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      Angiosarcoma metastásico de la cavidad oral: a propósito de un caso Translated title: Metastatic angiosarcoma of the oral cavity: report of a case

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          Abstract

          RESUMEN El angiosarcoma oral es un tumor endotelial raro que, debido a su presentación clínica, requiere ser comparada con varias lesiones similares como diagnóstico diferencial. Presentamos un paciente de 66 años en tratamiento por múltiples episodios hemorrágicos digestivos, derivada al Servicio de Cirugía Oral y Maxilofacial del Hospital de Carabineros de Santiago de Chile debido a la presencia de lesiones vasculares intraorales. Se diagnostican inicialmente como lesiones metastásicas de un tumor estromal gastrointestinal, siendo el diagnóstico histopatológico final de un angiosarcoma agresivo y diseminado. La presentación clínica de este tipo de lesiones dificulta el diagnóstico definitivo y, en consecuencia, el manejo de este tipo de pacientes.

          Translated abstract

          ABSTRACT Oral angiosarcoma is a rare endothelial tumour that requires to be included as a differential diagnosis of several similar lesions due to its clinical presentation. We present a 66-year-old patient undergoing treatment for multiple digestive bleeding episodes, referred to the oral and maxillofacial surgery service of the Hospital de Carabineros de Santiago de Chile due to the presence of intraoral vascular lesions initially diagnosed as metastatic lesions of a gastrointestinal stromal tumour, with the final histopathological diagnosis being an aggressive and disseminated angiosarcoma. The clinical presentation of these lesions makes the diagnosis and, consequently, the management of this type of patient difficult.

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          Most cited references10

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          Current clinical management of gastrointestinal stromal tumor

          Gastrointestinal stromal tumors (GISTs) are the most common malignant subepithelial lesions (SELs) of the gastrointestinal tract. They originate from the interstitial cells of Cajal located within the muscle layer and are characterized by over-expression of the tyrosine kinase receptor KIT. Pathologically, diagnosis of a GIST relies on morphology and immunohistochemistry [KIT and/or discovered on gastrointestinal stromal tumor 1 (DOG1) is generally positive]. The prognosis of this disease is associated with the tumor size and mitotic index. The standard treatment of a GIST without metastasis is surgical resection. A GIST with metastasis is usually only treated by tyrosine kinase inhibitors without radical cure; thus, early diagnosis is the only way to improve its prognosis. However, a GIST is usually detected as a SEL during endoscopy, and many benign and malignant conditions may manifest as SELs. Conventional endoscopic biopsy is difficult for tumors without ulceration. Most SELs have therefore been managed without a histological diagnosis. However, a favorable prognosis of a GIST is associated with early histological diagnosis and R0 resection. Endoscopic ultrasonography (EUS) and EUS-guided fine needle aspiration (EUS-FNA) are critical for an accurate diagnosis of SELs. EUS-FNA is safe and effective in enabling an early histological diagnosis and adequate treatment. This review outlines the current evidence for the diagnosis and management of GISTs, with an emphasis on early management of small SELs.
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            A gist of gastrointestinal stromal tumors: A review.

            Gastrointestinal stromal tumors (GISTs) have been recognized as a biologically distinctive tumor type, different from smooth muscle and neural tumors of the gastrointestinal tract (GIT). They constitute the majority of gastrointestinal mesenchymal tumors of the GIT and are known to be refractory to conventional chemotherapy or radiation. They are defined and diagnosed by the expression of a proto-oncogene protein detected by immunohistochemistry which serves as a crucial diagnostic and therapeutic target. The identification of these mutations has resulted in a better understanding of their oncogenic mechanisms. The remarkable antitumor effects of the molecular inhibitor imatinib have necessitated accurate diagnosis of GIST and their distinction from other gastrointestinal mesenchymal tumors. Both traditional and minimally invasive surgery are used to remove these tumors with minimal morbidity and excellent perioperative outcomes. The revolutionary use of specific, molecularly-targeted therapies, such as imatinib mesylate, reduces the frequency of disease recurrence when used as an adjuvant following complete resection. Neoadjuvant treatment with these agents appears to stabilize disease in the majority of patients and may reduce the extent of surgical resection required for subsequent complete tumor removal. The important interplay between the molecular genetics of GIST and responses to targeted therapeutics serves as a model for the study of targeted therapies in other solid tumors. This review summarizes our current knowledge and recent advances regarding the histogenesis, pathology, molecular biology, the basis for the novel targeted cancer therapy and current evidence based management of these unique tumors.
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              Predisposing factors for poor prognosis of angiosarcoma of the scalp and face: Systematic review and meta-analysis.

              Angiosarcoma of the scalp and face is an aggressive tumor with a high rate of local recurrence. Although many previous studies have demonstrated risk factors for poor prognosis, debate remains. We identified the predisposing factors for poor prognosis through comprehensive review of selected studies with meta-analysis.
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                Author and article information

                Journal
                maxi
                Revista Española de Cirugía Oral y Maxilofacial
                Rev Esp Cirug Oral y Maxilofac
                Sociedad Española de Cirugía Oral y Maxilofacial y de Cabeza y Cuello (Madrid, Madrid, Spain )
                1130-0558
                2173-9161
                June 2022
                : 44
                : 2
                : 83-86
                Affiliations
                [1] Santiago Santiago de Chile orgnameUniversidad de Los Andes orgdiv1Hospital de Carabineros (HOSCAR). Santiago, Chile orgdiv2Departamento de Cirugía Oral y Maxilofacial Chile
                Article
                S1130-05582022000200083 S1130-0558(22)04400200083
                10.20986/recom.2022.1360/2022
                5ff452c6-52fb-4dc8-856c-849b817e0d2b

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

                History
                : 31 May 2022
                : 09 April 2022
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 10, Pages: 4
                Product

                SciELO Spain

                Categories
                Casos Clínicos

                angiosarcoma,tumores vasculares,Oral cavity,vascular tumors,Cavidad oral

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