SAT361 A Rare Case of Ovarian Steroid Cell Tumor in a Patient With Known Polycystic Ovarian Syndrome

Disclosure: O.A. Aluko: None. S. Patel: None. A. Graul: None. M. Deshmukh: None. Introduction.Sex cord stromal cell tumors are rare tumors of the ovary that usually occur inthe first twotothreedecades of life. Specifically, steroid cell tumors of the ovary account for 0.1% of all ovariantumors.Testosterone productionis typical, with hirsutism and virilizationbeing the most commonsymptoms. Amarkedly elevated testosterone level >200ng/dl is animportant diagnostic threshold for discriminatingandrogen- secreting tumors from non-neoplastic lesions.We report acase of ovariansteroid cell tumor inapatient with knownPCOS.Case ReportA 28-year old female with past medical history of PCOS and infertility was referred tothe Endocrinologyoffice for evaluationof worsening hirsutism and elevated testosterone level.She reported increased facial hair especially onher upper lipand chin, abdomenand inner thighs. Shehad ahistory of irregular periods and had beenonoral contraceptives as aresult. She was started onspironolactone 50mg daily by her PCP with noimprovement insymptoms.Bloodwork done prior tovisit showed elevated total testosterone of 312ng/dL (n13-71ng/dL) withelevated free testosterone of 5.8pg/dL (n0.0- 4.2pg/dL). Pelvic ultrasound showed peripherally orientedfollicles inkeeping with knowndiagnosis of PCOS with nosuspicious adnexal mass or loculatedcollections. Repeat bloodwork done three weeks after discontinuing spironolactone showed worsening elevatedserum testosterone of 561ng/dL with elevated free testosterone of 7.7pg/dL. Further workuptorule outother underlying causes returned negative. DHEA-S, Androstenedione, 24hr urinary cortisol, salivarycortisol X3, ACTH, InhibinB, LDH, Ca-125, betaHCG, were all withinnormal limits.CT abdomenand pelvis showed anenhancing nodule inthe right ovary with normal adrenal glands.Repeat pelvic ultrasound showed anew 2.9cm right ovariansolid isoechoic hyperemic nodule O-RADScategory 5(50% risk of malignancy) similar toMRI findings.She underwent aright laparoscopic ovariancystectomy with pathology diagnosis of sex cord steroidal celltumor with noevidence of increased mitotic activity or necrosis. Immuno-histochemistry supported thediagnosis with positive steroidogenic factor-1(SF-1) gene. Repeat total testosterone and freetestosterone levels six weeks after surgery were 44ng/dl and 2.4ng/dl respectively, both withinnormallimits.ConclusionInthe setting of rapid onset hirsutism with significantly elevated testosterone levels, there should be ahigh clinical suspicionfor anandrogensecreting tumor which must be excluded, irrespective of knowndiagnosis of PCOS. Repeat imaging after non-neoplastic processes are ruled out should be consideredas surgical resectionis the primary treatment for ovariansteroid cell tumors and should not be missed. Presentation Date: Saturday, June 17, 2023