Androgen-secreting adrenocortical carcinoma
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Kamal P Galketiya, Sumathi Ranjikumar, Usama Majeed
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 upper- quadrant abdominal mass. A CT scan of the abdomen revealed a 10 cm adrenal mass (figure, 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) concentration was 22 umol/L (reference range 1.0-11.6 umol/L). The free androgen index (ratio of testosterone to sex-hormone-binding globulin) was 30.2% (reference range <0.8%). The serum concentrations of oestradiol, progesterone, luteinising hormone, follicle-stimulating hormone, and prolactin were within normal limits. The remaining endocrine studies (thyroid function tests, serum cortisol, aldosterone-to-renin ratio, plasma free metanephrines and normetanephrines, and urine biogenic amines) were also within normal limits. The increased concentrations of DHEAS and testosterone suggested an androgen- secreting adrenal neoplasm with virilising symptoms. 18F-fluorodeoxyglucose PET (FDG-PET) showed no evidence of metastatic disease.
An open right adrenalectomy was undertaken. Histology showed a neoplasm arising from the adrenal gland, fulfilling both macroscopic (460 g and 105 cm) and four of nine microscopic (Weiss) criteria for adrenocortical adenocarcinoma (high Fuhrman nuclear grade, more than five mitoses per 50 high-power fields, eosinophilic cytoplasm >75% of tumour cells, and necrosis; figure, C [haematoxylin and eosin staining]). Immunohisto- chemistry was positive for melan-A, vimentin, synaptophysin, and epithelial membrane antigen. Cytoplasmic staining was positive with calretinin (figure, D) and inhibin (figure, 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.
Contributors
KPG: writing the report; KPG, UM: managing the patient; SR: pathology. Written consent to publish was obtained.
Conflicts of interest
We declare that we have no conflicts of interest.
Lancet Diabetes Endocrinol 2013; 1: e10
Published Online January 30, 2013 http://dx.doi.org/10.1016/ S2213-8587(13)70005-6 Department of Surgery, Canberra Hospital, Garran, ACT, Australia (K P Galketiya FRACS, U Majeed FRACS); and Capital Pathology, Histology Department, Deakin, ACT, Australia (S Ranjikumar FRCPA)
Correspondence to: Dr Kamal P Galketiya, Department of Surgery, Canberra Hospital, Garran, ACT 2605, Australia kamaldanielle@gmail.com
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