Pheochromocytoma (PCC) is a rare tumor arising from the adrenal medulla as well as extra-adrenal paraganglion system and secreting catecholamines causing severe hypertension in children. The gold standard for diagnosis is the measurement of free plasma levels of metanephrines while management evaluates the location and size of the tumor and possible metastatic lesions. Preoperative treatment with alpha blockers, beta blockers and tyrosine hydroxylase inhibitors improves safety of the surgical procedure.
Objectives: To describe the clinical characteristics, investigation data and outcome of patients with PCC.
Subjects and Methods: this is case study including clinical symptom descriptions, biochemical and imaging investigations, and management for two children with PCC.
Results: The female patient was 7 years old, with no medical history. She presented with vomiting, headache, abdominal pain and convulsions. Blood pressure was 220/190 mmHg and heart rate 130 / min on admission. Abdominal CT showed a left adrenal tumor of 24x25 mm in size. Plasma level of cortisol and noradrenaline were 1374 nmol / L and 971 pg / ml, respectively; and urinary HVA level was 7.8 µmol/mmol of creatinine. Preoperative blood pressure was controlled with nicardipine, doxazosin mesylate and amlodipine. A 2x3 cm tumour was removed through endoscopy. After surgery, patient was stable with normal blood pressure.
Conclusions: PCC is the cause of treatable secondary hypertension. Stabilizing blood pressure prior to surgery, contributes to ensure surgical treatment safety. Multidisciplinary collaboration is warranted to optimize the management of patients.
19 - 21 Sep 2019
European Society for Paediatric Endocrinology