Treatment of Pediatric Adrenocortical Carcinoma With Surgery, Retroperitoneal Lymph Node Dissection, and Chemotherapy: The Children's Oncology Group ARAR0332 Protocol.

ABSTRACT:

Purpose

Adrenocortical carcinoma (ACC) is a rare aggressive pediatric malignancy with distinct biology. Its treatment follows the principles developed for adults; pediatric-specific studies are scarce.

Patients and methods

Prospective single-arm risk-stratified interventional study. Study objectives were (1) to describe the outcome of patients with stage I ACC treated with adrenalectomy alone; (2) to describe the outcome of stage II patients (completely resected > 200 cc or > 100 g) treated with adrenalectomy and retroperitoneal lymph node dissection; and (3) to describe the outcome of patients with stage III or IV treated with mitotane and chemotherapy.

Results

Between September 2006 and May 2013, 78 patients (77 eligible, 51 females) were enrolled. The 5-year event-

SUBMITTER: Rodriguez-Galindo C 

PROVIDER: S-EPMC8462560 | biostudies-literature | 2021 Aug

REPOSITORIES: biostudies-literature