A CAUSE OF PAROXYSMAL HYPERTENSION NOT TO BE OVERLOODED: CHILDREN'S PHEOCHROMOCYTOMA

Abstract

Objective: We aimed to assess the clinical presentation, natural history, and treatment response of hypertension in children with pheochromocytoma. Design and method: O. Mohamed, an 8-year-old boy, without past medical history, hospitalized for paroxysmal hypertension evolving for 6 months with severe headaches, generating insomnia. No similar episodes in the family. Clinical features: BMI of 15. 1 kg/m2 (height: 1.26 m, weight: 24 kg). heart rate of 128 bpm (tachycardia) and blood pressure of 175/84 mm Hg (stage 2 for systolic hypertension and stage 1 for diastolic hypertension, table 1). The physical examination was unremarkable except for tenderness in the right hypochondrium. Biological tests: serum creatinine and proteinuria testing was normal. Ophthalmic examination: stage I hypertensive retinopathy (Arterial narrowing). Abdominal ultrasound: right adrenal mass, measuring 65–57–61 mm in size, well-defined, heterogeneous echo structure, with multiple echogenic areas. No calcifications. Abdominal CT scan: Solid right adrenal mass (Figures 1 & 2) and no lymph node involvement. Urinary Vanyl Mandelic Acid: high level at 27.18 μmol/l (normal < 10 μmol/l). MIBG scintigraphy: intense pathological uptake, posterior, projecting over the right renal region, intensifying at 48 hours. No other sites of uptake. Results: In summary, right adrenal pheochromocytoma revealed by a severe paroxysmal hypertension. Preoperative management: Nicardipine IV. Alpha-blocker combined with a beta-blocker were not used. Evolution under medical treatment while waiting for excision of the tumor is favorable with a significant reduction in blood pressure. The child was operated with complete excision of the tumor. Pathological examination of the surgical specimen: ovoid formation made of epithelial cells with basophilic nuclei arranged in cords separated by thin vascular axes = aspect of pheochromocytoma Post-operative evolution was favorable with disappearance of symptoms and normalization of blood pressure after stopping antihypertensives. Actually, the patient is asymptomatic after 4-year follow-up with normal blood pressure and no relapse. The family investigation does not report any family cases. Conclusions: Pheochromocytoma is rare in children. It often presents with severe paroxysmal hypertension. Malignant forms are rare in children; however, rigorous surveillance is necessary for at least 5 years.