ESPE Abstracts

Introduction: Paediatric adrenocortical tumors (ACT) include both benign adenomas and highly virulent malignant tumors. However, it is very rare among children. The aim of this study is to evaluate the clinicopathological data of children presented with ACT and to assess the survival.

Methods: This is a retrospective cohort study which includes patients diagnosed with ACT from August 2020 to July 2021 and followed up at Lady Ridgeway Hospital, Colombo.

Results: 5 children were diagnosed with adrenal cortical tumors during the study period. The age of them ranged from 10 months to 6 and half years. 4 of them were boys and the oldest one was a girl. All of them were presented with features of gonadotrophin independent precocious puberty; boys with increased stretched penile length, increased body hair and voice changes, and the girl with clitromegaly. One boy phenotypically had features of Beckwith-wiedemann syndrome but not genetically confirmed. The median time duration of diagnosis since the onset of symptoms was 4 months. 3 out of 5 children had rapid height and weight gain. 3 of them had hypertension requiring anti-hypertensives. The preoperative diagnosis was made through clinical manifestations, elevated DHEAS levels and enlarged supra renal masses in CT. All 4 boys had right side supra renal masses while the girl had it on the left side. All underwent surgery for tumor resection. The diagnosis was confirmed based on the histopathology of the adrenal specimens. None of them were malignant tumors. The youngest developed adrenal insufficiency following surgery requiring corticosteroid replacement. To date, all 5 of them are doing well without any disease recurrence.

Discussion: Even though these tumors are rare, an increased number of cases are detected in Brazilian children due to specific gene mutations. There are two peak times for presentation, during the 1st 2 years of life and peripubertally. Girls are more predominant to develop ACT though we have a different sex predominance in our study. Most of the ACT are functional in children and 80-90% of them have endocrine manifestations at the diagnosis. Viralization is the commonest presentation but 10-15% will present with Cushing’s syndrome. Histopathology is essential to distinguish adenomas from carcinomas.

Conclusion: It is important to consider adrenal tumor as a possibility when children present with precocious puberty. To ensure early diagnosis, we need to raise awareness about the signs of viralization among parents and the general-public.