A 38-year-old woman presented with symptoms suggesting Cushings syndrome. She has a history of surgery of mature ovarian teratoma (hysterectomy, ovarectomy) associated to radio and chemotherapy 15 years ago. The malignant teratoma relaps with hepatic and grelic metastases, and was unresectable. Main complaints were weight gain with centripetal fat distribution, muscle weakness, melanodermia and purpule striae on the skin of the abdomen, thighs, breasts and arms. She has a history of uncontrolled diabetes mellitus type 2 on insulin therapy and hypertension. Investigations: Cortisol failed to suppress after low dose and high dose on Dexamethasone suppression test. Plasma ACTH was 180 pg/ml (normal <60 pg/ml). A brain MRI confirmed the absence of any pituitary abnormality. Therefore, a diagnosis of ectopic Cushing syndrome was established. Computed tomography (CT) chest and abdomen showed multiple hepatic and intraabdominal and intraperitoneal masses, suspected radiologically to be a hamartoma. In-111 OctreoScan revealed suspected lesions located in the liver. The review and the re-reading of slides from ovarectomy showed a a focus of 3 mm with endocrine cell proliferation. Patient received ketoconazole treatment during 3 years with a moderate regression of the symptoms, then she received somatostatin analog therapy during 3 months. Conclusion: Ectopic cause of ACTH-secretion should be always included in differential diagnosis of Cushings syndrome especially with malignoma in patients history. Remarkable in this case is the delay of teratoma recurrence with paraneoplastic ACTH-secretion after 15 years of tumor-free interval.
18 - 21 May 2019
European Society of Endocrinology