Unusual Cancers of Childhood Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Abdominal Cancers
Treatment of adrenocortical tumors
At the time of diagnosis, two-thirds of pediatric patients have limited disease (tumors can be completely resected), and the remaining patients have either unresectable or metastatic disease.
Treatment of childhood adrenocortical tumors has evolved from the data derived from the adult studies, and the same guidelines are used; surgery is the most important mode of therapy, and mitotane and cisplatin-based regimens, usually incorporating doxorubicin and etoposide, are recommended for patients with advanced disease.[7,26,27] An aggressive surgical approach of the primary tumor and all metastatic sites is recommended when feasible. Because of tumor friability, rupture of the capsule with resultant tumor spillage is frequent (approximately 20% of initial resections and 43% of resections after recurrence).[3,10] When the diagnosis of adrenocortical tumor is suspected, laparotomy and a curative procedure are recommended rather than fine-needle aspiration, to avoid the risk of tumor rupture. Laparoscopic resection is associated with a high risk of rupture and peritoneal carcinomatosis; thus, open adrenalectomy remains the standard of care.
Little information is available about the use of mitotane in children, although response rates appear to be similar to those seen in adults.[1,26] A retrospective analysis in Italy and Germany identified 177 adult patients with adrenocortical carcinoma. Recurrence-free survival was significantly prolonged by the use of adjuvant mitotane. Benefit was present with 1 to 3 g per day of mitotane and was associated with fewer toxic side effects than doses of 3 to 5 g per day. In a review of 11 children with advanced adrenocortical tumors treated with mitotane and a cisplatin-based chemotherapeutic regimen, measurable responses were seen in seven patients. The mitotane daily dose required for therapeutic levels was around 4 g/m2, and therapeutic levels were achieved after 4 to 6 months of therapy.
The use of radiation therapy in pediatric patients with adrenocortical tumors has not been consistently investigated. Adrenocortical tumors are generally considered to be radioresistant. Furthermore, because many children with adrenocortical tumors carry germline TP53 mutations that predispose to cancer, radiation may increase the incidence of secondary tumors. One study reported three of five long-term survivors of pediatric adrenocortical tumors died of secondary sarcoma that arose within the radiation field.
(Refer to the PDQ summary on adult Adrenocortical Carcinoma Treatment for more information.)
Treatment options under clinical evaluation
The following is an example of a national and/or institutional clinical trial that is currently being conducted. Information about ongoing clinical trials is available from the NCI Web site.
- COG-ARAR0332 (Cisplatin-Based Chemotherapy and/or Surgery in Treating Young Patients With Adrenocortical Tumor): This Children's Oncology Group trial is evaluating the treatment of adrenocortical tumors with surgery and lymph node dissection. Patients with advanced disease will receive multiagent chemotherapy. Patients with stage I or stage II disease will have resection and retroperitoneal lymph node sampling (stage I) or dissection (stage II). Patients with stage III and stage IV disease will receive chemotherapy before resection. The chemotherapy regimen is cisplatin, doxorubicin, etoposide, and oral mitotane.