Tumor tritón maligno en región mandibular y cervical / Malignant triton tumor in the mandibular and cervical region

Introducción: El tumor tritón maligno es una neoplasia rara en la que se encuentran células rabdomioblásticas en un tumor maligno de la vaina de nervios periféricos, que se caracteriza por su agresividad y mal pronóstico. La localización en la cabeza y el cuello es poco frecuente. La inmunohistoquímica juega un papel importante en el diagnóstico. Objetivo: Describir un tumor tritón maligno de tamaño inusual. Presentación del caso: Paciente femenino, de 16 años, es referida al servicio de cirugía maxilofacial del Instituto Nacional de Pediatría, Ciudad de México, con un diagnóstico de tumor neuroectodérmico en región facial y cervical de un año de evolución. Clínicamente el tumor era exofítico, multilobulado, con zonas extensas de necrosis, superficie de varias tonalidades y un tamaño aproximado de 18 x 10 x 12 cm. Se realizó una biopsia e inmunohistoquímica que confirmó el diagnóstico de tumor tritón maligno. La paciente fue intervenida quirúrgicamente, procedimiento con el cual se eliminó totalmente la lesión, con márgenes de seguridad. La paciente presentó una evolución tórpida, con desenlace fatal al cabo de seis meses del tratamiento. Conclusiones: El tumor tritón es una neoplasia agresiva y su detección oportuna orienta al cirujano a ofrecer al paciente un tratamiento adecuado(AU)

Introduction: Malignant triton tumor is a rare neoplasm in which rhabdomyoblasts are present in a malignant tumor of the peripheral nerve sheath. This condition is characterized by its aggressiveness and bad prognosis. Location in the head and neck is infrequent. Immunohistochemical testing plays an important role in its diagnosis. Objective: Describe an unusually large malignant triton tumor. Case presentation: A case is presented of a female 16-year-old patient referred to the maxillofacial surgery service of the National Institute of Pediatrics in Mexico City with a diagnosis of neuroectodermal tumor of one year's evolution in the facial and cervical region. In clinical terms, the tumor was exophytic, multilobed, with extensive areas of necrosis, a surface in several shades of color and an approximate size of 18 x 10 x 12 cm. Biopsy and immunohistochemical testing confirmed the diagnosis of malignant triton tumor. The patient underwent surgery in which the lesion was totally excised with a safety margin. Evolution was clumsy, with a fatal outcome at six months of treatment. Conclusions: Triton tumor is an aggressive neoplasm whose early detection makes it possible for surgeons to provide an appropriate treatment(AU)

Características histopatológicas e inmunohistoquímicas de los mixomas cardiacos / Histopathological and immunohistochemical features of cardiac myxomas

Los mixomas son los tumores cardiacos primarios más frecuentes, con una incidencia estimada de 0,5-1 por 10(6) individuos por año. Estos tumores han generado interés debido a su peculiar localización (el lado izquierdo del septum auricular cerca de la fossa ovalis), su presentación clínica variable y su histogénesis que aún no ha sido definida. La mayoría de los mixomas cardiacos son esporádicos mientras que aproximadamente el 10% de los casos forman parte del complejo de Carney. Esta neoplasia es de histogénesis incierta, sin embargo, se ha propuesto diferenciación endotelial, neurogénica, fibroblástica, muscular lisa, muscular cardiaca y raramente puede presentar diferenciación glandular. Recientemente, por la expresión de algunos factores específicos cardiomiogénicos, se ha propuesto un origen en células progenitoras mesenquimatosas cardiomiocíticas. Histológicamente los mixomas cardiacos están compuestos por células estelares fusiformes y poligonales inmersas en una matriz mixoide amorfa. Por inmunohistoquímica algunos marcadores endoteliales están presentes como el CD31, CD34 y FVIIIAg. Ha sido también informada positividad a la proteína S-100, calretinina, vimentina, desmina, miosina de músculo liso, CD56, α1-antitripsina, y α1-antiquimiotripsina. La resección quirúrgica es actualmente el único tratamiento. Presentamos en este artículo una revisión histopatológica e inmunohistoquímica de los mixomas cardiacos.

Mixomas are the most common primary cardiac tumors with an estimate incidence of 0,5-1 per 10(6) individuals per year. These tumors have generated interest due to their unique location (left side of the atrial septum near the fossa ovalis), variable clinical presentation and undefined histogenesis. Most cardiac myxomas occur sporadically while approximately 10% of diagnosed cases develop as part of Carney complex. This neoplasm is of uncertain histogenesis, however, endothelial, neurogenic, fibroblastic, and cardiac and smooth muscle cells differentiation has been proposed, and rarely glandular differentiation has been observed. Recently, due to the expression of certain cardiomyocyte-specific factors, an origin of mesenchymal cardiomyocytes progenitor cells has been suggested. Histologically cardiac myxomas are mainly composed of stellated, fusiform and polygonal cells, immersed in an amorphous myxoid matrix. Immunohistochemically some endothelial markers, such as CD31, CD34, FVIIIAg, are present. Positive staining has also been reported for S-100 protein, calretinin, vimentin, desmin, smooth muscle myosin, CD56, α1 antitrypsin and α 1 antichymotrypsin. Surgical resection is currently the only treatment of choice. We present in this article a histopathological and immunohistochemical review of cardiac myxomas.

[Histopathological and immunohistochemical features of cardiac myxomas]. / Características histopatológicas e inmunohistoquímicas de los mixomas cardiacos.

Mixomas are the most common primary cardiac tumors with an estimate incidence of 0,5-1 per 10(6) individuals per year. These tumors have generated interest due to their unique location (left side of the atrial septum near the fossa ovalis), variable clinical presentation and undefined histogenesis. Most cardiac myxomas occur sporadically while approximately 10% of diagnosed cases develop as part of Carney complex. This neoplasm is of uncertain histogenesis, however, endothelial, neurogenic, fibroblastic, and cardiac and smooth muscle cells differentiation has been proposed, and rarely glandular differentiation has been observed. Recently, due to the expression of certain cardiomyocyte-specific factors, an origin of mesenchymal cardiomyocytes progenitor cells has been suggested. Histologically cardiac myxomas are mainly composed of stellated, fusiform and polygonal cells, immersed in an amorphous myxoid matrix. Immunohistochemically some endothelial markers, such as CD31, CD34, FVIIIAg, are present. Positive staining has also been reported for S-100 protein, calretinin, vimentin, desmin, smooth muscle myosin, CD56, α1 antitrypsin and α 1antichymotrypsin. Surgical resection is currently the only treatment of choice. We present in this article a histopathological and immunohistochemical review of cardiac myxomas.