Gliosarcoma cerebeloso asociado aneurofibromatosis tipo I: presentación de caso / Cerebellar gliosarcoma associated with neurofibromatosis type I: case presentation

El Gliosarcoma es un raro Glioblastoma que contiene tantos elementos gliales comparable con un Glioblastoma como componentes mesenquimal. Aproximadamente entre 2-8% de todos los Glioblastomas están asociados con elementos sarcomatosos. Clínica y genéticamente muy parecido a los Glioblastomas, excepto por la ausencia de amplificación del EGFR. El gliosarcoma es un tumor de alto grado de malignidad y pobre pronóstico, con alta tasa de recurrencia. Presentamos el caso de un paciente masculino de 54 años de edad con diagnóstico de gliosarcoma cerebeloso, asociado a Neurofibromatosis tipo I. La NF I es el síndrome hereditario más común que predispone a la neoplasia, es una enfermedad polifacética asociado no sólo a tumores benignos.

Gliosarcoma are rare glioblastomas that contain an anaplastic glial component comparable to a glioblastoma, as well as,a mesenchymal component, that have a biphasic pattern. Approximately 2-8% of all glioblastomas are associated with a sarcomatous element. Clinically and genetically close to glioblastomas, except for the absence of EGFR amplification. The Gliosarcoma is a high-grade tumor of malignity and poor prognosis, with high rate of recurrence. We present the case of a masculine patient of 54 elderly years, with diagnosis of Gliosarcoma Cerebellar associate to Neurofibromatosis type I. The NF I is the hereditary syndrome more common that predisposes to the tumor, it is a versatile disease that not only becomes a partner of benign tumors.

Gliosarcoma-Pathology and review of literature

Gliosarcoma is a rare primary brain tumour, estimated to constitute 2-8% of all glioblastomas. We present a case and review of the literature Gliosarcoma is composed of neoplastic glial cells mixed with sarcomatous elements. The distinction between glioblastoma and gliosarcoma is usually made at histopathological examination. These tumours have similar location sex, age distribution and median survivals

Gliosarcoma: a case with unusual epithelial feature

Astrocytic tumors, particularly gliosarcoma, may contain epithelial features in the form of trabecular, adenoid, papillary arrangement, and squamous metaplasia. A case of gliosarcoma with unusual epithelial feature is described. The patient was a 60-year-old male with frequent seizures. The mass was 4 cm and in the left frontal lobe. Trabecular or rarely adenoid arrangement of neoplastic astrocytes was present in the mucinous stroma, and there was a distinctive transition between the trabecular area and typical anaplastic astrocytoma. The tumor cells in the trabecular area showed positive immunostain for glial fibrillary acidic protein, but did not react with various kinds of cytokeratin. The sarcomatous area was undifferentiated and was not labeled by factor-VIII, desmin, and anti-smooth muscle actin. Occurrence and histogenesis of epithelial features in gliosarcoma are reviewed. The importance to recognize the existence of epithelial feature in malignant astrocytic tumor is emphasized.