Cushings syndrome in pregnancy due to LH/hCG receptor positive adrenocortical carcinoma: case report
S Tigas1, K Zikopoulos2, Z Lei3, C Rao4 & A Tsatsoulis1
Introduction: Cushings syndrome is rare during pregnancy perhaps because hypersecretion of glucocorticoids supresses pituitary gonadotrophin secretion resulting in subfertility. When present, the syndrome is usually due to adrenal disease and less commonly to pituitary adenomas or ectopic ACTH production. In the contrary, Cushings syndrome outside pregnancy is usually due to pituitary adenomas whereas non ACTH-dependent Cushings is less frequent.
Case report: We describe the case of a 27-year-old female primigravida, who was admitted during the 22nd week of pregnancy with hypertension and probable preeclampsia. On admission, blood pressure was 130/90 mmHg on oral nifedipine and methylpdopa. Clinical examination revealed signs of florid Cushings syndrome. Laboratory investigations: plasma glucose 103 mg/dl, routine biochemical testing within normal range. Urine collection revealed proteinuria of 5.6 g/24 h. Serum cortisol: 49.7 μg/dl (5.023.0) with loss of diurnal variation and low ACTH (5.2 pg/ml). DHEAS was normal, total testosterone raised at 4.5 ng/ml (0.31.4) due to raised SHBG. Serum cortisol following overnight dexamethasone (1 mg) suppression testing was raised (>50 μg/dl). Abdominal ultrasound showed a 5.6 cm hypoechoic mass in the left adrenal. The patient underwent laparoscopic left adrenalectomy; a 5.9 cm mass was excised. Histological and immunohistochemical findings were consistent with adrenocortical carcinoma (Weiss score 5/9). In addition, immunohistochemistry showed expression of LH/hCG receptors in the tumour tissue. Postoperatively, the patients condition improved with normalization of the blood pressure and gradual improvement of the proteinuria (0.9 g/24 h). The patient underwent caesarian section (36th week of pregnancy), giving birth to a healthy male infant weighing 1800 g.
Conclusion: This is the second report of adrenocortical carcinoma presenting with Cushings syndrome during pregnancy. The presence of abberant LH/hCG receptors in adrenal tumour tissue might have contributed to the development of Cushings syndrome in pregnancy and possibly to tumour growth in this case.