en POLSKI
eISSN: 2083-8441
ISSN: 2081-237X
Pediatric Endocrinology Diabetes and Metabolism
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1/2022
vol. 28
 
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abstract:
Case report

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

Hiya Boro
1
,
Suraj Kubihal
1
,
Rimlee Dutta
2
,
Vijay Kubihal
3
,
Sarah Alam
1
,
Nikhil Tandon
1

1.
Department of Endocrinology and Metabolism, All India Institute of Medical Sciences (AIIMS), New Delhi, India
2.
Department of Pathology, All India Institute of Medical Sciences (AIIMS), New Delhi, India
3.
Department of Radiodiagnosis, All India Institute of Medical Sciences (AIIMS), New Delhi, India
Pediatr Endocrinol Diabetes Metab 2022; 28 (1): 81–87
Online publish date: 2022/02/21
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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.
keywords:

precocious puberty, adrenocortical adenoma, Cushing's syndrome, Wieneke criteria


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