Adrenocortical Carcinoma with Precocious Puberty in a 34-month-old Boy

Introduction: Adrenocortical carcinoma (ACC) is rare condition in childhood. Clinical presentation includes, most commonly, signs of virilization due to hormones secretion. We report a case of precocious puberty in 34-month-old boy.

Case report: A 34-month-old Tunisien boy, with no medical past history, was admitted for exploration of pubic hair development. Clinical examination showed precocious puberty (Tanner stage T1 P2) well developed penile (length: 10 cm) with an accelerated growth curve (height: +2, 9 SD). No associated malformations were found. The bone age was 7 years. The hormonal profile revealed raised testosterone (9,6 nmol/l).The abdominal computed tomography (CT) revealed a large right adrenal mass measuring 5*5*7,3 cm with peripheral calcifications repressing the kidney with pre-aortic and mediastinal lymphadenopathy. No evidence of metastasis was found. Adrenocortical carcinoma with an androgen secretion was suspected. Surgical treatment consisted on adrenalectomy. Pathological examination confirms the diagnostic of ACC with a Weiss score at 5.The postoperative course was uneventful, with partial regression of the pubertal signs and normalization of the testosterone 5 months after surgery.

Conclusion: Adrenocortical carcinomas are very rare cause of gonadotropin independent precocious puberty. It can be found at any age, even in very young children. Treatment consists of surgical resection which should be complete without shredding of the tumor. Diagnosis can be very complicated.