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

Ammar A. Javed, Michael J. Wright, Alina Hasanain, Kevin Chang, Richard Burkhart, Ralph H Hruban, Elizabeth Thompson, Elliot K Fishman, John L Cameron, Jin He, Christopher Wolfgang, Matthew J Weiss

Research output: Contribution to journalArticle

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.

Original languageEnglish (US)
JournalJournal of Gastrointestinal Surgery
DOIs
StatePublished - Jan 1 2019

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Nerve Sheath Neoplasms
Diagnostic Errors
Neoplasms
Lost to Follow-Up
Databases

Keywords

  • Hepatobilliary surgery
  • Nerve sheath tumor
  • Pancreatic neoplasm
  • Pancreatic surgery
  • Pancreatic tumor
  • Schwannoma

ASJC Scopus subject areas

  • Surgery
  • Gastroenterology

Cite this

@article{6c82b03a10304e73ad5b499ecf59937e,
title = "Pancreatic Nerve Sheath Tumors: a Single Institutional Series and Systematic Review of the Literature",
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.",
keywords = "Hepatobilliary surgery, Nerve sheath tumor, Pancreatic neoplasm, Pancreatic surgery, Pancreatic tumor, Schwannoma",
author = "Javed, {Ammar A.} and Wright, {Michael J.} and Alina Hasanain and Kevin Chang and Richard Burkhart and Hruban, {Ralph H} and Elizabeth Thompson and Fishman, {Elliot K} and Cameron, {John L} and Jin He and Christopher Wolfgang and Weiss, {Matthew J}",
year = "2019",
month = "1",
day = "1",
doi = "10.1007/s11605-019-04201-4",
language = "English (US)",
journal = "Journal of Gastrointestinal Surgery",
issn = "1091-255X",
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TY - JOUR

T1 - Pancreatic Nerve Sheath Tumors

T2 - a Single Institutional Series and Systematic Review of the Literature

AU - Javed, Ammar A.

AU - Wright, Michael J.

AU - Hasanain, Alina

AU - Chang, Kevin

AU - Burkhart, Richard

AU - Hruban, Ralph H

AU - Thompson, Elizabeth

AU - Fishman, Elliot K

AU - Cameron, John L

AU - He, Jin

AU - Wolfgang, Christopher

AU - Weiss, Matthew J

PY - 2019/1/1

Y1 - 2019/1/1

N2 - 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.

AB - 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.

KW - Hepatobilliary surgery

KW - Nerve sheath tumor

KW - Pancreatic neoplasm

KW - Pancreatic surgery

KW - Pancreatic tumor

KW - Schwannoma

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