Introduction: In the pediatric age group, the incidence of the adrenocortical cancer (ACC) is 0.2%. The effective treatment is surgical resection. The only medical option is mitotane but it has negative effects on steroidogenesis. The difficulty in the management of mitotane therapy is discussed in this case.
Case report: An 1110/12 years-old boy was referred with A 5 cm diameter solid-hypoecoic mass observed by sonography in the left surrenal region. The physical examination was all normal at presentation. His pubertal stage was Tanner grade 3. His clinical findings were normal. In the adrenal hormone profile, DHEA, androstenedion, total testesterone (TT) elevated while the others (11-deoxycortisole, 17-OH progesterone, cortizole) were normal. His plasma renin activity and aldesterone were normal. Analysis of 24-h urine specimen revealed high cortisole levels. The case was diagnosed as grade 3 ACC and treated via surgical resection. Postoperatively chemotherapy, mitotane and hydrocortisone treatments were started. In the first month of the treatment hormonal profile was all normal but TT levels started to increase (>1500 ng/dl) at second month. In the follow-up, height growth stopped and bilateral gynecomasty developed. Magnetic resonance imaging and pozitron emission tomography scans and scrotal US were negative for recurrance or metastases at 6 months. The clinic was diagnosed as hypergonadotropic hypogonadism (HH) due to mitotane treatment. The follow-up of the case is continuing.
Discussion: Mitotane treatment leads to HH via reducing the gonadal steroidogenesis. Additionally, treatment increases the levels of sex-hormone binding globuline and decreases the activity of 5-alfa reductase that results with high levels of testesteron. In our case the testosterone levels were high but free androgen index was normal. The high levels of testesterone can be the result of metastase, recurrance or mitotane treatment adverse effect. This is a struggling problem in the management of mitotane therapy.
10 - 12 Sep 2016
European Society for Paediatric Endocrinology