Background: Hyperadrogenemia is one of the most commonly encountered endocrine disorders in reproductive-aged women. By far the most common causes of androgen excess are functional which account for more than 90% of cases. Androgen and ovarian tumours producing excess androgen are however very rare and only account for about 0.2% of cases.
Case history: A 62-year-old lady admitted to hospital with pneumonia was found to have an ill defined, firm, non-tender right upper quadrant mass, extensive hirsutism involving the face, lower abdomen and extremities, male pattern baldness, facial plethora and proximal myopathy. Initial investigation with ultrasound of the abdomen confirmed the presence of a 21 cm diffuse mass infiltrating the right kidney. A clinical diagnosis of androgen and cortisol secreting adrenocortical cancer was made and further investigation of full adrenal hormonal profile and CT scan imaging was arranged.
Her hormonal profile confirmed a high testosterone level of 52 nmol/l (normal <2.4 nmol/l) and androstendione of 35 nmol/l (normal 111.5 nmol/l) but normal DHEAS level. She also had raised 24 h urinary free cortisol level of 479 nmol/24 h (normal <280 nmol/24 h) and failed to suppress morning serum cortisol level following 1 mg. overnight dexamethasone suppression test (0900 h cortisol of 1369 nmol/l, normal <50 nmol/l). Her plasma metanephrine levels were within normal range. A CT scan of the abdomen confirmed the presence of a 24×15×14 cm heterogeneous suprarenal mass infiltrating the kidney along with evidence of liver and pulmonary metastasis and tumour thrombus in the inferior vena. An ultrasound guided biopsy of the mass confirmed the diagnosis of adrenocortical carcinoma.
Conclusion: Although hirsutism is commonly related to functional (non-tumour) causes, the presence of rapidly progressive and extensive hirsutism should alert physicians to undertake basic hormonal and imaging investigations to facilitate early diagnosis of malignant adrenal and ovarian androgen secreting tumours.