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      • Record: found
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      Extraskeletal Ewing’s sarcoma/peripheral primitive neuroectodermal tumor of the small bowel presenting with gastrointestinal perforation

      case-report

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          Abstract

          Extraskeletal Ewing’s sarcoma (E-EWS)/peripheral primitive neuroectodermal tumor (pPNET) is a rare soft tissue tumor that arises from a multipotent progenitor cell and is considered to be of neuroectodermal origin. Although soft tissue E-EWS/pPNETs are common, they are exceedingly rare in the small bowel. Only 30 cases of E-EWS/pPNET of the small bowel have been reported. However, only one case of gastrointestinal perforation has been reported till today. Here, we report the second case of E- EWS/pPNET with gastrointestinal perforation.

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          The Ewing family of tumors--a subgroup of small-round-cell tumors defined by specific chimeric transcripts.

          O Delattre, J Zucman, T. Melot … (1994)
          Precise diagnosis of small-round-cell tumors is often a challenge to the pathologist and the clinical oncologist. In Ewing's sarcomas and related peripheral primitive neuroectodermal tumors, a t(11;22) translocation or a (21,22) rearrangement is associated with hybrid transcripts of the EWS gene with the FLI1 or ERG gene. To investigate the diagnostic implication of this observation, we searched for these hybrid transcripts in tumors from patients with clinical and radiologic features of Ewing's sarcoma or peripheral primitive neuroectodermal tumors. Samples of RNA from 114 tumors were reverse transcribed and subjected to the polymerase chain reaction with primers designed to amplify the relevant chimeric transcripts. All amplified products were sequenced. In-frame hybrid transcripts were observed in 89 cases. A hybrid transcript was found in 83 of 87 cases (95 percent) of Ewing's sarcoma or peripheral primitive neuroectodermal tumors. Samples of RNA from all of 12 tumors that had been proved to be other than Ewing's sarcoma or neuroectodermal tumors had no hybrid transcript. However, 6 of 15 undifferentiated tumors whose type was ambiguous (nonsecreting, poorly differentiated neuroblastoma or undifferentiated sarcoma) contained a hybrid transcript, suggesting that they might have to be reclassified. A subgroup of small-round-cell tumors identified as belonging to the Ewing family of tumors can be defined according to a specific molecular genetic lesion that is detectable by a rapid, reliable, and efficient method. This approach can be applied to small specimens obtained by fine-needle biopsies.
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            MIC2 is a specific marker for Ewing's sarcoma and peripheral primitive neuroectodermal tumors. Evidence for a common histogenesis of Ewing's sarcoma and peripheral primitive neuroectodermal tumors from MIC2 expression and specific chromosome aberration.

            H Kovar, M Salzer-Kuntschik, S Strehl … (1991)
            This study reports on the specific expression of the MIC2 gene, a pseudoautosomal gene located on the short arms of the X and Y chromosomes, on Ewing's sarcoma (ES) and peripheral primitive neuroectodermal tumor (pPNET) cells. The gene product, a cell membrane protein, is recognized by the newly established monoclonal antibody (MoAb) HBA-71 and the previously described MoAb 12E7 and RFB-1. Furthermore, the reaction pattern of the MIC2 antibodies, especially HBA-71, with normal tissues and a great number of benign and malignant tumors (70 different tumors, 199 tumor samples), as well as the correlation between the specific chromosomal aberrations, i.e., the t(11;22) and the del(22) and the expression of this antigen, are demonstrated. Both ES and pPNET cells express the MIC2 gene in very high amounts, which represents a highly selective and almost unique feature of these cells, making an assignment of these tumors in one entity even more likely. The MIC2 antibodies are of great value for clinical and research purposes.
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              NKX2.2 is a useful immunohistochemical marker for Ewing sarcoma.

              Akihiko Yoshida, Koji Tsuta, Koh Furuta … (2012)
              Ewing sarcoma is a high-grade round cell sarcoma that affects bones and soft tissues in children and young adults. Its diagnosis can be challenging, and the differential diagnoses include a wide variety of small round cell tumors. CD99 and FLI-1 are the currently accepted immunohistochemical markers for Ewing sarcoma, but their accuracy has been controversial. NKX2.2 is a homeodomain-containing transcription factor that plays a critical role in neuroendocrine/glial differentiation. The NKX2.2 gene was recently identified as a target of EWS-FLI-1, the fusion protein specific to Ewing sarcoma, and was shown to be differentially upregulated in Ewing sarcoma on the basis of array-based gene expression analysis. However, the immunohistochemical diagnostic potential of this marker has not been tested. We immunostained representative sections of 30 genetically confirmed Ewing sarcomas and 130 non-Ewing small round cell tumors by using an antibody to NKX2.2. Nuclear staining in at least 5% of the cells was deemed positive. Twenty-eight (93%) of the 30 Ewing sarcomas were positive for NKX2.2. The staining was diffuse (>50%) in all the positive cases and was moderate or strong in intensity for most cases (25 of 28). NKX2.2 was also positive in 14 non-Ewing tumors, including all the olfactory neuroblastomas and a minor subset of small cell carcinomas, synovial sarcomas, mesenchymal chondrosarcomas, and malignant melanomas. All the other non-Ewing tumors tested were negative for this marker. NKX2.2 is a valuable marker for Ewing sarcoma, with a sensitivity of 93% and a specificity of 89%, and aids in the differential diagnosis of small round cell tumors.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Clin Exp Gastroenterol
                Journal ID (iso-abbrev): Clin Exp Gastroenterol
                Journal ID (publisher-id): CEG
                Journal ID (pmc): ceg
                Title: Clinical and Experimental Gastroenterology
                Publisher: Dove
                ISSN (Electronic): 1178-7023
                Publication date (Electronic): 25 June 2019
                Publication date Collection: 2019
                Volume: 12
                Pages: 279-285
                Affiliations
                [1 ] Department of Surgical Gastroenterology, Nishtha surgical hospital and research center , Patan, Gujarat, India
                [2 ] Department of surgery, SSR Medical College , Belle Rive, Mauritius
                Author notes
                Correspondence: Vipul D YagnikDepartment of Surgical Gastroenterology, Nishtha surgical hospital and research center , 77, Siddhraj Nagar, Rajmahal Road, Patan, Gujarat384265, IndiaTel +91 987 950 3296Email vipul.yagnik@ 123456gmail.com
                Article
                Publisher ID: 203697
                DOI: 10.2147/CEG.S203697
                PMC ID: 6600760
                SO-VID: 56afe446-6234-4b49-bc52-a2becfae2d32
                Copyright © © 2019 Yagnik and Dawka.
                License:

                This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License ( http://creativecommons.org/licenses/by-nc/3.0/). By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms ( https://www.dovepress.com/terms.php).

                History
                Date received : 31 January 2019
                Date accepted : 03 April 2019
                Page count
                Figures: 5, Tables: 1, References: 39, Pages: 7
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Gastroenterology & Hepatology
                Keywords: peripheral primitive neuroectodermal tumor,immunohistochemistry,gastrointestinal perforation,surgery,chemotherapy
                Data availability:
                ScienceOpen disciplines: Gastroenterology & Hepatology
                Keywords: peripheral primitive neuroectodermal tumor, immunohistochemistry, gastrointestinal perforation, surgery, chemotherapy

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