https://www.cancer.gov/espanol/tipos/feocromocitoma/pro. (Consulta: 21 noviembre 2023).
Lenders J, Eisenhofer G, Mannelli M, Pacak K. Phaeochromocytoma. Lancet 2005; 366(9486):665-75. doi: 10.1016/S0140-6736(05)67139-5.
Januszewicz P, Wieteska A, Wyszynska T. Pheochromocytoma in children: difficulties in diagnosis and localization. Clin Exp Hypertens A 1990; 12(4):571-9. doi: 10.3109/10641969009073485.
Oleaga A, Goñi F. Feocromocitoma: actualización diagnóstica y terapéutica. Endocrinol Nutr 2008; 55(5):202-16. doi: 10.1016/S1575-0922(08)70669-7.
Achilli L, Bertoglio V. Feocromocitoma. Rosario, ARG: UNR, Cátedra de Anatomía y Fisiología Patológicas, Unidad de ciencias médicas, 2011. Disponible en: http://www.patologiafcm.com.ar/feocromocitoma. (Consulta: 21 noviembre 2023).
van Nederveen F, Gaal J, Favier J, Korpershoek E, Oldenburg R, de Bruyn E, et al. An immunohistochemical procedure to detect patients with paraganglioma and phaeochromocytoma with germline SDHB, SDHC, or SDHD gene mutations: a retrospective and prospective analysis. Lancet Oncol 2009; 10(8):764-71. doi: 10.1016/S1470-2045(09)70164-0.
Coulthard M. Managing severe hypertension in children. Pediatr Nephrol 2023; 38(10):3229-39. doi: 10.1007/s00467-023-05896-z.
Vaca L, Zayas H, García M, Granados J. Manejo anestésico de emergencia hipertensiva perioperatoria secundaria a tumores neuroendocrinos. Rev Fac Med (Méx) 2016; 59(6):27-31.
Mamilla D, Araque K, Brofferio A, Gonzales M, Sullivan J, Nilubol N, et al. Postoperative management in patients with pheochromocytoma and paraganglioma. Cancers (Basel) 2019; 11(7):936. doi: 10.3390/cancers11070936.
Weidmann P, Siegenthaler W, Ziegler W, Sulser H, Endres P, Werning C. Hypertension associated with tumors adjacent to renal arteries. Am J Med 1969; 47(4):528-33. doi: 10.1016/0002-9343(69)90183-1.
Hermida A, Lazaré H, López J, Calvo C. Hipertensión renovascular secundaria a feocromocitoma. Rev Clin Esp 2012; 212(9):467-73. doi: 10.1016/j.rce.2012.03.005.
Mitsnefes M, Wühl E. Role of hypertension in progression of pediatric CKD. Pediatr Nephrol 2023; 38(11):3519-28. doi: 10.1007/s00467-023-05894-1.
Flynn J, Urbina E, Brady T, Baker C, Daniels S, Hayman L, et al. Ambulatory blood pressure monitoring in children and adolescents: 2022 update: a scientific statement from the American Heart Association. Hypertension 2022; 79(7):e114-24. doi: 10.1161/HYP.0000000000000215.
Perrin E, Ravi H, Borra G, South A. Prevalence and risk factors of disordered eating behavior in youth with hypertension disorders. Pediatr Nephrol 2023; 38(11):3779-89. doi: 10.1007/s00467-023-05921-1.
Jochmanova I, Abcede A, Guerrero R, Malong C, Wesley R, Huynh T, et al. Clinical characteristics and outcomes of SDHB-related pheochromocytoma and paraganglioma in children and adolescents. J Cancer Res Clin Oncol 2020; 146(4):1051-63. doi: 10.1007/s00432-020-03138-5.
Strajina V, Dy B, Farley D, Richards M, McKenzie T, Bible K, et al. Surgical treatment of malignant pheochromocytoma and paraganglioma: retrospective case series. Ann Surg Oncol 2017; 24(6):1546-50. doi: 10.1245/s10434-016-5739-5.
Children's Oncology Group. Long-term follow-up guidelines for survivors of childhood, adolescent, and young adult cancers. Version 6.0 (October 2023). Disponible en: http://www.survivorshipguidelines.org. (Consulta: 21 noviembre 2023).
Weingarten T, Welch T, Moore T, Walters G, Whipple J, Cavalcante A, et al. Preoperative levels of catecholamines and metanephrines and intraoperative hemodynamics of patients undergoing pheochromocytoma and paraganglioma resection. Urology 2017; 100:131-8. doi: 10.1016/j.urology.2016.10.012.
Butz J, Weingarten T, Cavalcante A, Bancos I, Young WJr, McKenzie T, et al. Perioperative hemodynamics and outcomes of patients on metyrosine undergoing resection of pheochromocytoma or paraganglioma. Int J Surg 2017; 46:1-6. doi: 10.1016/j.ijsu.2017.08.026.
This work is licensed under a Creative Commons Attribution 4.0 International License.
Copyright (c) 2025 Archives of Pediatrics of Uruguay