Tumor glioneural mixto maligno con heterogeneidad intratumoral: reporte de un caso en un paciente adulto. (Spanish)

BACKGROUND: Gangliogliomas are rare brain tumors, most often affecting children. Histologically, they are neoplasms made up of a neuronal and a glial component. There are few cases in which the neuronal component exhibits malignant transformation. CLINICAL CASE: A 49-year-old male patient, who began his current condition with temporary headache and later aphasia was added. Tomography and magnetic resonance imaging showed a cystic lesion with heterogeneous intramural involvement and central necrosis in the left temporal lobe. Histological study showed areas of anaplastic ganglioglioma, areas of desmoplasia, and an angiomatoid pattern. In the ganglioglioma areas, the neurons were atypical and pleomorphic, with mitotic figures, positive for ENE, synaptophysin, neurofilaments, Neu-N, CD56, nestin and CD99, PGFA, vimentin and CD34. The desmoplastic areas were intensely GFAP. In the mesenchymal and angiomatoid areas there were pleomorphic, atypical, elongated cells inside and outside the capillaries, which phenotypically corresponded to neurons. The tumor was negative for FRAFpE600 and positive for mutated IDH1. mixed malignant neuronal tumor with desmoplastic and angiomatoid pattern, grade III, according to WHO criteria. The patient was discharged and two months later no complementary treatment had been started. CONCLUSIONS: Molecular genetic studies are required to better understand the nature of neural and glioneural tumors, and their respective histological patterns. [ABSTRACT FROM AUTHOR]

ANTECEDENTES: Los gangliogliomas son tumores cerebrales excepcionales, que afectan con mayor frecuencia a los niños. Desde el punto de vista histológico, son neoplasias formadas por un componente neuronal y otro glial. Existen pocos casos en los que el componente neuronal exhibe transformación maligna. CASO CLÍNICO: Paciente masculino de 49 años, que inició su padecimiento actual cefalea temporal y posteriormente se agregó afasia. La tomografía y la resonancia magnética evidenciaron una lesión quística con afectación intramural heterogénea y necrosis central en el lóbulo temporal izquierda. El estudio histológico mostró áreas de ganglioglioma anaplásico, zonas de desmoplasia y un patrón angiomatoide. En las áreas del ganglioglioma, las neuronas eran atípicas y pleomórficas, con figuras de mitosis, positivas a ENE, sinaptofisina, neurofilamentos, Neu-N, CD56, nestina y CD99, PGFA, vimentina y CD34. Las áreas desmoplásicas fueron intensamente a GFAP. En las áreas mesenquimatosas y angiomatoides hubo células pleomórficas, atípicas, alargadas dentro y fuera de los capilares, que correspondían fenotípicamente a neuronas. El tumor fue negativo para FRAFpE600 y positivo para IDH1 mutado. de tumor mixto neuronal maligno con patrón desmoplásico y angiomatoide, grado III, según los criterios de la OMS. El paciente se dio de alta y dos meses después no se había iniciado tratamiento complementario. CONCLUSIONES: Se requieren estudios genéticos moleculares para entender de forma más clara la naturaleza de los tumores neurales y glioneurales, y sus respectivos patrones histológicos. [ABSTRACT FROM AUTHOR]

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