Hepatic Failure: A Life-Threatening Complication of Severe Insulin Resistance
Presentation Number: SAT 622
Date of Presentation: April 1st, 2017
Shuchie Jaggi Jain*1, Cherie Lisa Vaz1 and Shanti Parkash2
1Temple Hospital, Philadelphia, PA, 2Temple Hospital
Introduction: Virilization is rarely seen in post-menopausal women. Most common causes of virilization are androgen secreting adrenal tumors, ovarian hyperthecosis and neoplastic production of excess androgens in post-menopausal women. The most commonly reported germ cell tumor is an ovarian teratoma however an active hormonally secreting one is exceedingly rare. We present a rare case of ovarian dermoid cyst secreting testosterone presumably causing severe insulin resistance and NASH.
Case:A 60 year old Hispanic female with history of DM Type 2, hyperlipidemia, obesity (BMI of 39) was referred to endocrinology for uncontrolled diabetes mellitus. Hemoglobin A1C was 8.6% at the time. On physical exam she was noted to be hirsute and started developing hirsuitism in her early 40s. Laboratory analysis showed an Insulin level of 30 (uIU/ml) and C-peptide 3.71ng/ml (0.8-3.10 ng/ml) Fasting glucose was 218 with HOMA-IR of 16.14 which were high (levels measured prior to initiation of insulin). Cushing’s work up was negative with DST. Total testosterone of 191ng/dL (2-45 ng/Dl), measured free testosterone of 7.9 pg/ml (0.2-5.0 pg/ml), sex hormone binding globulin of 115 nmol/L (14-73 nnmol/L), albumin of 3.7 with calculated free testosterone of 1.47 ng/dL. She also had an estradiol level of 30 pg/ml (postmenopausal < 31pg/ml) and DHEA-DO4 of <15 mcg/dL (normal <145). Liver enzymes were elevated at AST of 63 (10-35 U/L) and ALT of 36 (6-29 U/L) with INR of 1.2. CT of the pelvis showed a finding of circular calcification in the right adnexa with an involuted hemorrhagic cyst wall. A liver ultrasound showed heterogenous and nodular appearance suggesting cirrhosis. Patient was started on insulin with total daily dose of 185 units and gradually increased up to 360 units. A GLP-1 agonist (Liraglutide) was added and she was switched to U-500. Her HgA1c improved to 7.9% but continued to have worsening liver failure. A liver biopsy revealed several ballooned hepatocytes and mallory bodies suggesting coexisting steatohepatitis. She was diagnosed with cirrhosis of liver from NASH. She is now scheduled for surgery for removal of ovarian dermoid cyst.
Discussion:Some causes of severe insulin resistance (IR) are obesity, excess counterregulatory hormones, lipodystrophies and PCOS. Elevated testosterone levels in females have been reportedly associated with IR. In PCOS elevated testosterone is known to cause IR and IR results in worsening PCOS syndrome. Levels of Testosterone in PCOS are of mild-moderate severity causing IR. Thus we postulated with levels of testosterone in our patient that there would be resulting in severe IR. It is rare for dermoid cyst to produce active testosterone in this range, as seen in our case, especially in a post-menopausal woman. Surgical removal should be considered in hormonally active dermoid tumors with marked elevated testosterone before complications of severe IR occur.
Nothing to Disclose: SJJ, CLV, SP