Pediatric Adrenal Carcinoma Treatment & Management

Medical care in patients with adrenal carcinomas is supportive or adjuvant to surgical resection.

• Chemotherapy is as follows:

  • When metastatic disease cannot be removed or when biochemical evidence of tumor secretion persists after surgical removal, chemotherapy is often considered. At present, no data suggest that chemotherapy can fulfill a truly adjuvant role that improves patient survival and prevents relapse after incomplete or total resection.
  • Anecdotal data suggest that at least 2 drugs have single-agent activity that may prolong survival. If these drugs are thoroughly studied, they may ultimately demonstrate the possibility of having an adjuvant role. The 2 drugs are mitotane (trivial name, o,p’-DDD; trade name, Lysodren) and cisplatin (trivial name, CDDP; trade name, Platinol). Researchers have long known that mitotane dramatically decreases the production of adrenocortical hormone and ultimately ablates the adrenal cortex. As a single agent, cisplatin can shrink metastatic disease. In recent studies, etoposide and doxorubicin were added to cisplatin and mitotane.
  • Mitotane and cisplatin are most commonly used for palliation. Mitotane is used to decrease residual cortical hormonal production, and cisplatin is used to shrink metastases in the lung and elsewhere to prolong survival after the disease becomes unresectable.
  • Small studies have been conducted to examine etoposide and platinum or etoposide, doxorubicin, and platinum for adjuvant use.
  • Although no program of conventional chemotherapy appears curative in metastatic adrenal carcinoma, prolonged survival has been seen with the use of mitotane and other chemotherapy drugs and surgery.^[5]
• Radiation therapy may be helpful in the palliation of unresectable disease that is unresponsive to medical therapy.
• An anecdotal case of successful autologous transplantation, chemotherapy, and surgically reduced disease in a young child was reported, with 2-year follow up.^[6]

• Treatment of adrenocortical carcinoma begins with surgical care. Surgery is the mainstay of treatment and currently appears to be the major hope for cure. Every reasonable attempt should be made to render the patient disease-free at the primary site, at sites of local invasion, and at sites of metastatic disease.
• In the author’s experience, aggressive surgical care led to survival for longer than 10 years in 2 patients, one of whom was concurrently treated with medical therapy. This patient had a large, bulky tumor with no local invasion; the other patient had a stage II tumor and underwent several follow-up thoracotomy procedures.
• Case reports indicate that repeated thoracotomy can allow for more than 10 years of high-quality survival despite recurring crops of metastatic disease. In a study at Memorial Sloan-Kettering Cancer center, investigators evaluated patients with adrenocortical carcinoma.^[7] Aggressive primary surgical removal and aggressive surgical treatment of local or distant relapse led to long-term survival rates far superior to reported in previous studies, regardless of the patients' ages. One important feature of this study was that patients who underwent a complete second resection had a median survival of 74 months (5-y survival rate, 57%). The current role of surgical resection in the treatment of adrenocortical carcinoma cannot be overemphasized.
• Patients with symptoms of hormone overproduction and biochemical evidence of adrenocortical hormone overproduction should be supported in the postoperative period after major resection. These patients have suppressed endogenous adrenocortical hormone production. Stress doses of hydrocortisone that ultimately lead to maintenance doses and tapering (should the patient not be given further therapy) are indicated.

• Optimal care of pediatric patients with adrenocortical carcinoma includes consultation with a pediatric surgeon who has extensive experience in cancer surgery and consultation with a pediatric oncologist who may have managed cases of adrenocortical carcinoma.