Letter to the Editor
Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. Jan 27, 2022; 14(1): 300-303
Published online Jan 27, 2022. doi: 10.4254/wjh.v14.i1.300
Current highlights on solid pseudopapillary neoplasm of the pancreas
Simone Sibio, Sara Di Carlo
Simone Sibio, Department of Surgery Pietro Valdoni, Sapienza University of Rome, Umberto I Hospital, Rome 00161, Italy
Sara Di Carlo, Minimally Invasive Surgery Unit, Tor Vergata Hospital, Tor Vergata University of Rome, Rome 00133, Italy
Author contributions: Both Sibio S and Di Carlo S contributed equally in revising current literature, writing the manuscript, and reviewing the drafts.
Conflict-of-interest statement: The authors declare no conflict of interest.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (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: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Simone Sibio, MD, PhD, Associate Professor, Surgical Oncologist, Department of Surgery Pietro Valdoni, Sapienza University of Rome, Umberto I Hospital, Viale del Policlinico 155, Rome 00161, Italy. simone.sibio@uniroma1.it
Received: September 4, 2021
Peer-review started: September 4, 2021
First decision: October 18, 2021
Revised: October 25, 2021
Accepted: January 5, 2022
Article in press: January 5, 2022
Published online: January 27, 2022
Processing time: 138 Days and 15.5 Hours
Abstract

Solid pseudopapillary neoplasm of the pancreas is a low-grade malignant tumor that predominantly affects young women in their third and fourth decade. Etiology and risk factors are unknown. Clinical symptoms are aspecific and most commonly due to mass effect. Diagnosis is made by computed tomography scan or magnetic resonance imaging and histological characterization is obtained by endoscopic ultrasound-guided fine needle biopsy. Microscopically, these lesions are composed by both solid and pseudopapillary structures with necrotic and hemorrhagic areas. Occasionally, the biological behavior is aggressive with tumor recurrence and distant metastasis. Usually, curative R0 surgical resection is the best option able to provide long term survival even in advanced disease. Unresectable disease is the main predictor of poor prognosis. Chemotherapy and radiotherapy regimens are not well standardized. However, they could be effective in reducing tumor size as neoadjuvant treatment or disease control in palliative setting. Although complete surgical resection provides a cure rate of > 95%, considering young age of the patients and morbidity associated to pancreatic surgery, further studies are needed to better investigate risk factors and responsiveness to hormones in order to allow early diagnosis and follow up strategies that could avoid unnecessary surgery in less aggressive disease.

Keywords: Pseudopapillary neoplasm; Pancreatic tumor; Pancreaticoduodenectomy; Distal pancreatectomy; Pancreas; Surgery

Core Tip: This letter aims to underline the utmost importance of early diagnosis and standardization of treatment for a subset of rare pancreatic malignant tumors that affect young women and have good prognosis when curative surgery is performed. However, little is known about clinical behavior and hormonal responsiveness of such diseases and treatment option availability is still scarce for advanced, recurrent and metastatic disease so further investigation is claimed.