El histiocitoma fibroso maligno es el sarcoma de partes blandas más frecuente en el adulto, con una mayor incidencia entre los 40 y 60 años, afectando mayoritariamente a los varones. El 70% de los casos se sitúan en las extremidades. La localización intracraneal del HFM es muy poco frecuente y la mayoría de las veces se originan en la duramadre o las leptomeninges.
Presentamos el caso clínico de un paciente de 60 años afecto de un histiocitoma fibroso maligno, originado en la duramadre craneal, que fue extirpado completamente, y con un seguimiento de 12 meses tras el diagnóstico.
Realizamos una revisión de los 24 casos recogidos en la literatura y analizamos las características clínicas y terapéuticas.
Malignant fibrous histiocytoma is the most common soft tissue sarcoma in adults with the majority of cases that occur in patients between 50–70 years and most of cases occurs in male. Seventy percent originate from extremities. Primary MFH involving the central nervous system is a rare occurrence with most cases originating from the dura or leptomeninges.
A case of primary intracranial malignant fibrous histiocytoma in a 60-year-old men is presented. The tumor was successfully excised and the patient remain well at follow-up of 12 months.
The clinical features of this case and the therapeutic prognosis of 24 cases reported previously in the literature were reviewed.
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