Adrenocortical carcinoma in a 6-month-old infant

Abstract

Adrenocortical carcinoma (ACC) is a rare malignant tumor arising from the adrenal cortex and it is even rarer in infancy. These tumors are usually hormonally active. We report a case of ACC in a 6-month-old infant who was brought by her mother with complaints of hirsutism and abnormally looking genitalia. Serum 17-alpha-hydroxyprogesterone and serum cortisol levels were raised. Subsequently, imaging studies revealed a large well-circumscribed soft-tissue mass in the right suprarenal region. The tumor was excised, and on histopathology in conjunction with immunohistochemistry, a diagnosis of ACC was given. Only four other cases of ACC have been reported till date in the literature, with an age of 6 months or younger. Hereby, we present a case of ACC in a young infant.

Keywords

• 17-alpha-hydroxyprogesterone
• adrenocortical carcinoma
• cortisol
• hirsutism