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      Schwannoma intraóseo mandibular. Reporte de un caso clínico Translated title: Mandibular intraosseous schwannoma. A clinical case report

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          Abstract

          Resumen Objetivo El schwannoma es un tumor neuroectodérmico benigno de la vaina nerviosa o vaina de mielina formada por células de Schwann. Aproximadamente entre el 25 y el 48% de los casos presentan localización en el territorio cervicofacial, especialmente en los tejidos blandos de esta región. Se los puede clasificar como schwannomas periféricos o intraóseos, éstos últimos también denominados centrales. Los schwannomas intraóseos son poco comunes, constituyen menos del 1% de los schwannomas presentes en la región en cuestión y menos del 0,2% de todos los tumores primarios óseos. El presente trabajo tiene como objetivo reportar un caso clínico de un schwannoma intraóseo mandibular, revisando aspectos clínicos, radiográficos y anatomopatológicos. El schwannoma intraóseo es una entidad poco común, o al menos se encuentra en una condición de subregistro en Argentina, por lo que este caso constituye una rareza. Caso clínico Se presentó a la consulta una paciente de 30 años de edad, derivada al servicio de Cirugía y Traumatología Bucomaxilofacial del Hospital “Parmenio Piñero” de la Ciudad Autónoma de Buenos Aires por su odontólogo de cabecera, a raíz de un hallazgo radiográfico durante un control de rutina. Se planificó realizar una biopsia incisional, cuyo resultado anatomopatológico fue compatible con el diagnóstico de schwannoma intraóseo. Se procedió a realizar la enucleación completa. Finalmente, la paciente evolucionó sin complicaciones.

          Translated abstract

          Abstract Aim Schwannoma is a benign neuroectodermal tumor of the nerve sheath or myelin sheath formed by Schwann cells. Approximately between 25 and 48% of the cases are located in the cervicofacial territory, especially in the soft tissues of this region. They can be classified into peripheral and intraosseous schwannomas, the last one can also be reported as central. Intraosseous schwannomas are rare, constituting less than 1% of schwannomas present in the region and less than 0.2% of all primary bone tumors. This publication aims to report a clinical case of mandibular intraosseous schwannoma, reviewing clinical, radiographic and anatomopathological aspects. Intraosseous schwannoma is a rare entity, or at least is under a condition of underreport in Argentina, so this case is a rarity. Clinical case A 30-year-old patient, referred to the Buccomaxillofacial Surgery and Traumatology service of the “Parmenio Piñero” Hospital of Ciudad Autónoma de Buenos Aires by her dentist, because of a radiographic finding during a routine check. An incisional biopsy was performed, the anatomopathological result of which was compatible with the diagnosis of intraosseous schwannoma. A complete enucleation was performed under local anesthesia. Finally, the patient evolved without complications.

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          Most cited references22

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          Schwannomas and their pathogenesis.

          Schwannomas may occur spontaneously, or in the context of a familial tumor syndrome such as neurofibromatosis type 2 (NF2), schwannomatosis and Carney's complex. Schwannomas have a variety of morphological appearances, but they behave as World Health Organization (WHO) grade I tumors, and only very rarely undergo malignant transformation. Central to the pathogenesis of these tumors is loss of function of merlin, either by direct genetic change involving the NF2 gene on chromosome 22 or secondarily to merlin inactivation. The genetic pathways and morphological features of schwannomas associated with different genetic syndromes will be discussed. Merlin has multiple functions, including within the nucleus and at the cell membrane, and this review summarizes our current understanding of the mechanisms by which merlin loss is involved in schwannoma pathogenesis, highlighting potential areas for therapeutic intervention.
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            Morphologic and immunohistochemical features of malignant peripheral nerve sheath tumors and cellular schwannomas.

            Cellular schwannoma is an uncommon, but well-recognized, benign peripheral nerve sheath tumor, which can be misdiagnosed as malignant peripheral nerve sheath tumor. To develop consensus diagnostic criteria for cellular schwannoma, we reviewed 115 malignant peripheral nerve sheath tumor and 26 cellular schwannoma cases from two institutions. Clinical data were retrieved from the electronic medical records, and morphologic features, maximal mitotic counts, Ki67 labeling indices, and immunohistochemical profiles (SOX10, SOX2, p75NTR, p16, p53, EGFR, and neurofibromin) were assessed. Several features distinguish cellular schwannoma from malignant peripheral nerve sheath tumor. First, in contrast to patients with malignant peripheral nerve sheath tumor, no metastases or disease-specific deaths were found in patients with cellular schwannoma. More specifically, 5-year progression-free survival rates were 100 and 18%, and 5-year disease-specific survival rates were 100 and 32% for cellular schwannoma and malignant peripheral nerve sheath tumor, respectively. Second, the presence of Schwannian whorls, a peritumoral capsule, subcapsular lymphocytes, macrophage-rich infiltrates, and the absence of fascicles favored the diagnosis of cellular schwannoma, while the presence of perivascular hypercellularity, tumor herniation into vascular lumens, and necrosis favor malignant peripheral nerve sheath tumor. Third, complete loss of SOX10, neurofibromin or p16 expression, or the presence of EGFR immunoreactivity was specific for malignant peripheral nerve sheath tumor (P<0.001 for each). Expression of p75NTR was observed in 80% of malignant peripheral nerve sheath tumors compared with 31% of cellular schwannomas (P<0.001). Fourth, Ki-67 labeling indices ≥20% were highly predictive of malignant peripheral nerve sheath tumor (87% sensitivity and 96% specificity). Taken together, the combinations of these histopathological and immunohistochemical features provide useful criteria to distinguish between malignant peripheral nerve sheath tumor and cellular schwannoma with high sensitivity and specificity. Additional retrospective and prospective multicenter studies with larger data sets will be required to validate these findings.
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              Ancient schwannoma of upper lip: case report with distinct histologic features and review of the literature.

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                Author and article information

                Journal
                raoa
                Revista de la Asociación Odontológica Argentina
                Rev. Asoc. Odontol. Argent.
                Asociación Odontológica Argentina (Ciudad Autónoma de Buenos Aires, , Argentina )
                2683-7226
                December 2022
                : 110
                : 3
                : 4
                Affiliations
                [1] Buenos Aires Buenos Aires orgnameUniversidad de Buenos Aires orgdiv1Servicio de Cirugía General Argentina
                [2] Buenos Aires orgnameHospital General de Agudos “Parmenio Piñero” orgdiv1Servicio de Cirugía y Traumatología Bucomaxilofacial Argentina
                Article
                S2683-72262022000300004 S2683-7226(22)11000300004
                10.52979/raoa1101232.1202
                49f8d113-359e-4063-a32f-2710addf209c

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

                History
                : 24 October 2022
                : 07 September 2022
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 22, Pages: 1
                Product

                SciELO Argentina

                Categories
                Cirugía

                tumor,intraosseous,nervous,neurilemmoma,schwannoma,intraóseo,nervioso,neurilenoma

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