Advances in treatment and molecular studies of pediatric pheochromocytoma (PCC) and paraganglioma (PGL): a large single center cohort.

DRA ANA VIEITES; DRA GABRIELA SANSO; IGNACIO DÍAZ SAUBIDET,; MARÍA EUGENIA RODRÍGUEZ, ; HORACIO BIGNON, ; IGNACIO BERGADÁ

Cusco

Congreso; XXVIII Sociedad Latinoamericana de Endocrinología Pediátrica; 2018

SLEP

Advances in Treatment and Molecular Studiesof Pediatric Pheochromocytoma (PCC) andParaganglioma (PGL): A Large Single Center CohortAna Vieites1, Maria Masnata1, Gabriela Sanso1, Ignacio Diaz2,Maria Rodríguez1, Horacio Bignon2, Ignacio Bergadá11Endocrine Research Center ?Dr. César Bergadá? CONICET-FEIDivision of Endocrinology, Children?s Hospital Ricardo Gutierrez,2Department of General Surgery, Unit XV Ricardo GutiérrezChildren?s HospitalPheochromocytoma and paraganglioma are rare Tumors thatcauses 0.5?2% of hypertension in pediatric Patients and Constitutea treatable cause of surgically arterial hypertension. These Tumorsare inherited in as much as 80% in pediatric cases, and all Patientswith Mutations Should be followed closely for the risk of recurrence and malignancy. Advances in surgical treatment and molecular studies Have improved the management of These patients.Aim: To report a large series of pediatric pheochromocytomaand paraganglioma Patients with seen at a single center since 2005to 2017.Results: Patients seventeen (12 ills) were included surgical procedures and 20 Were performed. All Patients HAD catecholamines elevated levels. Eleven Patients HAD unilateral PCC (4 recurrence), bilateral PCC 3 3 2 ADH PGL and simultaneous PGLand PCC. 16/20 In laparoscopic surgeries was performed, One ofThese ended in an open surgery. In all of them intra arterial surgical Control of hypertension was adequate. Four surgeries Weremade with the traditional technique due to the complexity of tumor location. Mean time for laparoscopic surgical approach was134 ± 66 vs 173 ± 92 minutes for open surgical approach (p = 0.22).Time of hospitalization was lower in Patients Significantly WHOunderwent open surgery laparoscopic vs (66.8 ± 13.8 vs 105.6 ± 36hours, respectively, p = 0.04). In all studies Patients genetic Wereperformed. Ten Patients ADH gene mutations in vhl and 4 inSDHB gene. One patient probably had a pathogenic variant in theSDHD gene and a probably benign variant in the SDHB gene. Inconclusion, we highlight that Pediatric Patients with PCC and PGLcan be Treated with a minimally invasive surgical procedure Minimizing the risk of hypertensive crises and lowering days of hospitalization. In Addition, molecular genetics is mandatory In ThesePatients in order to improve increase follow-up