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Journal of Gastrointestinal Surgery
Published in: Journal of Gastrointestinal Surgery 4/2020

01-04-2020 | Schwannoma | Original Article

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

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

Published in: Journal of Gastrointestinal Surgery | Issue 4/2020

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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.
Literature
1.
Duma, N., et al., Enlarging Pancreatic Schwannoma: A Case Report and Review of the Literature. Clin Pract, 2015. 5(4): p. 793.PubMedPubMedCentral
2.
Ferrozzi, F., D. Bova, and G. Garlaschi, Pancreatic schwannoma: report of three cases. Clin Radiol, 1995. 50(7): p. 492–5.PubMed
3.
Abu-Zaid, A., et al., Pancreatic tail schwannoma in a 44-year-old male: a case report and literature review. Case Rep Oncol Med, 2013. 2013: p. 416713.PubMedPubMedCentral
4.
Aggarwal, G., et al., Rare asymptomatic presentations of schwannomas in early adolescence: three cases with review of literature. Int J Surg, 2010. 8(3): p. 203–6.PubMed
5.
Akiyoshi, T., et al., Melanotic schwannoma of the pancreas: report of a case. Surg Today, 2004. 34(6): p. 550–3.PubMed
6.
Almo, K.M. and L.W. Traverso, Pancreatic schwannoma: an uncommon but important entity. J Gastrointest Surg, 2001. 5(4): p. 359–63.PubMed
7.
Brown, S.Z., et al., Schwannoma of the pancreas: a report of two cases and a review of the literature. Mod Pathol, 1998. 11(12): p. 1178–82.PubMed
8.
Bui, T.D., et al., Pancreatic schwannoma. A case report and review of the literature. JOP, 2004. 5(6): p. 520–6.PubMed
9.
Ciledag, N., K. Arda, and M. Aksoy, Pancreatic schwannoma: A case report and review of the literature. Oncol Lett, 2014. 8(6): p. 2741–2743.PubMedPubMedCentral
10.
Coombs, R.J., Case of the season. Malignant neurogenic tumor of duodenum and pancreas. Semin Roentgenol, 1990. 25(2): p. 127–9.PubMed
11.
David, S. and J.S. Barkin, Pancreatic schwannoma. Pancreas, 1993. 8(2): p. 274–6.PubMed
12.
Di Benedetto, F., et al., Pancreatic schwannoma of the body involving the splenic vein: case report and review of the literature. Eur J Surg Oncol, 2007. 33(7): p. 926–8.PubMed
13.
Dorsey, F., M.W. Taggart, and W.E. Fisher, Image of the month. Pancreatic schwannoma. Arch Surg, 2010. 145(9): p. 913–4.PubMed
14.
Eggermont, A., et al., Solitary malignant schwannoma of the pancreas: report of a case and ultrastructural examination. J Surg Oncol, 1987. 36(1): p. 21–5.PubMed
15.
Fasanella, K.E., K.K. Lee, and N. Kaushik, Clinical challenges and images in GI.Benign schwannoma of the pancreatic head. Gastroenterology, 2007. 132(2): p. 489, 830.PubMed
16.
Feldman, L., et al., Pancreatic schwannoma: report of two cases and review of the literature. Pancreas, 1997. 15(1): p. 99–105.PubMed
17.
Gupta, A., et al., Pancreatic schwannoma: literature review. J Surg Educ, 2009. 66(3): p. 168–73.PubMed
18.
Hirabayashi, K., et al., Cytological features of the cystic fluid of pancreatic schwannoma with cystic degeneration. A case report. JOP, 2008. 9(2): p. 203–8.PubMed
19.
Hsiao, W.C., P.W. Lin, and K.C. Chang, Benign retroperitoneal schwannoma mimicking a pancreatic cystic tumor: case report and literature review. Hepatogastroenterology, 1998. 45(24): p. 2418–20.PubMed
20.
21.
Kameyama, N., et al., Single-port transumbilical laparoscopic excision of retroperitoneal schwannoma mimicking a nonfunctional endocrine tumor in the body of the pancreas: a case report. Surg Innov, 2013. 20(6): p. NP30–4.PubMed
22.
Kim, G., et al., Pancreatic benign schwannoma: combined with hemorrhage in an internal cyst. J Dig Dis, 2011. 12(2): p. 138–41.PubMed
23.
Kinhal, V.A., et al., Pancreatic schwannoma: Report of a case and review of literature. Indian J Surg, 2010. 72(Suppl 1): p. 296–8.PubMedPubMedCentral
24.
Lee, J.S., et al., Ancient schwannoma of the pancreas mimicking a cystic tumor. Virchows Arch, 2001. 439(5): p. 697–9.PubMed
25.
Li, S., et al., Intrapancreatic schwannoma diagnosed by endoscopic ultrasound-guided fine-needle aspiration cytology. Diagn Cytopathol, 2009. 37(2): p. 132–5.PubMed
26.
Liegl, B., et al., Microcystic/reticular schwannoma of the pancreas: a potential diagnostic pitfall. Pathol Int, 2011. 61(2): p. 88–92.PubMed
27.
Liessi, G., et al., CT and MR imaging of melanocytic schwannomas; report of three cases. Eur J Radiol, 1990. 11(2): p. 138–42.PubMed
28.
Melato, M., et al., The schwannoma: an uncommon type of cystic lesion of the pancreas. Ital J Gastroenterol, 1993. 25(7): p. 385–7.PubMed
29.
Moller Pedersen, V., A. Hede, and N. Graem, A solitary malignant schwannoma mimicking a pancreatic pseudocyst. A case report. Acta Chir Scand, 1982. 148(8): p. 697–8.PubMed
30.
Moriya, T., et al., Pancreatic schwannoma: Case report and an updated 30-year review of the literature yielding 47 cases. World J Gastroenterol, 2012. 18(13): p. 1538–44.PubMedPubMedCentral
31.
Mourra, N., J. Calvo, and L. Arrive, Incidental Finding of Cystic Pancreatic Schwannoma Mimicking a Neuroendocrine Tumor. Appl Immunohistochem Mol Morphol, 2016. 24(2): p. 149–50.PubMed
32.
Mummadi, R.R., et al., Pancreatic Schwannoma presenting as a cystic lesion. Gastrointest Endosc, 2009. 69(2): p. 341; discussio 341.PubMed
33.
Novellas, S., et al., MRI features of a pancreatic schwannoma. Clin Imaging, 2005. 29(6): p. 434–6.PubMed
34.
Ohbatake, Y., et al., A case of pancreatic schwannoma - The features in imaging studies compared with its pathological findings: Report of a case. Clin J Gastroenterol, 2014. 7(3): p. 265–70.PubMed
35.
Okuma, T., et al., Pancreatic schwannoma: report of a case. Surg Today, 2008. 38(3): p. 266–70.PubMed
36.
Oshima, M., S. Yachida, and Y. Suzuki, Pancreatic schwannoma in a 32-year-old woman mimicking a solid-pseudopapillary neoplasm. Clin Gastroenterol Hepatol, 2010. 8(1): p. e1–2.PubMed
37.
Paranjape, C., et al., Clinical characteristics, treatment, and outcome of pancreatic Schwannomas. J Gastrointest Surg, 2004. 8(6): p. 706–12.PubMed
38.
Poosawang, W. and P. Kiatkungwankai, Pancreatic schwannoma: A case report and review of literature. J Med Assoc Thai, 2013. 96(1): p. 112–6.PubMed
39.
Soumaoro, L.T., et al., Benign schwannoma of the pancreas. J Gastrointest Surg, 2005. 9(2): p. 288–90.PubMed
40.
Stojanovic, M.P., et al., Malignant schwannoma of the pancreas involving transversal colon treated with en-bloc resection. World J Gastroenterol, 2010. 16(1): p. 119–22.PubMedPubMedCentral
41.
Suzuki, S., et al., Pancreatic schwannoma: a case report and literature review with special reference to imaging features. JOP, 2010. 11(1): p. 31–5.PubMed
42.
Tafe, L.J. and A.A. Suriawinata, Cystic pancreatic schwannoma in a 46-year-old man. Ann Diagn Pathol, 2008. 12(4): p. 296–300.PubMed
43.
Tan, G., et al., Cystic schwannoma of the pancreas. Ann Diagn Pathol, 2003. 7(5): p. 285–91.PubMed
44.
45.
Urban, B.A., et al., CT findings in cystic schwannoma of the pancreas. J Comput Assist Tomogr, 1992. 16(3): p. 492–3.PubMed
46.
von Dobschuetz, E., et al., Giant ancient schwannoma of pancreatic head treated by extended pancreatoduodenectomy. Pancreatology, 2004. 4(6): p. 505–8.
47.
Walsh, M.M. and K. Brandspigel, Gastrointestinal bleeding due to pancreatic schwannoma complicating von Recklinghausen’s disease. Gastroenterology, 1989. 97(6): p. 1550–1.PubMed
48.
Wu, W., et al., A Contemporary Evaluation of the Cause of Death and Long-Term Quality of Life After Total Pancreatectomy. World J Surg, 2016. 40(10): p. 2513–8.PubMed
49.
Yu, R.S. and J.Z. Sun, Pancreatic schwannoma: CT findings. Abdom Imaging, 2006. 31(1): p. 103–5.PubMed
50.
Edge, S.B. and C.C. Compton, The American Joint Committee on Cancer: the 7th edition of the AJCC cancer staging manual and the future of TNM. Ann Surg Oncol, 2010. 17(6): p. 1471–4.PubMed
51.
Weiss, S.W., J.M. Langloss, and F.M. Enzinger, Value of S-100 protein in the diagnosis of soft tissue tumors with particular reference to benign and malignant Schwann cell tumors. Lab Invest, 1983. 49(3): p. 299–308.PubMed
52.
Barron, M.R., et al., Does preoperative cross-sectional imaging accurately predict main duct involvement in intraductal papillary mucinous neoplasm? J Gastrointest Surg, 2014. 18(3): p. 447–55; discussion 5455-6.PubMed
Metadata
Title
Pancreatic Nerve Sheath Tumors: a Single Institutional Series and Systematic Review of the Literature
Authors
Ammar A. Javed
Michael J. Wright
Alina Hasanain
Kevin Chang
Richard A. Burkhart
Ralph H. Hruban
Elizabeth Thompson
Elliot K. Fishman
John L. Cameron
Jin He
Christopher L. Wolfgang
Matthew J. Weiss
Publication date
01-04-2020
Publisher
Springer US
Published in
Journal of Gastrointestinal Surgery / Issue 4/2020
Print ISSN: 1091-255X
Electronic ISSN: 1873-4626
DOI
https://doi.org/10.1007/s11605-019-04201-4

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