Large Adrenocortical Carcinoma Presenting as an Adenoma with Precocious Puberty
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 distinction 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 muscular 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 rounded, 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 13x10 cm tumor was found in left suprarenal area with intact capsule and hemorrhagic areas (Fig. 1) without any evidence of invasion. Considering the Weiss criteria, weight, size and presence of necrosis and hemorrhage (Fig. 2) diagnosis of adrenocortical carcinoma was considered. The hormonal profile revealed raised 17 hydroxy progesterone (5.14 ng/ml) testosterone (30 ng/dl) DHEAS (543 mg/dl) and cortisol (104 mg/ml) levels which came down to serum cortisol level of 48 mg/dl, serum testosterone of 10 ng/dl and serum DHEAS level of 152 mg/dl which were within normal limits 3 mo after surgery. Adrenocortical carcinomas are very rare cause of gonadotropin 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
The Indian Journal of Pediatrics