Androgen-secreting adrenocortical carcinoma

Abstract

A 47-year-old woman presented with abdominal pain. She had recently noticed increased growth of facial hair and loss of scalp hair, and her menstrual cycle had become erratic. Abdominal examination revealed a right upperquadrant abdominal mass. A CT scan of the abdomen revealed a 10 cm adrenal mass (fi gure, A, B; green arrows) that was well encapsulated and heterogeneous. Endocrine studies were ordered to exclude a functioning adrenal neoplasm. The serum total testosterone concentration was 8·6 nmol/L (reference range 0·13–2·53 nmol/L) and the serum dehydroepiandrostenedione sulphate (DHEAS) con cen tration was 22 μmol/L (reference range 1·0–11·6 μmol/L). The free androgen index (ratio of testosterone to sex-hormone-binding globulin) was 30·2% (reference range 75% of tumour cells, and necrosis; fi gure, C [haematoxylin and eosin staining]). Immuno histochemistry was positive for melan-A, vimentin, synaptophysin, and epithelial membrane antigen. Cytoplasmic staining was positive with calretinin (fi gure, D) and inhibin (fi gure, E). The Ki-67 proliferative index was 32%. Mitotane was recommended as adjuvant therapy for 2 years. No evidence of recurrence or metastatic disease was recorded 12 months postoperatively and androgen concentrations remain suppressed. Women presenting with hirsutism or virilisation are more likely to have polycystic ovarian syndrome than an androgen-secreting adrenal neoplasm. Assessment of patients requires thorough hormonal investigation and imaging, including CT, MRI, and FDG-PET.