WIP 55 - Pediatric Pheochromocytoma and Paraganglioma: Updates and Practice Patterns on Blood Pressure Management

Publication Number: WIP 55.7470

Francis V. Lomanta, University of Utah School of Medicine, Salt Lake City, UT, United States; Amy C. Wilson, Indiana University School of Medicine, Indianapolis, IN, United States; Sabina Kennedy, Emory University School of Medicine, Atlanta, GA, United States; Laurence A. Greenbaum, Emory University School of Medicine, Decatur, GA, United States; Amrish Jain, Central Michigan University College of Medicine, Detroit, MI, United States; Mahmoud Kallash, Nationwide Children's Hospital, Columbus, OH, United States; Ahmed Zeid, UH Rainbow Babies & Children's Hospital, Cleveland, OH, United States; Melissa Muff-Luett, University of Nebraska Medical School, Omaha, NE, United States; Stefan G. Kiessling, Kentucky Children's Hospital, Lexington, KY, United States; Ei Khin, Texas Tech University Health Sciences Center Paul L. Foster School of Medicine, El Paso, TX, United States; Afsana Jahan, Northwell health cohen children medical center, Long Island, NY, United States; Andrew M.. South, Wake Forest School of Medicine of Wake Forest Baptist Medical Center, Winston Salem, NC, United States; Sherene Mason, University of Connecticut School of Medicine, Hartford, CT, United States; Ikuyo Yamaguchi, The University of Oklahoma Health Sciences Center, Department of Pediatrics, Oklahoma City, OK, United States; Meredith L.. Seamon, University of Utah School of Medicine, Salt Lake City, UT, United States

Background: Pheochromocytomas and paragangliomas (PPGL) are rare catecholamine-secreting tumors with a yearly incidence rate of 1 per 300,000 in adults and 0.2 per 300,000 in children. The definitive treatment is surgical resection. Early surgical attempts noted a 50% intraoperative mortality risk in adults, as tumor manipulation resulted in catecholamine surges with hemodynamic instability. The advent of preoperative blood pressure management has significantly reduced mortality rates. Current preoperative blood pressure management recommendations include alpha-adrenergic blockade followed by beta-blockade for reflex tachycardia. The non-selective alpha-blocker, phenoxybenzamine, has classically been used as the agent of choice. However, some institutions prefer the selective alpha-blockers prazosin and doxazosin due to their lower cost, better availability, and reduced side effect profile. Recent evidence has shown no difference in mortality between the non-selective and selective alpha-blockers.

Objective: To highlight current practice patterns in pediatric PPGL clinical management and compare outcomes between the use of non-selective vs. selective alpha blockade

Design/Methods: This was a retrospective study of 69 patients from 13 participating centers within the Pediatric Nephrology Research Consortium (PNRC). Local IRB approval and data transfer agreements were obtained before chart review and data exchange. Patients diagnosed with PPGL at less than 17 years old from 01/01/2007 to 11/01/2024 were included. Data collected included demographics, family history, genetic testing, diagnostic imaging, serum chemistries, urine and plasma catecholamines and metanephrines, preoperative blood pressures, pre and postoperative antihypertensives used, intraoperative course, ICU course, and length of stay. The analysis will highlight the clinical practice variability between centers, including diagnostics and preoperative blood pressure management. A comparison of intra and postoperative hemodynamic instability rates between non-selective and selective alpha blockade will be calculated.