Abstract

Adrenal cortex is a cancer of the adrenal cortex, rare in children; it accounts for 0.2% of pediatric cancers with a ratio of 1.5 in girls and is sometimes part of predisposing syndromes. The clinical signs suggestive of adrenocortical carcinoma are most frequently endocrine, present in 90% of cases. The hormonal increased secretion is made up of cortisol, androgens, estrogen and more rarely, Aldosterone. Symptoms of virilization are most commonly seen and may or may not be associated with Cushing’s syndrome. Arterial hypertension (hypertension) caused by increased secretion of aldosterone is rarer. More rarely, the mode of discovery is the presence of an abdominal mass, most often associated with abdominal pain, or sometimes local signs of compression.
The prognosis of these tumors, as well as the survival rate, is very variable depending on the tumor stage. It is very pejorative for stage III and IV tumors, compared to stage I and II tumors. The 5-year overall survival rate, all stages combined, fluctuates between 49% and 57% depending on the series, but it can vary between 20 and 90% depending on the tumor stages. Surgery is the cornerstone of treatment, hence the question all surgeons ask themselves: the possibility of complete excision. We report the case of an infant, brought to consult for a Genu Varum, who’s clinical and radiological investigations concluded in an adrenal cortex. The treatment was surgical with an anatomopathological study confirming the diagnosis. The outcome was favorable thereafter with regression of endocrine signs over a 6-month follow-up. At the end of this observation, we highlight this very rare pathology as well as its management.

Keywords: Adrenocortical carcinoma; Virilizing tumors; Adrenal tumors; Child