Intensive chemotherapy with autologous PBSCT for advanced adrenocortical carcinoma in a child.

Adrenocortical carcinoma (ACC) is a rare tumor in children. Surgical excision is the most effective therapy for ACC. However, the prognosis of ACC with metastases or unresectability is poor and the therapies for these are not well established. We report successful treatment of a 3-year-old girl with ACC and lung metastases. She had adrenalectomy followed by chemotherapy with cisplatinum, etoposide, and bleomycin. Thoracotomy for lung metastases was done after partial response to chemotherapy. Autologous peripheral blood stem-cell transplantation was performed after preconditioning with carboplatinum, etoposide, and melphalan. She has been well for 2 years since the time of diagnosis. Surgical resection of the primary and metastatic lesions with chemotherapy followed by autologous peripheral blood stem-cell transplantation could be an effective treatment for advanced ACC. However, prospective-controlled studies are necessary for firm conclusion as this is a single case report and the follow-up is not long enough.