Human chorionic gonadotrophin responsive adrenal cortical carcinoma and Cushing's Syndrome in pregnancy
S Hoashi1, J O'Connell1, SN Kelly4, L Young4, J Fitzpatrick2, C O'Keane3 & BT Kinsley1
A 28 years old lady presented at 21 weeks gestation with bipedal oedema and 4 week history of easy bruising and weakness and aching of the thighs. She had no significant past medical history and had prior uneventful pregnancy 17 months previously. On examination she was hypertensive (180/100 mmHg), had puffy face, prominent abdominal striae and bilateral leg oedema. Serum potassium was 2.6 mmol/l and random plasma glucose was 8.6 mmol/l. OGTT confirmed gestational diabetes and she was commenced on insulin. Investigations for Cushing's Syndrome showed elevated 24 hour urine free cortisol and 8am plasma cortisol, and did not suppress after overnight 1mg dexamethasone (1628nmol/l). Ultrasound and MRI revealed a suprarenal mass measuring 15cm. BP control remained difficult despite therapy with labetalol and methyldopa and she remained hypokalaemic. Elective Caesarian section was planned for 28 weeks gestation but she went into spontaneous labour at 27 weeks and a healthy baby boy was delivered. Post partum, BP lowered to 140/80 mmHg and 8am plasma cortisol was 870nmol/l. 15 days post partum, she underwent right adrenalectomy and nephrectomy and removal of IVC thrombus. Histology confirmed adrenal carcinoma.
An in vitro study of tissue was performed. Adrenal tissue was grown in tissue culture medium. Cortisol was measured in control sample without stimulation, following stimulation with either Forskolin (ACTH) or Profassi 5000U (HCG). Cortisol levels were 317.6 (basal), 339.5 (+11.9 nmol/l, 3.7% rise) and 479.1 (+161.5 nmol/l, 50.9% rise) nmol/l respectively, suggesting HCG responsive tumour tissue.
Summary: This 28 years old lady presented with Cushing's Syndrome in the second trimester of pregnancy secondary to HCG responsive adrenal carcinoma, had safe delivery of baby at 27 weeks and had successful resection of tumour with resolution of cushingoid features.
Conclusion: The elevated HCG levels of pregnancy may have contributed to the clinical signs of Cushing's Syndrome and large size and growth of this adrenal carcinoma.