Subbiah Sridhar, Nahar Uma, Samujh Ram, Bhansali Anil
Department of Endocrinology, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Ann Saudi Med. 2013 May-Jun;33(3):294-7. doi: 10.5144/0256-4947.2013.294.
Adrenocortical oncocytomas are extremely rare, and most of the tumors are benign and nonfunctioning. To our knowledge, only 30 cases have been reported in English published studies, and most patients are 40 to 60 years of age. So far, in the pediatric age group, only three cases of functioning adrenocortical oncocytoma have been reported. We report a case of functioning adrenocortical oncocytoma in a 3 1/2-year-old female child who presented with premature pubarche, clitoromegaly, and increased serum dehydroepiandrosterone sulfate and testosterone. She was managed successfully with right adrenalectomy, and the tumor histology was consistent with adrenal oncocytoma.
肾上腺皮质嗜酸性细胞瘤极为罕见，大多数肿瘤为良性且无功能。据我们所知，英文发表的研究中仅报道了30例，大多数患者年龄在40至60岁之间。迄今为止，在儿童年龄组中，仅报道了3例功能性肾上腺皮质嗜酸性细胞瘤。我们报告一例3岁半女童功能性肾上腺皮质嗜酸性细胞瘤，该患儿表现为青春期过早发育、阴蒂肥大，血清硫酸脱氢表雄酮和睾酮升高。她接受了右侧肾上腺切除术并成功治愈，肿瘤组织学与肾上腺嗜酸性细胞瘤相符。
