Gastrointestinal schwannomas: a rare but important differential diagnosis of mesenchymal tumors of gastrointestinal tract

Abstract Background Schwannomas of gastrointestinal tract are rare, mostly benign and notably different neoplasms from conventional schwannomas that arise in soft tissue or the central nervous system. These tumors are of clinical importance since they should always be considered in the differential...

Full description

Bibliographic Details
Main Authors: Alexandros Mekras, Veit Krenn, Aristotelis Perrakis, Roland S Croner, Vasileios Kalles, Cem Atamer, Robert Grützmann, Nikolaos Vassos
Format: Article
Language:English
Published: BMC 2018-07-01
Series:BMC Surgery
Subjects:
Online Access:http://link.springer.com/article/10.1186/s12893-018-0379-2
_version_ 1819211140695064576
author Alexandros Mekras
Veit Krenn
Aristotelis Perrakis
Roland S Croner
Vasileios Kalles
Cem Atamer
Robert Grützmann
Nikolaos Vassos
author_facet Alexandros Mekras
Veit Krenn
Aristotelis Perrakis
Roland S Croner
Vasileios Kalles
Cem Atamer
Robert Grützmann
Nikolaos Vassos
author_sort Alexandros Mekras
collection DOAJ
description Abstract Background Schwannomas of gastrointestinal tract are rare, mostly benign and notably different neoplasms from conventional schwannomas that arise in soft tissue or the central nervous system. These tumors are of clinical importance since they should always be considered in the differential diagnosis of submucosal lesions of gastrointestinal tract. Methods Seven patients with a pathologically proven gastrointestinal schwannoma were identified in our series of mesenchymal tumors and reviewed retrospectively. Clinicopathological and immunohistochemical parameters along with the follow-up results were analysed. Results The series included two males and five females, with a mean age 69 years (range, 39–81). Most patients were asymptomatic on presentation, except for two patients with abdominal pain. In the other cases (n = 5), the tumor was an incidental finding during other medical, imaging or surgical procedures. The tumors were located in the stomach (n = 4) and in the small intestine (n = 3) with an average size of 29 mm (range, 12–70). A preoperative diagnosis was achieved only in one case with a CT-guided core biopsy. Otherwise the clinical, intraoperative, endoscopic or radiological findings were unspecific. Patients with gastric tumor underwent either laparoscopic (n = 2) or open (n = 2) gastric wedge resection of the tumor; in the cases of intestinal tumor (n = 3) a segmentectomy was performed. Pathological examination revealed solid homogenous tumors, which were highly cellular and composed of spindle cells with positive staining for S100 protein, and confirmed the diagnosis of schwannoma. All tumors were negative for c-Kit, smooth muscle actin, desmin and DOG-1 and showed very low proliferation index. There were negative resection margins and no malignant variants were recognized. At an average follow-up of 60 months (range, 24–185) all patients were free of disease with no signs of recurrence or metastases and acceptable gastrointestinal function. Conclusions Schwannomas are rare, slow-growing and mostly asymptomatic gastrointestinal mesenchymal tumors. They are difficult to be diagnosed preoperatively as endoscopic and radiological findings are nonspecific but histological and immunohistochemical features are of paramount importance to differentiate between benign and malignant schwannomas, or other spindle cell sarcomas. The treatment of choice is complete surgical excision without a conclusive preoperative diagnosis, and the long-term outcome is excellent as these lesions are mostly benign.
first_indexed 2024-12-23T06:22:20Z
format Article
id doaj.art-6879886fe34e4cbbb99621757ba8c5da
institution Directory Open Access Journal
issn 1471-2482
language English
last_indexed 2024-12-23T06:22:20Z
publishDate 2018-07-01
publisher BMC
record_format Article
series BMC Surgery
spelling doaj.art-6879886fe34e4cbbb99621757ba8c5da2022-12-21T17:57:09ZengBMCBMC Surgery1471-24822018-07-011811710.1186/s12893-018-0379-2Gastrointestinal schwannomas: a rare but important differential diagnosis of mesenchymal tumors of gastrointestinal tractAlexandros Mekras0Veit Krenn1Aristotelis Perrakis2Roland S Croner3Vasileios Kalles4Cem Atamer5Robert Grützmann6Nikolaos Vassos7Department of Surgery, S. Elisabeth HospitalMVZ-Zentrum für Histologie, Zytologie und Molekulare DiagnostikDepartment of Surgery, University Hospital ErlangenDepartment of Surgery, University Hospital ErlangenDepartment of Surgery, S. Elisabeth HospitalDepartment of Surgery, S. Elisabeth HospitalDepartment of Surgery, University Hospital ErlangenDepartment of Surgery, University Hospital ErlangenAbstract Background Schwannomas of gastrointestinal tract are rare, mostly benign and notably different neoplasms from conventional schwannomas that arise in soft tissue or the central nervous system. These tumors are of clinical importance since they should always be considered in the differential diagnosis of submucosal lesions of gastrointestinal tract. Methods Seven patients with a pathologically proven gastrointestinal schwannoma were identified in our series of mesenchymal tumors and reviewed retrospectively. Clinicopathological and immunohistochemical parameters along with the follow-up results were analysed. Results The series included two males and five females, with a mean age 69 years (range, 39–81). Most patients were asymptomatic on presentation, except for two patients with abdominal pain. In the other cases (n = 5), the tumor was an incidental finding during other medical, imaging or surgical procedures. The tumors were located in the stomach (n = 4) and in the small intestine (n = 3) with an average size of 29 mm (range, 12–70). A preoperative diagnosis was achieved only in one case with a CT-guided core biopsy. Otherwise the clinical, intraoperative, endoscopic or radiological findings were unspecific. Patients with gastric tumor underwent either laparoscopic (n = 2) or open (n = 2) gastric wedge resection of the tumor; in the cases of intestinal tumor (n = 3) a segmentectomy was performed. Pathological examination revealed solid homogenous tumors, which were highly cellular and composed of spindle cells with positive staining for S100 protein, and confirmed the diagnosis of schwannoma. All tumors were negative for c-Kit, smooth muscle actin, desmin and DOG-1 and showed very low proliferation index. There were negative resection margins and no malignant variants were recognized. At an average follow-up of 60 months (range, 24–185) all patients were free of disease with no signs of recurrence or metastases and acceptable gastrointestinal function. Conclusions Schwannomas are rare, slow-growing and mostly asymptomatic gastrointestinal mesenchymal tumors. They are difficult to be diagnosed preoperatively as endoscopic and radiological findings are nonspecific but histological and immunohistochemical features are of paramount importance to differentiate between benign and malignant schwannomas, or other spindle cell sarcomas. The treatment of choice is complete surgical excision without a conclusive preoperative diagnosis, and the long-term outcome is excellent as these lesions are mostly benign.http://link.springer.com/article/10.1186/s12893-018-0379-2SchwannomaGastrointestinalMesenchymal tumor
spellingShingle Alexandros Mekras
Veit Krenn
Aristotelis Perrakis
Roland S Croner
Vasileios Kalles
Cem Atamer
Robert Grützmann
Nikolaos Vassos
Gastrointestinal schwannomas: a rare but important differential diagnosis of mesenchymal tumors of gastrointestinal tract
BMC Surgery
Schwannoma
Gastrointestinal
Mesenchymal tumor
title Gastrointestinal schwannomas: a rare but important differential diagnosis of mesenchymal tumors of gastrointestinal tract
title_full Gastrointestinal schwannomas: a rare but important differential diagnosis of mesenchymal tumors of gastrointestinal tract
title_fullStr Gastrointestinal schwannomas: a rare but important differential diagnosis of mesenchymal tumors of gastrointestinal tract
title_full_unstemmed Gastrointestinal schwannomas: a rare but important differential diagnosis of mesenchymal tumors of gastrointestinal tract
title_short Gastrointestinal schwannomas: a rare but important differential diagnosis of mesenchymal tumors of gastrointestinal tract
title_sort gastrointestinal schwannomas a rare but important differential diagnosis of mesenchymal tumors of gastrointestinal tract
topic Schwannoma
Gastrointestinal
Mesenchymal tumor
url http://link.springer.com/article/10.1186/s12893-018-0379-2
work_keys_str_mv AT alexandrosmekras gastrointestinalschwannomasararebutimportantdifferentialdiagnosisofmesenchymaltumorsofgastrointestinaltract
AT veitkrenn gastrointestinalschwannomasararebutimportantdifferentialdiagnosisofmesenchymaltumorsofgastrointestinaltract
AT aristotelisperrakis gastrointestinalschwannomasararebutimportantdifferentialdiagnosisofmesenchymaltumorsofgastrointestinaltract
AT rolandscroner gastrointestinalschwannomasararebutimportantdifferentialdiagnosisofmesenchymaltumorsofgastrointestinaltract
AT vasileioskalles gastrointestinalschwannomasararebutimportantdifferentialdiagnosisofmesenchymaltumorsofgastrointestinaltract
AT cematamer gastrointestinalschwannomasararebutimportantdifferentialdiagnosisofmesenchymaltumorsofgastrointestinaltract
AT robertgrutzmann gastrointestinalschwannomasararebutimportantdifferentialdiagnosisofmesenchymaltumorsofgastrointestinaltract
AT nikolaosvassos gastrointestinalschwannomasararebutimportantdifferentialdiagnosisofmesenchymaltumorsofgastrointestinaltract