Successful Treatment of a Locally Advanced Adrenocortical Carcinoma With Neoadjuvant Chemotherapy and Surgical Resection: A Report of a Pediatric Case

Abstract

Pediatric adrenocortical carcinoma is a malignant tumor with poor prognosis. We report a case of an infant with advanced adrenocortical carcinoma that was safely and completely resected after neoadjuvant chemotherapy. A five-month-old girl with an abdominal mass was admitted to our hospital. Computed tomography showed a right adrenal tumor with direct invasion into the liver and lymph node metastasis. The diagnosis of right adrenocortical carcinoma was obtained through an incisional biopsy. Neoadjuvant chemotherapy with mitotane and antitumor agents successfully reduced the volume of the tumor and resulted in a partial response. The right adrenal tumor and posterior segment of the liver were resected en bloc. Paraaortic and right renal hilar lymphadenectomy were additionally performed. Histopathology showed viable tumor cells in the primary tumor as well as in the lymph nodes. After additional courses of adjuvant chemotherapy, the patient remains relapse-free for two years and seven months. Complete resection of the tumor is essential in the treatment of pediatric adrenocortical carcinoma. On the other hand, the efficacy of chemotherapy has not been clarified. Although the long-term prognosis of advanced cases reported in our country was generally poor, chemotherapy tended to improve the prognosis. Notably, the combination of mitotane and antitumor agents correlated significantly with prolonged survival. Neoadjuvant chemotherapy may reduce tumor size and enable a safe and complete resection in locally advanced cases.