Systematic Reviews
Copyright ©The Author(s) 2025. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastrointest Oncol. Feb 15, 2025; 17(2): 97298
Published online Feb 15, 2025. doi: 10.4251/wjgo.v17.i2.97298
Pancreatic primitive neuroectodermal tumors: Clinical features, treatment, and influencing factors
Yan-Fei He, Huan-Zhi Wang, Xiao-Dong Hu, Jun-Qiang Liu, Hu-Ming Li, Jie Wang, Shuang-Feng Lu
Yan-Fei He, Jie Wang, Shuang-Feng Lu, Health Management Center, The Sixth Medical Center, Chinese PLA General Hospital, Beijing 100048, China
Huan-Zhi Wang, College of Atmospheric Sciences, Lanzhou University, Lanzhou 730000, Gansu Province, China
Xiao-Dong Hu, Department of Endocrinology, The Sixth Medical Center, Chinese PLA General Hospital, Beijing 100048, China
Jun-Qiang Liu, Department of Thoracic Surgery, The Sixth Medical Center, Chinese PLA General Hospital, Beijing 100048, China
Hu-Ming Li, Department of Respiratory Medicine, The Sixth Medical Center, Chinese PLA General Hospital, Beijing 100048, China
Author contributions: He YF designed the study, reviewed the literature, and drafted the manuscript; Wang HZ contributed to the data analyses; Hu XD, Liu JQ, and Wang J contributed to the data collection; Wang HZ, Li HM, and Lu SF contributed to the study design; All authors read and approved the final version of the manuscript.
Conflict-of-interest statement: The authors have no conflicts of interest to declare.
PRISMA 2009 Checklist statement: The authors have read the PRISMA 2009 Checklist, and the manuscript was prepared and revised according to the PRISMA 2009 Checklist.
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: Yan-Fei He, MD, Associate Chief Physician, Doctor, Health Management Center, The Sixth Medical Center, Chinese PLA General Hospital, No. 6 Fu Cheng Road, Haidian District, Beijing 100048, China. heyanfeilc@163.com
Received: May 27, 2024
Revised: October 1, 2024
Accepted: November 1, 2024
Published online: February 15, 2025
Processing time: 235 Days and 16.4 Hours
Abstract
BACKGROUND

Data on clinical characteristics, treatment outcomes, and prognosis of pancreatic primitive neuroectodermal tumors (PNETs) are limited.

AIM

To analyze the clinical data of 30 patients with pancreatic PNETs to identify their clinical characteristics and factors associated with prognosis.

METHODS

We used the keywords "primary neuroectodermal tumor," "digestive tract," "pancreas," "pancreatic," and "gastrointestinal," individually or in combination, to collect data from a global database for all patients with pancreatic PNET to date. Univariate and Cox regression analyses were performed to identify prognostic factors for patient survival.

RESULTS

A total of 30 cases of pancreatic PNET were included in this study: 15 males and 15 females with a mean age of 24 years. The main symptom was abdominal pain (73.3%), and the median tumor size was 7.85 cm. Twenty-four patients (80.0%) underwent surgery and nineteen patients received adjuvant therapy. Local metastasis was observed in 13 patients (43.3%), lymph node metastasis in 10 patients (33.3%), and distant metastasis in 6 patients (20.0%). Local recurrence was observed in 13 patients (43.3%). The median survival time of all patients was 29.4 months, and the overall estimated 1-year and 3-year survival rates were approximately 66.0% and 36.4%, respectively. Univariate analysis showed that chemotherapy (P = 0.036), local metastasis (P = 0.041), lymph node metastasis (P = 0.003), distant metastasis (P = 0.049), and surgical margins (P = 0.048) were the prognostic factors affecting survival. Multivariate analysis revealed only lymph node metastasis (P = 0.012) as a prognostic factor.

CONCLUSION

Pancreatic PNET is extremely rare, occurs in young adults, has no apparent sex predisposition, has a high rate of metastasis and early recurrence, and has a very poor prognosis. The diagnosis of pancreatic PNET requires a combination of clinical symptoms, pathologic features, immunohistochemistry, and cytogenetic analysis. Univariate analysis suggested that chemotherapy, metastasis, and surgical margins were prognostic factors affecting survival, and multivariate analysis suggested that lymph node metastasis is an important prognostic factor. Therefore, early diagnosis, early and extensive resection, and adjuvant chemoradiotherapy may help improve prognosis.

Keywords: Primitive neuroectodermal tumor; Pancreas; Sarcoma; Diagnosis; Prognosis; Treatment

Core Tip: Pancreatic primitive neuroectodermal tumor (PNET) is a rare disease, which lacks sound treatment strategies and prognostic data. We identified 30 cases of pancreatic PNET from global databases and retrospectively analyzed their demographic data, clinical characteristics, and treatment factors. Univariate analysis showed that surgical margins, chemotherapy, local metastasis, lymph node metastasis, and distant metastasis were the prognostic factors affecting survival. Multivariate analysis revealed that only lymph node metastasis was a significant prognostic factor. The results of this study suggest that early diagnosis of pancreatic PNET is critical, and early wide resection and adjuvant radiotherapy may help to improve prognosis.