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婴儿期库欣综合征和假性性早熟表现的肾上腺皮质腺瘤。

Adrenocortical adenoma manifesting as Cushing's syndrome and pseudo-precocious puberty in a toddler.

机构信息

Department of Endocrinology and Metabolism, All India Institute of Medical Sciences (AIIMS), New Delhi, India.

Department of Pathology, All India Institute of Medical Sciences (AIIMS), New Delhi, India.

出版信息

Pediatr Endocrinol Diabetes Metab. 2022;28(1):81-87. doi: 10.5114/pedm.2021.109122.

Abstract

Cushing's syndrome is a rare disease in the paediatric age group. Adrenocortical carcinomas (ACC) constitute the most common cause of Cushing's syndrome between 1 and 5 years of age. Often, adrenocortical carcinomas co-secrete other hormones such as androgens (testosterone), deoxy-corticosterone (DOCA), or 17-hydroxy-progesterone [17(OH)P] in addition to cortisol. This may manifest with symptoms and signs of precocious puberty along with Cushing's syndrome. It is rare for a benign adrenocortical adenoma to co-secrete androgens and other hormones in addition to cortisol. Differentiation between adenoma and carcinoma is difficult in all aspects: clinical, radiological, and histopathological. Here, we describe the case of a 2.5-year-old male child who presented with Cushing's syndrome and virilization. Although we suspected ACC clinically, the radiological and histopathological findings were suggestive of benign adrenocortical adenoma. Our case represents the diagnostic challenge that exists in paediatric adrenocortical tumours.

摘要

库欣综合征在儿科中较为罕见。在 1 至 5 岁的儿童中,肾上腺皮质癌(ACC)是库欣综合征最常见的病因。通常情况下,肾上腺皮质癌除了分泌皮质醇外,还会共同分泌其他激素,如雄激素(睾酮)、脱氧皮质酮(DOCA)或 17-羟孕酮[17(OH)P]。这可能会出现性早熟的症状和体征,同时伴有库欣综合征。良性肾上腺皮质腺瘤除了皮质醇外,共同分泌雄激素和其他激素的情况非常少见。在临床、影像学和组织病理学等各个方面,区分腺瘤和癌都很困难。在此,我们描述了一例 2.5 岁的男性儿童,其表现为库欣综合征和男性化。尽管我们临床上怀疑为 ACC,但影像学和组织病理学结果提示良性肾上腺皮质腺瘤。我们的病例代表了儿科肾上腺皮质肿瘤中存在的诊断挑战。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3f3d/10226367/282af4871db3/PEDM-28-45145-g001.jpg

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