1387 Late Recurrence of Metastatic Pancreatic Angiomyolipoma in Tuberous Sclerosis Following Bilateral Nephrectomy and Transplant

Abstract

INTRODUCTION: Angiomyolipomas are typically benign tumors often associated with tuberous sclerosis, most commonly occurring in the kidneys. Some variants have malignant potential. Surgical resection remains the most effective form of treatment; however, recurrence after nephrectomy is rare, especially in the pancreas. CASE DESCRIPTION/METHODS: We report a unique case of a 59-year-old female with tuberous sclerosis who underwent bilateral nephrectomy for malignant, hemorrhagic angiomyolipomas with subsequent renal transplant. Almost 10 years after initial discovery of renal angiomyolipoma, patient presented to gastroenterology clinic with elevated liver function tests and was subsequently referred for endoscopic ultrasound (EUS) to evaluate for biliary obstruction as the etiology. If no obstruction was found, an EUS guided liver biopsy was requested. The EUS showed a normal non-dilated common bile duct (CBD) with no evidence of obstruction. EUS guided liver biopsy was then performed using a 19G core biopsy needle. Incidentally, she was found to have pancreatic tail mass lesion which measured 13 mm in diameter. This was not visualized on her previous surveillance CT scans. EUS fine needle aspiration was performed on the pancreatic tail mass with a 25G needle. The liver biopsy showed mild iron overload and minimal macrovesicular steatosis. The cytology from the pancreatic lesion was reported as an angiomyolipoma. The patient was subsequently referred to surgical oncology for evaluation of distal pancreatectomy. DISCUSSION: This is the second reported case of a pancreatic angiomyolipoma in a tuberous sclerosis patient, however guidelines on surveillance are lacking. Radiological imaging has not been an effective form of detecting pancreatic angiomyolipomas. This case highlights the importance of continued surveillance, and the possible role of EUS in that surveillance, for recurrent angiomyolipoma following nephrectomy in patients with tuberous sclerosis.