P4702 - Uncharted Territory: Solid Pseudopapillary Neoplasm Arising from the Liver

Bryant Le, MD¹, Yousif Arif, BS², David L. Cheung, MD³, Nabil El Hage Chehade, MD⁴, Brian Mendoza, MD³, Joshua Kwon, MD¹, Peter H. Nguyen, MD³, Jason Samarasena, MD³, Frances Dang, MD^3
1University of California Irvine, Orange, CA; ²University of California Irvine School of Medicine, Orange, CA; ³University of California, Irvine, Orange, CA; ⁴Scripps Clinic, San Diego, CA

Introduction: Solid pseudopapillary neoplasms (SPN) tend to be rare, large, solid and cystic tumors of the pancreas that predominantly affect younger women. Although rare, common sites of metastases are the liver and local invasion to the duodenum, portal vein, and spleen. Here, we report a case of an SPN originating from the liver instead of the pancreas, which has not previously been reported in the literature.

Case Description/Methods: This is an 18-year-old female with a history of discoid lupus who initially presented to an outside hospital with acute abdominal pain. CT and MRI demonstrated a heterogenous 25 cm mass arising from the right hepatic lobe (Image A). CT guided biopsy showed central necrosis and histology consistent with a SPN. She was then admitted to our hospital 1 month later due to symptoms of weight loss, vomiting, and obstructive jaundice with elevated total bilirubin (5.0 mg/dL). Subsequent EUS/ERCP revealed a massive liver tumor (Image B), a long left intrahepatic stricture and a non-dilated common bile duct (Image C). A 10 Fr x 18 cm biliary stent was placed for biliary decompression. Despite improved bilirubin level after stenting, the patient continued to have intermittent abdominal pain. Thus, she underwent an exploratory laparotomy, open cholecystectomy, and intraoperative liver biopsy. Whipple procedure was aborted due to intraoperative findings which clearly identified that the tumor was confined to the liver and did not originate from the pancreas. Liver biopsies showed evidence of stage 2/4 periportal fibrosis with moderate macrovesicular steatosis. Biopsies of the liver mass showed preliminary results of neoplastic tissue, but final tumor classification is still pending.

Discussion: In our review of the literature, SPNs are most associated with and defined as being of pancreatic origin. To our knowledge, this is the first case of a SPN of liver origin. Despite multiple imaging modalities including CT, MRI, and EUS as well as open exploratory surgery, a lesion was not identified within the pancreas. Although SPNs occur most often in younger women, this patient’s age at presentation of 18 was significantly younger than the mean age of 28.5. Due to the favorable prognosis, surgical resection remains the mainstay of treatment. This case draws attention to the enigmatic nature of SPNs and warrants further investigation.



Disclosures:


Bryant Le, MD¹, Yousif Arif, BS², David L. Cheung, MD³, Nabil El Hage Chehade, MD⁴, Brian Mendoza, MD³, Joshua Kwon, MD¹, Peter H. Nguyen, MD³, Jason Samarasena, MD³, Frances Dang, MD³. P4702 - Uncharted Territory: Solid Pseudopapillary Neoplasm Arising from the Liver, ACG 2024 Annual Scientific Meeting Abstracts. Philadelphia, PA: American College of Gastroenterology.