MON-901 Primary Amenorrhea, Growth Arrest and Metabolic Syndrome Due to an Unclassified Hepatocellular Adenoma

Abstract

Primary Amenorrhea, Growth Arrest and Metabolic Syndrome Due to an Unclassfied Hepatocellalar Adenoma. Background: Hepatocellular adenoma is a rare benign neoplasm, seldom ascribed as the cause of endocrine and metabolic derrangement. We herein report a case of primary amenorrhea, growth arrest and metabolic syndrome due to huge hepatocellular adenoma. En bloc resection of the tumor normalized all the disturbances. Clinical Case: A 16-year-old girl, who is one of the quintuplets, complained of primary amenorrhea and growth arrest for the past 2 years. Her height (150cm) and weight (40kg) was at the 3rd percentile, whereas waist circumference (75cm) was at the 90th percentile for chronological age. She was hypertensive (145/115mmHg) on admission. Plasma cholesterols (TC 6.3mmol/L, LDL-c 3.76mmol/L), triglyceride (2.66mmol/L) and uric acid (532μmol/L) were elevated. Evaluation of GH/IGF-1 axis showed normal GH (0.90–2.53 μg/L) with extremly low IGF-1 concentration (35.29–39.74 ng/mL), and the latter was unresponsive to hGH stimulation. Computer tomography identified a huge liver mass (18.2cm×13.7cm×21cm). The patient underwent an uneventful open right hepatic lobectomy and cholecystectomy, and the tumor was en bloc resected. Immunohistochemistry indicated an unclassified hepatocellular adenoma, which was confirmed by whole exome sequencing. Her menarche started 6 months later followed by regular cycles without hormone replacement. IGF-1 concentration (471 ng/mL), blood pressure (102/62mmHg), lipid profile (TC 4.2mmol/L, LDL-c 2.51mmol/L, TG 1.44mmol/L) were all nomalized 10 months after surgery, and the girl had a reduction in waist circumference by 5cm, and a small gain in height by 2cm. Conclusion: We provide evidence that liver-derived IGF-1 has a direct effect on skeletal and pubertal development, blood pressure, viseral adiposity and dyslipidemia. Though rare, we propose the need to look into cases with heptocellular adenoma, for the existence of IGF-1 deficiency and its impact on endocrine and metabolic derangment.