Background: There are two main problems that should be solved when encountering adrenal incidentaloma. The first of these is to decide whether the adrenal mass is benign or malignant and the second is to determine whether the mass is hormonally active or not.
Objective and hypotheses: We aimed to focus on the approach to adrenal masses in childhood.
Method: A case of phaeochromocytoma, which was diagnosed as incidentaloma is presented.
Results: A 17-year-old male patient was admitted with complaint of weight gain. Liver function tests were high and an abdominal ultrasonography was requested. In the ultrasonography, a uniform 60×51 mm hypoechoic mass was detected, localized in the left adrenal gland, in addition to Grade 2 steatosis and hepatomegaly. The abdominal tomography revealed the mass that consisted of areas of cystic necrosis and had dense heterogeneous contrast-enhancement. Plasma ACTH levels, serum cortisol levels, and 24-h urine free cortisol levels, which were examined for subclinical Cushings syndrome were within normal ranges. Serum potassium, renin, and aldosterone levels, which were examined for primary hyperaldosteronism, and DHEA-S, testosterone, 17-OH progesterone levels, which were examined for androgen-secreting tumour, were all normal. A two- to three-fold increase was detected in fractionated catecholamine and metanephrine levels in 24-h urine, and the patient was diagnosed with phaeochromocytoma. As the patients blood pressure values were accepted as hypertensive in 24-h blood pressure monitorization, selective α1 antagonist was initiated. After the preoperative preparation period, a left adrenelectomy was performed, and the pathology results were consistent with phaeochromocytoma. The patient was screened in terms of accompanying syndromes (MEN type 2, Von Hippel-Lindau Disease, Neurofibromatosis type 1, familial phaeochromocytoma/paraganglioma syndromes) and no positive findings were detected.
Conclusion: The present case is a rare case of phaeochromocytoma that was diagnosed as adrenal incidentaloma in childhood. Phaeochromocytoma and Cushings syndrome should be excluded in all cases with adrenal incidentaloma. Primary aldosteronism should be excluded in patients with hypertensive and/or hypokalemic episodes. A mutation analysis should be conducted on all cases with phaeochromocytoma that are diagnosed below age 20, without considering the presence of syndromic or familial characteristics.
01 - 03 Oct 2015
European Society for Paediatric Endocrinology