Adrenocortical carcinoma (ACC) is an extremely rare tumor. Its early detection is difficult and its prognosis is poor.

We retrospectively analyzed the medical records of 13 patients with ACC between 1995 and 2005 at Samsung Medical Center. Their clinical features and prognosis were evaluated.

The average age of the patients with ACC was 40 years (2~75 years). Eight patients were men and five were women. On the first visit, eight of 13 patients (62%) complained abdominal pain, and abdominal mass was palpable in five of 13 patients (38%). Urinary concentration of 17-ketosteroid collected for 24 hours was increased in two of eight patients (25%). Three out of 13 patients were identified as having functioning adrenal tumors (1 Cushing's syndrome and 2 androgen-producing tumors), and the other 10 patients had hormonally inactive adrenal tumors. Ten patients had tumors in left adrenal gland, and three had in right adrenal gland. At the time of diagnosis, four patients were classified as having stage II, seven as stage III, and two as stage IV. Twelve patients underwent adrenalectomy. One of them received additional chemotherapy, and two patients were treated with external radiation therapy after surgery. The other one patient was treated only with chemotherapy due to the presence of liver metastasis. It was possible to continue the regular follow-up in eight of 13 patients. The median duration of follow-up was 39 months (7~114 months). Six of them are still alive (three have no evidence of disease, one had persistence of disease, and two had recurrence of disease during follow-up period). Two patients died of multiple metastases and lung metastasis, respectively.

Radical surgery is the only curative approach and is recommended for all patients with resectable tumors, even though in those patients with recurrent disease. There is no consensus concerning adjuvant therapy.