Endocrine Abstracts

Introduction: Adrenocortical carcinoma (ACC) is a rare cancer (estimated incidence, 0.7 to 2.0 cases per 1 million population per year) with a poor prognosis. Even after seemingly complete surgical resection, most patients develop recurrence within 5 years. The 5-year survival rate is <15% among patients with metastatic disease.

Methods: We present the results of six patients with adrenocortical carcinoma followed in our clinic.

Results: The mean age was 54 years and female/male ratio was 2/4. Tumor size ranges from 6 to 14 cm. At baseline, only one patient had high dehydroxyepiandrosterone-sulphate levels (Patient 1). Tumor was non-functional in 5 patients. One patient presented with Cushing’s syndrome and hypercortisolism was documented in her laboratory evaluations (Patient 4). All of them were treated with surgery. In one patient, tumor was bilateral and in others were on left adrenal. The patient with bilateral tumors had lung metastasis at diagnosis (Patient 3). Another patient developed lung metastasis 2 years after the operation (Patient 1). Patient 4 had lung metastasis and Patient 6 had lung, liver and lymph node metastasis. One patient was lost-to follow-up after the operation (Patient 5). Three patients died 5 months after the operation. Three out of six patients received mitotane after the operation. Two patients could not receive mitotane because of elevated liver enzymes (Patients 4 and 6). Patient 1 developed lung metastasis 2 years after the diagnosis and is now receiving chemotherapy (cisplatin-etoposide) with mitotane. This patient is being followed in our clinic for 53 months.

Conclusion: Treatment options for advanced ACC are limited. Even after successful complete excision of the tumor, local or distant metastatic recurrences are frequent. However, some patients on regular follow-up and mitotane as adjuvant therapy may benefit from treatment as seen in Patient 1.