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      Mesenchymal tumours of the gastrointestinal tract

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          Summary

          Mesenchymal tumours represent a heterogenous group of neoplasms encopassing benign, intermediate malignancy, and malignant entities. Sarcomas account for approximately 1% of human malignancies. In consideration of their rarity as well as of intrinsic complexity, diagnostic accuracy represents a major challenge. Traditionally, mesenchymal tumours are regarded as lesions the occurrence of which is mostly limited to somatic soft tissues. However, the occurrence of soft tissue tumours at visceral sites represent a well recognized event, and the GI-tract ranks among the most frequently involved visceral location. There exist entities such as gastrointestinal stromal tumours (GIST) and malignant gastointestinal neuroectodermal tumors that exhibit exquisite tropism for the GI-tract. This review will focus also on other relevant clinico-pathologic entities in which occurrence at visceral location is not at all negligible.

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          Localized benign and malignant fibrous tumors of the pleura. A clinicopathologic review of 223 cases.

          D M England, L Hochholzer, M J McCarthy (1989)
          We reviewed 223 localized fibrous tumors of the pleura and divided them histologically into 141 benign and 82 malignant neoplasms. The criteria used for a judgement of malignancy were high cellularity and mitotic activity (more than four mitotic figures per 10 high-power fields), pleomorphism, hemorrhage, and necrosis. The tumors occurred equally in both sexes, most commonly in the sixth to seventh decades of life. Presenting symptoms included chest pain, dyspnea, and cough; they were observed in three-fourths of patients with a malignant tumor. One in every four of these patients had hypoglycemia, clubbed digits, or pleural effusion. Two-thirds of the tumors were attached to visceral pleura, often by a pedicle. The rest arose from the parietal pleura of the chest wall, diaphragm, or mediastinum. Neoplasms in these atypical sites, together with fissural lesions and tumors "inverted" into peripheral lung, were more often malignant. Most neoplasms measured 5-10 cm and weighed 100-400 g. Microscopically, the "patternless pattern," or hemangiopericytic type, was seen in the majority of cases, and mixed patterns were seen in nearly 40% of tumors. Of the 169 tumors where follow-up was available, all of the benign and 45% of the malignant tumors were cured by simple excision. Patients surgically cured of a malignant neoplasm had pedunculated or well-circumscribed lesions. However, 55% of patients with malignant tumors succumbed to their disease secondary to invasion, recurrence, or metastasis. Resectability is the single most important indicator of clinical outcome. No tumor expressed epithelial differentiation, either immunohistochemically or ultrastructurally; therefore, we favor the term "localized fibrous tumor" of pleura instead of "localized mesothelioma."
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            Identification of recurrent NAB2-STAT6 gene fusions in solitary fibrous tumor by integrative sequencing.

            D. Robinson, Lakshmi Kunju, Juan Miguel Mosquera … (2013)
            A 44-year old woman with recurrent solitary fibrous tumor (SFT)/hemangiopericytoma was enrolled in a clinical sequencing program including whole-exome and transcriptome sequencing. A gene fusion of the transcriptional repressor NAB2 with the transcriptional activator STAT6 was detected. Transcriptome sequencing of 27 additional SFTs identified the presence of a NAB2-STAT6 gene fusion in all tumors. Using RT-PCR and sequencing, we detected this fusion in all 51 SFTs, indicating high levels of recurrence. Expression of NAB2-STAT6 fusion proteins was confirmed in SFT, and the predicted fusion products harbor the early growth response (EGR)-binding domain of NAB2 fused to the activation domain of STAT6. Overexpression of the NAB2-STAT6 gene fusion induced proliferation in cultured cells and activated the expression of EGR-responsive genes. These studies establish NAB2-STAT6 as the defining driver mutation of SFT and provide an example of how neoplasia can be initiated by converting a transcriptional repressor of mitogenic pathways into a transcriptional activator.
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              Risk of recurrence of gastrointestinal stromal tumour after surgery: an analysis of pooled population-based cohorts.

              Heikki Joensuu, Aki Vehtari, Jaakko Riihimäki … (2012)
              The risk of recurrence of gastrointestinal stromal tumour (GIST) after surgery needs to be estimated when considering adjuvant systemic therapy. We assessed prognostic factors of patients with operable GIST, to compare widely used risk-stratification schemes and to develop a new method for risk estimation. Population-based cohorts of patients diagnosed with operable GIST, who were not given adjuvant therapy, were identified from the literature. Data from ten series and 2560 patients were pooled. Risk of tumour recurrence was stratified using the National Institute of Health (NIH) consensus criteria, the modified consensus criteria, and the Armed Forces Institute of Pathology (AFIP) criteria. Prognostic factors were examined using proportional hazards and non-linear models. The results were validated in an independent centre-based cohort consisting of 920 patients with GIST. Estimated 15-year recurrence-free survival (RFS) after surgery was 59·9% (95% CI 56·2-63·6); few recurrences occurred after the first 10 years of follow-up. Large tumour size, high mitosis count, non-gastric location, presence of rupture, and male sex were independent adverse prognostic factors. In receiver operating characteristics curve analysis of 10-year RFS, the NIH consensus criteria, modified consensus criteria, and AFIP criteria resulted in an area under the curve (AUC) of 0·79 (95% CI 0·76-0·81), 0·78 (0·75-0·80), and 0·82 (0·80-0·85), respectively. The modified consensus criteria identified a single high-risk group. Since tumour size and mitosis count had a non-linear association with the risk of GIST recurrence, novel prognostic contour maps were generated using non-linear modelling of tumour size and mitosis count, and taking into account tumour site and rupture. The non-linear model accurately predicted the risk of recurrence (AUC 0·88, 0·86-0·90). The risk-stratification schemes assessed identify patients who are likely to be cured by surgery alone. Although the modified NIH classification is the best criteria to identify a single high-risk group for consideration of adjuvant therapy, the prognostic contour maps resulting from non-linear modelling are appropriate for estimation of individualised outcomes. Academy of Finland, Cancer Society of Finland, Sigrid Juselius Foundation and Helsinki University Research Funds. Copyright © 2012 Elsevier Ltd. All rights reserved.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Pathologica
                Journal ID (iso-abbrev): Pathologica
                Journal ID (publisher-id): PATHOL
                Title: Pathologica
                Publisher: Pacini Editore srl
                ISSN (Print): 0031-2983
                ISSN (Electronic): 1591-951X
                Publication date (Electronic): 01 June 2021
                Publication date Collection: June 2021
                Volume: 113
                Issue: 3
                Pages: 230-251
                Affiliations
                [1 ] Department of Pathology, Azienda Ospedale-Università Padova , Padua, Italy
                [2 ] Department of Medicine, University of Padua School of Medicine , Padua, Italy
                Author notes
                Correspondence Marta Sbaraglia Department of Pathology, Azienda Ospedale-Università Padova, Padua, Italy E-mail: marta.sbaraglia@ 123456unipd.it

                Conflict of interest

                The Authors declare no conflict of interest.

                Author information
                https://orcid.org/0000-0001-6515-5482
                https://orcid.org/0000-0002-1228-8940
                Article
                DOI: 10.32074/1591-951X-309
                PMC ID: 8299319
                PubMed ID: 34294940
                SO-VID: 04110d04-ddc9-422f-985c-3886e2dbc5e9
                Copyright © © 2021 Copyright by Società Italiana di Anatomia Patologica e Citopatologia Diagnostica, Divisione Italiana della International Academy of Pathology
                License:

                This is an open access journal distributed in accordance with the CC-BY-NC-ND (Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International) license: the work can be used by mentioning the author and the license, but only for non-commercial purposes and only in the original version. For further information: https://creativecommons.org/licenses/by-nc-nd/4.0/deed.en

                History
                Date received : 29 June 2021
                Date accepted : 29 June 2021
                Page count
                Figures: 36, Tables: 0, Equations: 0, References: 110, Pages: 22
                Categories
                Subject: Review

                Keywords: mesenchymal tumours,sarcoma,gastrointestinal tract,gist,rare tumours
                Data availability:
                Keywords: mesenchymal tumours, sarcoma, gastrointestinal tract, gist, rare tumours

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