SCIENTIFIC LETTER TO THE EDITOR Large Adrenocortical Carcinoma Presenting as an Adenoma with Precocious Puberty Aliza Mittal & Mukul Aggarwal & Pradeep Debata Received: 12 June 2011 /Accepted: 30 November 2011 /Published online: 16 December 2011 # Dr. K C Chaudhuri Foundation 2011 Sir, We present a case of peripheral precocious puberty due to a large adrenal mass, with hemorrhagic and necrotic areas, despite long history and no metastasis, making the distinc- tion between carcinoma and adenoma very difficult. A 3.5-y-boy presented with progressive enlargement of penis, appearance of pubic hair and hoarseness of voice for 2 y. There was no history of focal neurological deficits, testicular swelling or chronic drug intake. There was no family history of precocious puberty. The child had a mus- cular look, penile length and testicular volume were 8.5 cm and 2.3 cc respectively. Child belonged to Sexual Maturity Rating stage III. He weighed 23 kg, height was 107.5 cm and arm span of 103.7 cm. The blood pressure recorded was 90/60 mmHg which was normal for his age. A 5 cm round- ed, firm, non tender lump in left lumbar region was felt. The bone age was 13–15 y. CECT abdomen revealed a large well defined heterogeneously enhancing necrotic mass involving left adrenal with specs of calcifications, closely abutting spleen and left kidney, however the fat planes were maintained. No evidence of metastatis was found on MRI brain and CT chest. Per-operatively a 13×10 cm tumor was found in left suprarenal area with intact capsule and hemor- rhagic areas (Fig. 1), without any evidence of invasion. Considering the Weiss criteria, weight, size and presence of necrosis and hemorrhage (Fig. 2), diagnosis of adreno- cortical carcinoma was considered. The hormonal profile revealed raised 17 hydroxy proges- terone (5.14 ng/ml), testosterone (30 ng/dl), DHEAS (543 μg/dl) and cortisol (104 μg/ml) levels which came down to serum cortisol level of 48 μg/dl, serum testosterone of 10 ng/dl and serum DHEAS level of 152 μg/dl which were within normal limits 3 mo after surgery. Adrenocortical carcinomas are very rare cause of gonado- tropin independent precocious puberty with an incidence of 1– 2 per million populations annually. It is usually unilateral and seen in females [1]. It has a bimodal age distribution [2]. Clinically, virilization is most common and only 10% present with cushingoid features and palpable mass. In 20– 40% cases, no history of hormonal overproduction is found. Red flag signs for adrenal carcinoma are large size (>4 cm), intra-lesional calcification, hemorrhage, necrosis, tumor Fig. 1 Cut section of the mass showing areas of hemorrhage and necrosis A. Mittal : M. Aggarwal : P. Debata Department of Pediatrics, VMMC and Safdarjung Hospital, New Delhi, India A. Mittal (*) Room No 57, Second Floor, Resident Doctors Hostel, Safdarjung Hospital, New Delhi 110029, India e-mail: alizamittal@gmail.com Indian J Pediatr (June 2012) 79(6):820–821 DOI 10.1007/s12098-011-0645-0