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      Gastric schwannoma: a case report and literature review

      case-report

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          Abstract

          Background

          Gastric schwannoma is a rarely seen gastric tumor accounting for only 0.2% of all gastric tumors. It is difficult to distinguish a gastric schwannoma from other gastric tumors preoperatively.

          Case presentation: A 30-year-old man with no significant medical history or physical examination findings presented with a 1-month history of right upper abdominal discomfort. The preoperative diagnosis was a gastrointestinal stromal tumor, but the postoperative pathologic and immunohistochemical examinations confirmed a gastric schwannoma. The patient underwent laparoscopic wedge resection of the stomach without additional postoperative treatment, and his postoperative recovery was uneventful. No recurrence or metastasis was found at the 2-year follow-up examination.

          Conclusion

          Although gastric schwannomas are usually not malignant, they are difficult to distinguish from other malignant stromal tumors preoperatively. Surgical resection should be recommended when a schwannoma is malignant or considered to be at risk of becoming malignant.

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          Most cited references31

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          Gastrointestinal stromal tumors--definition, clinical, histological, immunohistochemical, and molecular genetic features and differential diagnosis.

          M Miettinen, J. Lasota (2001)
          Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal (GI) tract. They are defined here as KIT (CD117, stem cell factor receptor)-positive mesenchymal spindle cell or epithelioid neoplasms primary in the GI tract, omentum, and mesentery. GISTs typically present in older individuals and are most common in the stomach (60-70%), followed by small intestine (20-25%), colon and rectum (5%), and esophagus (<5%). Benign tumors outnumber the malignant ones by a wide margin. Approximately 70% of GISTs are positive for CD34, 20-30% are positive for smooth muscle actin (SMA), 10% are positive for S100 protein and <5% are positive for desmin. The expression of CD34 and SMA is often reciprocal. GISTs commonly have activating mutations in exon 11 (or rarely exon 9 and exon 13) of the KIT gene that encodes a tyrosine kinase receptor for the growth factor named stem cell factor or mast cell growth factor. Ligand-independent activation of KIT appears to be a strong candidate for molecular pathogenesis of GISTs, and it may be a target for future treatment for such tumors. Other genetic changes in GISTs discovered using comparative genomic hybridization include losses in 14q and 22q in both benign and malignant GISTs and occurrence in various gains predominantly in malignant GISTs. GISTs have phenotypic similarities with the interstitial cells of Cajal and, therefore, a histogenetic origin from these cells has been suggested. An alternative possibility, origin of pluripotential stem cells, is also possible; this is supported by the same origin of Cajal cells and smooth muscle and by the common SMA expression in GISTs. GISTs differ clinically and pathogenetically from true leiomyosarcomas (very rare in the GI tract) and leiomyomas. The latter occur in the GI tract, predominantly in the esophagus (intramural tumors) and the colon and rectum (muscularis mucosae tumors). They also differ from schwannomas that are benign S100-positive spindle cell tumors usually presenting in the stomach. GI autonomic nerve tumors (GANTs) are probably a subset of GIST. Other mesenchymal tumors that have to be separated from GISTs include inflammatory myofibroblastic tumors in children, desmoid, and dedifferentiated liposarcoma. Angiosarcomas and metastatic melanomas, both of which are often KIT-positive, should not be confused with GISTs.
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            Clinical significance of oncogenic KIT and PDGFRA mutations in gastrointestinal stromal tumours.

            J Lasota, M Miettinen (2008)
            Gastrointestinal stromal tumours (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract. Despite clinicopathological differences, GISTs share oncogenic KIT or platelet-derived growth factor-alpha (PDGFRA) mutations. Imatinib, KIT and PDGFRA inhibitor, has been successfully used in the treatment of metastatic GISTs. There are primary KIT or PDGFRA mutations diagnosed before imatinib treatment, linked to GIST pathogenesis, and secondary mutations detected during treatment, causing drug resistance. KIT exon 11 mutations are the most common. Gastric GISTs with exon 11 deletions are more aggressive than those with substitutions. KIT exon 11 mutants respond well to imatinib. Less common KIT exon 9 Ala502_Tyr503dup mutants occur predominantly in intestinal GISTs and are less sensitive to imatinib. An Asp842Val substitution in exon 18 is the most common PDGFRA mutation. GISTs with such mutation are resistant to imatinib. PDGFRA mutations are associated with gastric GISTs, epithelioid morphology and a less malignant course of disease. GISTs in neurofibromatosis 1, Carney triad and paediatric tumours generally lack KIT and PDGFRA mutations. Secondary KIT mutations affect exons 13-17. GISTs with secondary mutations in exon 13 and 14 are sensitive to sunitinib, another tyrosine kinase inhibitor. KIT and PDGFRA genotyping is important for GIST diagnosis and assessment of sensitivity to tyrosine kinase inhibitors.
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              Benign schwannoma of the gastrointestinal tract: a clinicopathologic and immunohistochemical study.

              Yutaka Daimaru, Hideki Kido, Hiroshi Hashimoto … (1988)
              A clinicopathologic and immunohistochemical review was made of 24 cases of distinctive nerve sheath tumors located in the gastrointestinal tract. The tumors were microscopically evident in the presence of peripheral lymphoid cuffing and benign nuclear atypia. The tumors arose in the muscularis propria of the stomach in 23 cases and in the ascending colon in 1 case. The patients included 9 men and 15 women whose ages ranged from 36 to 78 years (average, 58). No recurrence has developed in any patient who underwent resection. Although positive immunostaining for S-100 protein, Leu 7 antigen, and laminin might support the schwannian nature of these tumors, the positive immunoreactivity for glial fibrillary acidic protein (GFAP) indicated the possibility of a myenteric plexus origin. The pattern of S-100 protein immunostaining differed from that seen in cases of gastrointestinal stromal tumors associated with von Recklinghausen's neurofibromatosis or that noted in cases of conventional leiomyomas. We propose that these tumors be designated as benign schwannoma of the gastrointestinal tract.
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                Author and article information

                Journal
                Journal ID (nlm-ta): J Int Med Res
                Journal ID (iso-abbrev): J. Int. Med. Res
                Journal ID (publisher-id): IMR
                Journal ID (hwp): spimr
                Title: The Journal of International Medical Research
                Publisher: SAGE Publications (Sage UK: London, England )
                ISSN (Print): 0300-0605
                ISSN (Electronic): 1473-2300
                Publication date (Electronic): 22 September 2020
                Publication date Collection: September 2020
                Volume: 48
                Issue: 9
                Electronic Location Identifier: 0300060520957828
                Affiliations
                [1-0300060520957828]Department of General Surgery, Peking University International Hospital, Beijing, China
                Author notes
                [*]Changsheng Pu, Department of General Surgery, Peking University International Hospital, No. 1 Life Park Road, Zhongguancun Life Science Park, Changping District, Beijing 102206, China. Email: prz_001@ 123456163.com
                Author information
                https://orcid.org/0000-0002-5292-5721
                Article
                Publisher ID: 10.1177_0300060520957828
                DOI: 10.1177/0300060520957828
                PMC ID: 7518005
                PubMed ID: 32962485
                SO-VID: b5744e5f-0fb8-4f5d-a7d4-82323a4b056b
                Copyright © © The Author(s) 2020
                License:

                Creative Commons Non Commercial CC BY-NC: This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License ( https://creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages ( https://us.sagepub.com/en-us/nam/open-access-at-sage).

                History
                Date received : 25 June 2020
                Date accepted : 20 August 2020
                Categories
                Subject: Case Report
                Custom metadata
                typesetter ts2

                Keywords: schwannoma,gastric schwannoma,general surgery,gastroenterology and hepatology,gastrointestinal stromal tumor,laparoscopic wedge resection
                Data availability:
                Keywords: schwannoma, gastric schwannoma, general surgery, gastroenterology and hepatology, gastrointestinal stromal tumor, laparoscopic wedge resection

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