A Rare Case of Childhood Adrenocortical Carcinoma in a 4-years-old female child presented with features of Cushing Syndrome: Case report and Literature review

Abstract Childhood adrenocortical carcinoma is an extremely rare cancer with a poor prognosis. It usually presents during the first 5 years of life with a median age of 3–4 years, although there is a second smaller peak during the adolescence period. Here, we report a 4 – years-old female child diagnosed with childhood adrenocortical carcinoma with distant metastasis after she presented with features of Cushing syndrome and recent worsening of abdominal swelling of a month duration. Chest and abdomen CT scan showed a left adrenal mass with liver and lung metastasis. Serum ACTH was low and serum cortisol was high. Biopsy from the liver showed secondary deposits with malignant carcinoma. She started treatment with chemotherapy with EDP regimenwith palliative intent. Despite chemotherapy she had progression of disease with systemic multiorgan involvement.