Endocrine Abstracts

Adrenocortical carcinoma (ACC) is a rare malignancy, accounting for 0.02% of all annual cancer. The majority of these tumors are benign, non-functioning adenomas that are incidentally discovered on abdominal image studies. Others are functional adenomas secreting cortisol, aldosterone, or less commonly androgens or estrogens. Pure androgen-secreting adrenal tumors are very rare.

Case report: A 34-year-old female patient presented with pain in the left upper abdomen. A multiphasique CT scan abdomen was done, which revealed large adrenal mass measuring 18×14×14 mm in dimension with central hemorrage. Patient gave history of amenorrhea since 6 months associated with increased hair growth of chest, lower abdomen, and face. Moreover the patient declare recent weight loss (11 kg in 2 months) with asthenia, anorexia, and melanoderma.

On examination, heart rate was 84/min, blood pressure 120/70 mm of Hg. Hirsutism was evaluated based on Ferriman-Gallwey score (result = 13). Biochemical screening showed a total testosterone=8 ng/ml (0.06–0.82), DHEA sulfate =3.27 mg/ml (0.4–2.17 mg/ml), 17-hydroxyprogesterone >20 ng/ml (0.1–0.8 ng/ml), LH=0.83 mUI/l and FSH=0.1 mUI/l. The diagnosis of Addison’s disease was confirmed by basal serum ACTH=125.82 ng/l and serum cortisol=135 μg/l after ACTH test; serum aldosterone and 24 h urinary VMA were normal. A diagnosis of androgen-secreting adrenocortical cancer with adrenal insufficiency was made.

Patient underwent exploratory laparotomy with excision of large adrenal tumor without any complication. The histopathological examination described a mass 21×18×12 mm with microscopique features of tumor cells sharing mild nuclear atypia and rare mitotic figures: histological and immunohistochmical aspect of adrenocortical carcinoma (Weiss score=3, Ki67=10).

On follow-up after 6 months, patient was comfortable, and no longer had amenorrhea or hursitism. Total testosterone was 0.03 ng/ml (0.06–0.82), DHEA-sulfate was 0.174 μg/ml (0.4–2.17 μg/dl) and CT scan abdomen was normale.