Abstract 86: Paraganglioma in a pediatric patient with asymptomatic hypertension- A case report

Introduction: Paraganglioma is a rare type of neuroendocrine neoplasm with the ability to secrete neuropeptides and catecholamines. Hypertension and sympathetic hyperactivity are the most common clinical presentation. Although the incidence of PPGL in the pediatric population is 0.2–0.5 cases per million, they are the most common endocrine tumors in childhood and account for 0.5–1% of pediatric hypertensive cases. (1) Early diagnoses of functional paraganglioma are important because their removal is often curative. We present the case of a young boy with uncontrolled hypertension, who was later found to have functional paraganglioma. Clinical Case: A 11-year-old male child was referred from department of Paediatrics for evaluation of uncontrolled hypertension with headache. There was no history of palpitation, tremor, diaphoresis or chest pain. Family history was negative. Routine investigations and thyroid function tests were with in normal limit. Hormonal evaluations revealed 11 pm S Cortisol 0.68 ug/dl, S aldosterone 13.1 ng/dl, PRA- 5.6 ng/ml/hr, ARR- 2.33, 24 hr Urinary metanephrine 11 3.1 ug/24 hr (normal <350), metanephrine to Cr ratio 202.7 ug/g, Urine normetanephrine 1426 ug/24 hr (normal <600), normetanephrine to Cr ratio 2705.8 ug/g respectively. Fundoscopy suggestive of grade I & grade III hypertensive retinopathy in RE & LE respectively, USG abdomen/pelvis, 2D Echo and Renal Doppler study were normal. CECT adrenal protocol revealed a well defined mild lobulated SOL of size 31 x 19 x 25 mm seen in left para-aortic region, abutting left adrnal and left crus of diaphragm, abutting and superior to left renal vessels, with attenuation of baseline HU +40, arterial phase +120 HU, venous phase +100 HU and delayed +60 HU. Ga-68 DOTANOC PET-CT reveled a well defined globular mass lesion in the left paraaortic area inferior to left adrenal and superior to left renal vessels with size 2.8 x 2.6 x2.6 cm with SUV MAX – 6.77 with increased tracer activity seen in left adrenal that appears minimally bulky as compared to right adrenal, SUV MAX- 6.08. He underwent successful resection of left para-aortic mass. HPE revealed Paraganglioma. His blood pressure became normal without requiring antihypertensive. Conclusion: Paraganglioma should always be included in the differential diagnoses of any atypical presentation of hypertension. Early diagnosis of paraganglioma would fasten the optimal cure, alleviate the symptoms of catecholamine release, and reverse hypertension. Surgery is the cornerstone of treatment in patients with localized disease and it can be performed through an open or a minimally invasive approach.(2) Perioperative management, in this case, needs to be performed with a multidisciplinary board, including an endocrinologist, surgery department, and anesthesiologist; due to the increased risk of hypertensive crisis or episodes of unexpected hypotension, for that reason, medical interventions are focused on maintaining adequate intravascular volume and achieving alpha-beta blocking with anti-hypertensive treatments. (3) Even though there is very limited availability of genetic testing in our country, all patients should be offered genetic testing because of high rate of inheritance of these tumors in paediatrics and should be in regular follow up throughout their lifetime as the risk of recurrence and malignancy.