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Pancreatic Nerve Sheath Tumors: a Single Institutional Series and Systematic Review of the Literature

  • Original Article
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Journal of Gastrointestinal Surgery

Abstract

Introduction

Improvement in imaging has resulted in frequent diagnosis of benign and premalignant pancreatic tumors. Pancreatic nerve sheath (PNS) tumors are one of the rarest pancreatic tumors. Literature on PNS is limited and their biology is poorly understood. Here, we report the largest series of PNS tumors to date and review the literature to evaluate the current data available on PNS tumors.

Methods

An institutional database was used to identify patients who underwent resection for PNS tumors. Clinicopathological characteristics and outcomes of these patients were reported. Furthermore, a review of literature was performed.

Results

From January 1994 through December 2016, seven patients underwent resection for PNS tumors. The median age was 57.7 years (IQR, 44.9–61.9) and the sex was approximately equally distributed (male = 4; 57.1%). Three (42.9%) patients were diagnosed incidentally and six (85.7%) were misdiagnosed as having other pancreatic tumors. The median tumor size was 2.1 (IQR 1.8–3.0) cm and six (85.7%) had no nodal disease. At a median follow-up of 15.5 (IQR 13.7–49.3) months, six patients were alive without evidence of disease and one patient was lost to follow-up. The literature review identified 49 studies reporting 54 patients with PNS tumors. Forty-six were misdiagnosed as having other pancreatic tumors. The median tumor size was 3.6 (range 1–20) cm, nodal disease was present in six patients (22.2%), and no patient had distant metastatic disease. At the time of last follow-up, all patients were free of disease.

Conclusion

This is the largest single institution series on PNS tumors reported to date. These tumors are rare and are often misdiagnosed, given their radiological characteristics. PNS tumors have a benign course of disease and surgical resection results in favorable long-term outcomes.

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Author notes

  1. Ammar A. Javed and Michael J. Wright have contributed equally to the completion of this work.

Authors and Affiliations

  1. Departments of Surgery, Johns Hopkins Hospital, Baltimore, MD, USA

    Ammar A. Javed, Michael J. Wright, Alina Hasanain, Kevin Chang, Richard A. Burkhart, John L. Cameron, Jin He, Christopher L. Wolfgang & Matthew J. Weiss

  2. Departments of Pathology, Johns Hopkins Hospital, Baltimore, MD, USA

    Ralph H. Hruban & Elizabeth Thompson

  3. Departments of Radiology, Johns Hopkins Hospital, Baltimore, MD, USA

    Elliot K. Fishman

  4. Baltimore, USA

    Matthew J. Weiss

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  1. Ammar A. Javed
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Javed, A.A., Wright, M.J., Hasanain, A. et al. Pancreatic Nerve Sheath Tumors: a Single Institutional Series and Systematic Review of the Literature. J Gastrointest Surg 24, 841–848 (2020). https://doi.org/10.1007/s11605-019-04201-4

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