A GONADOTROPHIN-RESPONSIVE VIRILIZING ADRENAL TUMOUR IDENTIFIED AS A MIXED GANGLIONEUROMA AND ADRENOCORTICAL ADENOMA

Abstract

ABSTRACT A 38-year-old gravida 2, para 1 woman, who had suffered from virilization and amenorrhoea for 8 years was examined. She had peripheral serum testosterone (T) levels of 3.9–8.7 ng/ml (normal level: 0.32 ± 0.09 ng/ml) with normal serum levels of dehydroepiandrosterone (DHA), aldosterone and cortisol (F) and normal urinary 17-ketosteroids (17-KS). Dexamethasone (DXM 2 mg/day for 5 days) suppressed the serum F level adequately, but reduced the high T levels only slightly. The administration of commercial hCG 6000 IU for 3 days increased both the serum T levels (5.68 to 9.83 ng/ml) and the serum DHA levels (4.9 to 9.8 ng/ml, normal range 4–6 ng/ml), but synthetic ACTH-Z (Cortrosyn-Z®, 1 mg, Organon) did not affect the serum T level. The basal serum LH level was abnormally low, and constant infusion of synthetic LH-RH (200 μg/5 h) resulted in good responses of serum LH and FSH and increase in the serum T level from 5.2 to 9.1 ng/ml. Computerized tomography, ultrasonography and pneumo-retroperitoneumroentgenography showed the presence of a large tumour in the right adrenal. The tumour was a circumscribed irregularly lobulate mass, measuring 16×8×8 cm and weighing 500 g. Histologically, it was identified as a ganglioneuroma containing numerous scattered groups of large polyhydral cells similar to adrenocortical cells, forming islets in some places. After removal of the tumour from the right adrenal, the high serum T level decreased to the normal female level in 24 h.