Five cases of adrenocortical carcinoma (ACC) we experienced at our hospital

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: MON 1-36-Adrenal Incidentaloma & Carcinoma
Monday, June 17, 2013: 1:45 PM-3:45 PM
Expo Halls ABC (Moscone Center)

Poster Board MON-36
Mariko Sue*1, Aya Yoshihara2, Kenzaburo Oda3, Yasuyo Ando3, Tetsuo Nemoto4, Megumi Wakayama4, Kazutoshi Shibuya4, Naoki Hiroi5 and Takahisa Hirose3
1Toho Univ Sch of Medicine, Tokyo, Japan, 2Toho University School of Medici, Tokyo, Japan, 3Toho University, Tokyo, Japan, 4Toho University School of Medicine, Tokyo, Japan, 5Toho Univ Dept of Med, Tokyo, Japan

Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with the incidence of 0.5 to 2 per million people, and it’s prognosis is poor whose 5-year overall survival is 32-45%. The mean average in adults is 45 years and most cases are sporadic. It is also known to affect female more commonly than male with a ratio of 1.5:1. ACC is staged on the basis of TNM, and approximately 61 %, the majority of patients present with stage 4.


We experienced five cases of ACC in our hospital past five years. All five cases were female and sporadic, with a mean age of 36 ± 10 years old. Among five cases, two cases presented the symptoms related to Cushing’s syndrome, one case presented systemic inflammatory symptoms associated with elevated cytokine probably secreted from tumor, and two cases had cardiopulmonary symptoms associated with enlarged adrenal tumor or metastatic tumor. All five cases revealed Cushing’s syndrome and adrenal tumor was detected at right adrenal in two cases, left adrenal in two cases, except one case of ectopic ACC with normal bilateral adrenal glands.

Adrenalectomy was performed for three cases of stage 2, followed by mitotane administration. One case survived more than 5 years after operation without recurrence or metastasis, one case had recurrence 6 months after surgery which required re-operation, and the other case have no sign of recurrence 3 months after surgery.
As there was no indication of surgery for two cases of stage 4 ACC, we administrated chemotherapy of etoposide, doxorubicin and cisplatin (EDP therapy) combined with mitotane. The tumor extended into suprahepatic inferior vena cava and right atrium with tumor embolism and thrombosis in one case, and there was no response to EDP treatment. The other case of ectopic ACC rapidly developed multiple metastases even after six cycles of EDP therapy. Both patients died of multiple organ insufficiency. Interestingly, the case with ectopic ACC had identical twin sister, who had no medical problems at all.


Total resection is recommended in primary ACC for longer overall survival. The treatment for the cases without surgical indication is still controversial. Mitotane is reported to be the only effective curative for ACC. Two regimens are emerging as the options: mitotane in combination with streptozocin or in combination with EDP. However, the efficacy of chemotherapy is reported to be less than 50%, and the survival rate from ACC has not changed over the last 20 years. Several trails are now underway such as sunitinib, which targets several tyrosine kinase receptors, or temsirolimus, an mTOR inhibitor.


We experienced 5 cases of ACC in 6 years. The prognosis of two cases with stage 4 was poor whose survival rate was 0.75 year. Even for three cases with stage 2, one case recurred within one year after initial surgery. Further effective treatment or regimen is needed.

Nothing to Disclose: MS, AY, KO, YA, TN, MW, KS, NH, TH

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