Vurallı Doğuş, Kandemir Nurgün, Clark Graeme, Orhan Diclehan, Alikaşifoğlu Ayfer, Gönç Nazlı, Ekinci Saniye, Özön Alev
Division of Pediatric Endocrinology Departments of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.
University of Cambridge and NIHR Cambridge Biomedical Research Center, Department of Medical Genetics, Cambridge, United Kingdom.
Turk J Pediatr. 2017;59(2):200-206. doi: 10.24953/turkjped.2017.02.015.
Vurallı D, Kandemir N, Clark G, Orhan D, Alikaşifoğlu A, Gönç N, Ekinci S, Özön A. A pheochromocytoma case diagnosed as adrenal incidentaloma. Turk J Pediatr 2017; 59: 200-206. There are two problems that needs to be addressed in cases of an adrenal incidentaloma. The first 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. A 17-year-old male was admitted with the complaint of progressive weight gain. Abdominal ultrasonography was performed for elevation in transaminases which revealed a hypoechoic mass located in the left adrenal gland. Hormonal investigations revealed an increase in fractionated catecholamine and metanephrine levels in 24-hour urine. Surgery was performed and pathological examination was in accordance with pheochromocytoma. Mutation analysis was carried out. This is a rare case of pheochromocytoma presenting as adrenal incidentaloma during adolescence. In view of this case, we review the approach to incidentally discovered adrenal masses and the approach to pheochromocytoma. A mutation analysis should be performed on all cases with pheochromocytoma that are diagnosed below age 20.
武拉尔利·D、坎德米尔·N、克拉克·G、奥尔汉·D、阿利卡希福卢·A、贡奇·N、埃金奇·S、厄佐恩·A。一例被诊断为肾上腺偶发瘤的嗜铬细胞瘤病例。《土耳其儿科学杂志》2017年；59: 200 - 206。肾上腺偶发瘤病例有两个问题需要解决。第一个是确定肾上腺肿块是良性还是恶性，第二个是确定肿块是否具有激素活性。一名17岁男性因进行性体重增加主诉入院。因转氨酶升高进行腹部超声检查，发现左肾上腺有一个低回声肿块。激素检查显示24小时尿中去甲肾上腺素和间甲肾上腺素水平升高。进行了手术，病理检查符合嗜铬细胞瘤。进行了突变分析。这是一例青春期罕见的以肾上腺偶发瘤形式出现的嗜铬细胞瘤病例。鉴于此病例，我们回顾了偶然发现的肾上腺肿块的处理方法以及嗜铬细胞瘤的处理方法。对于所有20岁以下诊断为嗜铬细胞瘤的病例都应进行突变分析。
