Background: Adrenocortical tumor is very rare in the pediatric age group. These tumors may cause peripheric precocious puberty, Cushings syndrome or both. It is seen most commonly in children under 5 years of age and fourth decade. p53 mutation and other pathologies that may accompany should be investigated especially in young children.
Objective and hypotheses: A 18-month-old boy was brought with pubarche and phallic enlargement, and was noticed 6 months ago for the first time by parents. Parents defined growth spurt and erections. His medical records and family history were unremarkable. Chronological age 1.6 years, height: 86 cm (78 p+0.80 SDS), weight 14.5 kg (95 p), target height SDS −1.65, testiculary volume 2 ml/2 ml, streched penil lenght 6 cm (9097 p), and pubarche grade 2. There was no other findings of hyperandrogenism. Laboratory results were consistent with peripheric precocious puberty FSH: <0.3 U/l, LH: 0.05 U/l, total testosterone: 473 ng/dl, 17-OH P: 0.5 ng/dl, DHEA-SO4: 206 μg/dl, and androstenedione: 3.35 ng/ml. Thyroid functions, α-fetoprotein ve βHCG were normal. A mass with 22×17 mm was detected ultrasonogrphically in right adrenal gland and being 35 Hounsfield Unit, the mass was considered as nonadenomatosis. Tumor weighted 50 g with intact capsula after adrenalectomy. Pathological diagnosis was adrenocortical carcinoma and no metastasis was detected with PETCT scanning. p53 mutation analysis is being tested. Androgen hormones decreased to prepubertal levels in second day postoperatively. Mass originated from soft tissue was detected third month after adrenalectomy and resected material was consistent with rhabdomyosarcoma histopathologically.
Conclusion: Simple radiologic methods should be selected in peripheric precocious puberty before further complicated investigetions. Besides it is noteworthy to be careful for comorbidities such as other malignancy especially in young children.
20 - 22 Sep 2014
European Society for Paediatric Endocrinology