Resumen Un sarcoma es un tumor maligno procedente de células mesenquimales primitivas, que en circunstancias normales se desarrolla en los tejidos de apoyo como los músculos y los huesos. Existen tres variables del rabdomiosarcoma de acuerdo a sus presentaciones histológicas: el embrionario, el alveolar y el pleomórfico, siendo el rabdomiosarcoma alveolar el más agresivo. Se presenta en un paciente masculino de ocho años de edad, el cual inicia con elevación sin cambio de color en ala nasal izquierda de 3-4 mm, de crecimiento progresivo, diagnosticada como rabdomiosarcoma alveolar documentado por patología como tumor de células redondas, pequeñas y azules, con inmunohistoquímica positiva a desmina y miogenina. La resonancia de cráneo y cara, la tomografía computarizada de tórax y el aspirado de médula óseafueron negativos para extensión tumoral. Se efectúa resección de su lesión, la cual se resecó macroscópicamente al 100%, reportando patología focos aislados de tumor, con tumor residual microscópico en márgenes laterales, por lo que queda finalmente como E II. Se trata de una neoplasia maligna muy rara con una presentación inusual, con reporte de cuatro casos en la literatura, de ahí surge la importancia de tener en cuenta esta posibilidad diagnóstica, así como los conocimientos para su abordaje. (creativecommons.org/licenses/by-nc-nd/4.0/). Abstract A sarcoma is defined as a malignant tumor from primitive mesenchymal cells that, in normal circumstances, is developed in bone and muscular tissues. In accordance with their histological differences, three different types of rhabdomyosarcoma have been identified: embryonal, alveolar and pleomorphic, being the alveolar rhabdomyosarcoma the most aggressive of them. Male patient age 8, which starts with no color change elevation in left nasal wing of approximately 3-4 mm of progressive growth, diagnosed as alveolar rhabdomyosarcoma tumor documented as a tumor of small round blue cells, with desmin and myogenin positive immunohistochemistry. It was detected in the magnetic resonance of skull and face, in chest computed tomography and bone marrow aspirate, negative for tumor. Resection of the lesion, which is resected 100% macroscopically, reporting disease outbreaks isolated tumor with microscopic residual tumor in side margins, finally occurring as E II. This is a very rare malignancy with an unusual presentation, around 4 cases have been reported in the literature, hence the importance of taking into account this diagnostic possibility and knowledge to address them.
Sarcoma is a malignant tumor originating in mesenchymal primitive cells that in normal circumstances develops in supportive tissues such as muscle and bone. There are three rhabdomyosarcoma variants according to its histological presentation: Embryonal, alveolar, and pleomorphic with alveolar rhabdomyosarcoma being the most aggressive. The case is presented of an 8-year-old male patient who started with a progressively growing left nasal wing 3-4 mm mass with no color change diagnosed as an alveolar rhabdomyosarcoma with pathology documenting a small round blue cell tumor with positive immunohistochemistry to desmin and myogenin. Skull and face magnetic resonance, chest computed tomography, and bone marrow aspirate were negative for disease extension. The lesion was macroscopically resected at 100%, with pathology reporting isolated tumor foci, with microscopic residual tumor on lateral margins, and it is therefore finally classified as E II. This is a very rare neoplasm with an unusual presentation, with only 4 cases reported in the literature, hence, the importance of taking into account this diagnostic possibility as well as the knowledge on how to approach it. (creativecommons.org/licenses/by-nc-nd/4.0/).
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