ODP021 A Feminizing Adrenocortical Carcinoma Presenting with Marie-Antoinette Syndrome

Abstract Introduction Adrenocortical carcinomas (ACCs) are rare and aggressive malignant tumors often with an unfavorable prognosis accounting for 0. 05-0.2% of all cancers. ACCs can be separated into 2 groups: Functioning (hormone-producing) non-functioning tumors. About 60% synthesize hormones. Hormone-secreting most commonly cause virilization or Cushing's syndrome. On very occasions, lead to feminization in male subjects. Clinical manifestations gynecomastia and/or hypogonadism. Here, we present a case estradiol-secreting adrenal cortical carcinoma. Case presentation A 58-year-old presented patchy hair loss, change colour white, loss body eyebrows, breast tenderness. Hormone testing revealed significantly elevated estradiol came 582 pmol/L [NR<190] low testosterone 1.6 nmol/L [NR10-30], FSH 0.1 mIU/mL [NR 0.95-11.5] LH 0.6 [NR1.14-8.75]. testicular ultrasound was unremarkable. An MRI 11 cm heterogeneous right mass invading the inferior vena cava. FDG PET-CT intensely metabolically active tumor without any evidence metastatic disease. The successfully removed via open procedure. Histology confirmed adrenocortical carcinoma Weiss score 8 mitotic account 10/50 HPF Ki-67 labelling index 20-25%. There vascular invasion large areas necrosis, ENSAT (European Network Study Adrenal Tumours) stage II. Postoperatively, he made good clinical recovery offered adjuvant Mitotane EDP (Doxorubicin, Etoposide Cisplatin) chemotherapy. Due significant rise GGT platelets, had stopped temporarily. During course his treatment, developed hoarse voice CT neck abnormal thickening lateral aspects aortic arch suggesting possible recurrence aortitis. biopsy not diagnostic, but did show features malignancy. He completed five cycles Estradiol has remained normal improved. Conclusion We describe ACC presenting Marie-Antoinette syndrome as well paraneoplastic Targeted molecular analysis may guide further treatment options enrolment trials should considered. Presentation: No date time listed