Tang SG, Wei XL, Ye RJ, Xu HQ, Shang SH, Han YX, Wu LW, Xia WZ. Successful seven-year management of recurrent adult pancreatoblastoma with liver metastasis: A case report. World J Gastrointest Oncol 2026; 18(5): 116776 [DOI: 10.4251/wjgo.v18.i5.116776]
Corresponding Author of This Article
Wu-Zheng Xia, MD, PhD, Department of Organ Transplantation, Guangdong Provincial People’s Hospital, Guangdong Academy of Medical Sciences, Southern Medical University, No. 106 Zhongshan 2nd Road, Guangzhou 510080, Guangdong Province, China. xiawuzheng@gdph.org.cn
Research Domain of This Article
Gastroenterology & Hepatology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Shui-Guo Tang, Xiang-Ling Wei, Rong-Ji Ye, Hao-Qing Xu, Shu-Hao Shang, Yu-Xuan Han, Lin-Wei Wu, Wu-Zheng Xia, Department of Organ Transplantation, Guangdong Provincial People’s Hospital, Guangdong Academy of Medical Sciences, Southern Medical University, Guangzhou 510080, Guangdong Province, China
Wu-Zheng Xia, Department of General Surgery, Guangdong Provincial People’s Hospital Ganzhou Hospital, Ganzhou 341099, Jiangxi Province, China
Co-first authors: Shui-Guo Tang and Xiang-Ling Wei.
Co-corresponding authors: Lin-Wei Wu and Wu-Zheng Xia.
Author contributions: Tang SG and Wei XL collected the case information as co-first authors; Tang SG, Wei XL, Ye RJ, Xu HQ, Shang SH, and Han YX completed the manuscript draft; Wu LW and Xia WZ revised the manuscript as co-corresponding authors; all authors have read and approved the final manuscript.
Supported by Guangzhou Basic and Applied Basic Research Foundation, No. 2025A04J4730; National Natural Science Foundation of China, No. 82470635; and Jiangxi Provincial Natural Science Foundation, No. 20244BAB28028.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: All authors declare no conflict of interest in publishing the manuscript.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Corresponding author: Wu-Zheng Xia, MD, PhD, Department of Organ Transplantation, Guangdong Provincial People’s Hospital, Guangdong Academy of Medical Sciences, Southern Medical University, No. 106 Zhongshan 2nd Road, Guangzhou 510080, Guangdong Province, China. xiawuzheng@gdph.org.cn
Received: November 24, 2025 Revised: January 27, 2026 Accepted: March 30, 2026 Published online: May 15, 2026 Processing time: 172 Days and 10.8 Hours
Abstract
BACKGROUND
Pancreatoblastoma is a rare malignant epithelial tumor of the pancreas, predominantly affecting children. However, the prognosis is significantly poorer in adults, who typically present with liver metastasis. The management of adult pancreatoblastoma with liver metastases poses a significant challenge, and the median survival remains poor.
CASE SUMMARY
A 49-year-old male was incidentally found to have a pancreatic body/tail mass and liver metastasis during routine health screening. He underwent distal pancreatectomy, splenectomy, and hepatectomy, with pathology confirming pancreatoblastoma. Over a 7-year follow-up, the patient experienced multiple liver recurrences, managed with repeated resections, systemic chemotherapy, targeted therapy with surufatinib, transarterial chemoembolization, and internal radiation therapy following detection of fibroblast growth factor receptor 1-synaptonemal complex protein 1 gene rearrangement. Serial 18F-fluorodeoxyglucose positron emission tomography/computed tomography imaging played a crucial role in detecting recurrence and monitoring treatment response. The patient survived 87 months from initial diagnosis, significantly exceeding the reported median survival.
CONCLUSION
This case outlines the positron emission tomography/computed tomography presentation of and therapeutic strategies for pancreatoblastoma with liver metastases.
Core Tip: We report the 7-year follow-up of an adult pancreatoblastoma patient with liver metastases, emphasizing the importance of multimodal management, including repeated surgeries and targeted therapy. The case highlights the diagnostic utility of positron emission tomography/computed tomography and identifies a novel fibroblast growth factor receptor 1-synaptonemal complex protein 1 gene rearrangement, suggesting a potential target for future therapeutic strategies.
