Adrenocortical carcinoma presenting with heterosexual pseudo precociouspuberty shortly after birth: case report and review

Adrenocortical tumour is rare in children. We report on a female infant with adrenocorticalcarcinoma presenting with pseudoprecocious puberty at the age of two. She had a history ofgradually increasing public hair growth after birth. Physical examination showed signs ofvirilisation such as pubic hair growth and hirsutism with evidence of facial hair growth. Onbiochemical evaluation, DHEA-S, ۱۷-OH progesterone, and testosterone levels were elevated. Anabdominopelvic spiral computed tomography (CT) scan with intravenous contrast identified awell-defined heterogeneously enhanced mass with areas of necrosis in the right adrenal gland anddownward displacement of the underlying kidney. There was no evidence of distant metastasis onCT imaging. An exploratory laparotomy was performed in which a large, haemorrhagic andnecrotic mass in the right adrenal gland with pressure effect on right liver lobe and signs ofthrombosis in the inferior vena cava was detected. Pathologic examination confirmed theadrenocortical carcinoma. She received eight cycles of adjuvant chemotherapy with Carboplatin,Etoposide, and Doxorubicin regimens and underwent follow-up visits thereafter in which no signof recurrence was observed. In conclusion, adrenocortical carcinomas are rare in children, but theyshould be considered in any child presenting with signs of pseudoprecocious puberty.

Keywords:

adrenocortical carcinoma , pseudoprecocious puberty , virilisation , paediatric