Pediatric Adrenal Carcinoma Follow-up

• Patients with adrenal carcinomas who undergo complete surgical resection with no evidence of continuing functional hormone production do not require further inpatient care.
• If the patient has evidence of local or distant metastases during ambulatory follow-up, aggressive attempts at repeat resection should be undertaken. These attempts leads to additional inpatient care.
• If treatment includes intensive chemotherapy, further inpatient care is necessary to deliver chemotherapy or to treat chemotherapy-related toxicity.
• If lesions seem particularly sensitive to chemotherapy, with dramatic diminishment of tumoral masses in the chest or elsewhere, autologous transplantation might be a consideration. However, only anecdotal data suggest that transplantation is helpful in managing this disease. One study reported the use of chemotherapy, surgical debulking of lung metastases, and autologous transplantation; 2 years of continuous complete remission was reported.^[6]

• Ambulatory follow-up should occur every month for the first 2 years after treatment because repeat resection of locally recurring disease and resection of metastatic lung disease can substantially affect long-term survival.
• Scanning of the local area in the abdomen or pelvis and of sites of metastatic disease should continue every 3 months for the first 2 years, every 4 months for the next 2 years, and every 6 months during the fifth year.
• Patients should be monitored for the reappearance of adrenocortical hormone hyperactivity, along with scanning, unless their history suggests that Cushing syndrome or autonomous adrenocortical hormonal production is present. If this is the case, the physician should immediately search for recurrence.

The overall prognosis for patients with adrenal carcinoma is poor.

• In a study of pediatric patients with adrenocortical tumors, overall survival was 54.2%.^[1] The investigators did not distinguish adenoma from adenocarcinoma because of the difficulty (even with expert review) of separating these diagnoses at early stages. The excellent survival rate of patients with stage I tumors, the group who have adenomas, affects this rate. However, numerous valuable prognostic indicators have emerged; these serve to comfort patients with completely resected disease and suggest experimental or palliative care for those without.

  • Stage: By far the most important prognostic variable is the clinical stage. Because adenoma is usually stage I by definition, the observation that stage I survival rates approach 90% because of complete resection is no surprise. However, adenocarcinomas are noted in this group as well, and many investigators believe that a small (< 200 g) stage I carcinoma can be associated with a favorable prognosis when it is completely removed without tumor spill. With stage II disease, the survival rate decreases to close to 40%, even with complete resection. Stage III and IV disease result in equally poor survival rates of less than 20%.
  • Age: In the aforementioned study, patients younger than 4 years had an event-free survival rate of more than 70%. The event-free survival rate for patients aged 4-12 years decreased to 30-40%.
  • Function: Tumors that virilize alone or nonfunctioning tumors improve the prognosis compared with tumors that produce Cushing syndrome due to an overproduction of glucocorticoid.
• In a study of 31 patients at the Lahey Clinic over 30 years, mean survival was 17 months (range, 1-205 mo). The 5-year survival rate was 26%.^[8]
• As surgical procedures for tumor removal improve, patients' prognoses should also improve.
• At present, medical therapy has a palliative role. A well-conducted series of clinical trials is needed to prove that adjuvant therapy helps to prolong survival.