Malignant intra ovarian tumor secreting glucocorticoïds is extremely rare (<5 cases reported in the world). Our aim is to report the first case observed in our practice. We also try to relate different mechanisms which are discussed in literature to explain intra ovarian position of adrenocortical tissue.
Case report: KM, 34 years, female, mother of two children, came for lower limbs edema which began one month after last delivery. Clinical exam found systemic hypertension, Cushings syndrome and manifestations of diabetes mellitus. On biological exploration there was a metabolic and androgenic hypercorticism (cortisol>500 ng/ml N=50250, testosterone=4,5 ng/ml N=0,11,2) non ACTH dependent (ACTH<10 pg/ml N=0,46). Adrenocortical glands were normal on echosonography and TDM but there was an ovarian tumor with a size of 14 cm.
Surgical exploration confirmed normality of adrenocortical glands, kidney and liver but there was a big ovarian tumor with peritoneal bleeding and ganglia. Histological exam noted a malignant adrenocortical tumor surrounding by small ovarian tissue with peritoneal and ganglia metastasis.
Conclusion: In this observation non ACTH dependent Cushings syndrome with normal adrenocortical glands argue for ectopic glucocorticoïds secretion. Radiological and surgical explorations showed an ovarian localisation which is confirmed by histological findings. Malignancy is proved by peritoneal and ganglia metastasis. This intra-ovarian localisation of adrenal carcinoma is very rare and has a bad prognosis.
01 - 05 Apr 2006
European Society of Endocrinology