EDP chemotherapy combined with somatostatin analogues and mitotane in the treatment of disseminated adrenocortical carcinoma: a clinical case

Adrenocortical carcinoma is an orphan disease with an annual incidence of 1–2 cases per 1 million adults. The median overall survival (OS) is only 17 months. Definitive surgery increases the median OS to 28 months. The article presents a clinical case of a patient with hormonally active adrenocortical carcinoma of the right adrenal gland, accompanied by tumor thrombosis of the inferior vena cava extending to the right atrium. Surgery was performed for the thrombus removal. A wrong initial diagnosis led to a delay in adjuvant mitotane therapy. The recurrence of the disease was treated with mitotane and somatostatin analogues, considering the high expression of somatostatin receptors in the tumor. At the second progression, the patient was switched to chemotherapy in the EDP regimen combined with continued therapy with mitotane and octreotide, resulting in long-term control of the disease. The time without progression and OS were 18 and 42 months, respectively.