Myxoid adrenal cortical carcinoma, a rare type of adrenal cortical cancer. A case report

Abstract

Adrenocortical carcinoma (ACC) is a rare malignancy of the adrenal cortex with aggressive clinical behavior and an unfavorable prognosis. Its estimated annual incidence is 0.5 to 2 cases per 1,000 000. The unfavorable prognostic factors include its rare variants, such as myxoid ACC. This ACC type was first reported as early as in 1979; however, its low prevalence hinders any reliable assessment of its prognostic value. Few published data indicate more than 2-fold lower median survival of patients with myxoid ACC, compared to that in classical ACC. Here we present a rare clinical case of myxoid ACC in a 56-year old woman with Cushing's syndrome, invasion of the adjacent tissues and organ by the time of diagnosis, rapid progression of the disease with fatal outcome. We discuss the variations of the myxoid component, associated with different cellular growth patterns in ACC. This clinical case demonstrates the aggressive course of myxoid ACC type, compared to the classical one, and is aimed at drawing the attention of various medical specialists to rare ACC variants.

Keywords

• adrenocortical cancer
• myxoid variant
• myxoid carcinoma of the adrenal cortex