Endocrine Abstracts

Background: Adrenocortical carcinoma (ACC) is a rare malignant tumor with a poor prognosis. The only curative therapy is complete surgery (R0). A 5-year local recurrence and distant metastasis rate after R0 is 80–85%. Mitotane is widely used as an adjuvant treatment in patients with a high risk of recurrence. According to the most studies, it can delay and possibly prevent a recurrence of the disease in 35–50%. However, its efficacy is controversial, as well as the optimal duration of the treatment

Case Presentation: A 31-year-old white male patient with adrenocortical tumor (8 cm, 35–42 HU, non-contrast CT) underwent radical surgery in September, 2017. Microscopy and immunohistochemistry showed a poorly differentiated ACC with ki-67 from 10% to 45%. An adjuvant treatment with mitotane was initiated immediately (plasma level range, 13.4–16.7 μg/ml, 2 months after initiation and later). A follow-up included computed tomography (CT) of the abdomen, pelvis and chest, as well as hormonal and biochemical tests every 3 months. The patient had no clinical, laboratory and imaging findings of tumor recurrence or metastatic spread. Between March and June this year, the patient have not been taking mitotan for financial reasons. In June this year, CT revealed lung (S5) and multiple para-aortic lymph node metastasis (see Figure 1).

Conclusions: Adjuvant mitotan may be highly effective in patients with a poor prognosis. In addition, to determine an optimal duration of the treatment is essential. Some experts recommend to continue treatment for 3 to 5 years, while others discontinue after 2 to 3 years. It should last at least 2 years, anyway.

Figure 1 CT of the Abdomen, Pelvis and Chest. CT images show lung (Pannel B) and multiple para-aortic lymph node (Pannel A) metastasis.