Endocrine Abstracts

Introduction: Adrenocortical carcicnoma (ACC) is a rare malignancy with overall incidence of 0.7–2.0 cases/million. It’s a rare & aggressive childhood cancer with a reported incidence of 0.2– 0.3 new cases per 1 million. It has Bimodal distribution - at first decade - 85% functional & 5-6^th decade of life 15-30% functional. Paediatric virilising adrenal tumours have a better prognosis after complete resection than in adults. Surgery is the mainstay of treatment. Even after complete resection, a high risk of recurrence of ACC remains. 2yrs 3months – 1^st born male child brought by parents with complaints of appearance of pubic & axillary hair, enlargement of both breasts, accelerated height gain, increase in penile length without any increase in testicular size. On examination a palpable mass was noticed in right hypochondrium which was firm in consistency. A provisional diagnosis of Gonadotropin independent precocity (GIPP) due to adrenal mass lesion was made. Biochemical and Radiological evaluation was suggestive of Adrenocortical cancer. Subsequently child underwent Right adrenalectomy. Postop was uneventful and he was discharged on Day 6.

Discussion: Here is a male child with Premature development of axillary, pubic hair with features of androgenisation, Increase in penile length without Increase in testes size, with B/l Gynaecomastia, accelerated Ht gain, with palpable mass in RUQ which Implies -Precocious Puberty of Peripheral origin (Gonadotropin Independent) due to Adrenal neoplasm.

Conclusion: Though ACC is a rare cause of GIPP in paediatric age group, this entity should be considered in differential diagnosis.