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

Hiya Boro, Suraj Kubihal, Rimlee Dutta, Vijay Kubihal, Sarah Alam, Nikhil Tandon
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引用次数: 1

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.

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表现为库欣综合征和幼儿性早熟的肾上腺皮质腺瘤。
库欣综合征在儿科年龄组是一种罕见的疾病。肾上腺皮质癌(ACC)是1 - 5岁儿童库欣综合征最常见的病因。通常,肾上腺皮质癌除皮质醇外还会分泌其他激素,如雄激素(睾酮)、脱氧皮质酮(DOCA)或17-羟基孕酮[17(OH)P]。这可能表现为伴随库欣综合征的性早熟的症状和体征。这是罕见的良性肾上腺皮质腺瘤共同分泌雄激素和其他激素,除了皮质醇。腺瘤和癌的鉴别在临床、放射学和组织病理学各方面都很困难。在这里,我们描述了一个2.5岁的男孩谁提出了库欣综合征和男性化的情况。虽然我们在临床上怀疑ACC,但放射学和组织病理学结果提示为良性肾上腺皮质腺瘤。我们的病例代表了存在于儿科肾上腺皮质肿瘤的诊断挑战。
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来源期刊
Pediatric Endocrinology, Diabetes and Metabolism
Pediatric Endocrinology, Diabetes and Metabolism Medicine-Pediatrics, Perinatology and Child Health
CiteScore
2.00
自引率
0.00%
发文量
36
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