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Abstract | Full HTML Article | PDF ecancer 7 289 / https://doi.org/10.3332/ecancer.2013.289

Case Report

Adrenocortical carcinoma presenting with heterosexual pseudoprecocious puberty shortly after birth: case report and review

Adrenocortical tumour is rare in children. We report on a female infant with adrenocortical carcinoma presenting with pseudoprecocious puberty at the age of two. She had a history of gradually increasing public hair growth after birth. Physical examination showed signs of virilisation such as pubic hair growth and hirsutism with evidence of facial hair growth. On biochemical evaluation, DHEA-S, 17-OH progesterone, and testosterone levels were elevated. An abdominopelvic spiral computed tomography (CT) scan with intravenous contrast identified a well-defined heterogeneously enhanced mass with areas of necrosis in the right adrenal gland and downward displacement of the underlying kidney. There was no evidence of distant metastasis on CT imaging. An exploratory laparotomy was performed in which a large, haemorrhagic and necrotic mass in the right adrenal gland with pressure effect on right liver lobe and signs of thrombosis in the inferior vena cava was detected. Pathologic examination confirmed the adrenocortical carcinoma. She received eight cycles of adjuvant
chemotherapy with Carboplatin, Etoposide, and Doxorubicin regimens and underwent follow-up visits thereafter in which no sign of recurrence was observed. In conclusion, adrenocortical carcinomas are rare in children, but they should be considered in any child presenting with signs of pseudoprecocious puberty.


Keywords: adrenocortical carcinoma; pseudoprecocious puberty; virilisation; paediatric

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European Cancer Organisation European Institute of Oncology

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Foundazione Umberto Veronesi Fondazione IEO Swiss Bridge

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