The problem started with abnormally frequent urination, which would worsen at night. The parents of two-year-old Prema (Name changed) at first mistook it as a result of more than normal water intake and subsequently followed a measured regime as well. The mother also noticed that her daughter was developing hair in her private parts, and her voice was hoarse.

Worried parents reached out to the Gynaecologist, who ruled out any gynaecological correlation and referred Prema to Dr Abhishek Kulkarni, Paediatric Endocrinologist at SRCC Children’s Hospital, managed by Narayana Health. Staging investigations showed that the tumour was localised to the adrenal gland only.

Dr. Kulkarni diagnosed Prema with Hypertension and early signs of virilisation, a condition that initiates the development of masculine characteristics through the sensitisation of male hormones like androgens.

Further investigation identified the presence of a large tumour in the right adrenal gland, and she was referred to Dr Ruchira Misra, Paediatric Haemato-oncologist, followed by Dr Rasik Shah, Paediatric General and Laparoscopic Surgeon at SRCC Children’s Hospital, managed by Narayana Health.

After an extensive discussion among the paediatric specialists, it was decided to remove the tumour laparoscopically. Key-hole surgery was performed, and a tumour measuring 6 cm in diameter and weighing 68 grams was extracted. Later biopsy confirmed it as a case of Adrenocortical Carcinoma, a rare form of cancer and that too for a child, with a prevalence of only one to two cases per million in paediatric age group.

It has been observed that any adrenal tumour weighing more than 250 grams, along with symptoms of marked hormone overproduction, detected in children above three and half years, is to be considered malignant with a poor prognosis.

Pure virilising carcinomas generally appear to have a better prognosis than other adrenal carcinomas. And if removed completely in the early stage, patients have a good prognosis as well. Prema is kept in follow-up to detect any recurrence in the early stage by doing regular blood tests to monitor the hormone cortisol level.

Name:	Dr. Abhishek Kulkarni
Specialty:	Paediatric Endocrinologist
Expertise:	Growth Disorders Pubertal Disorders Obesity Juvenile Diabetes Metabolic Bone Diseases Disorders of Sex Development
Name:	Dr. Rasik Shah
Specialty:	Paediatric General & Laparoscopic Surgeon
Expertise:	Laparoscopy Oncosurgery Urology