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Vol. 24. Issue 4.
Pages 178-182 (July - August 2013)
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Vol. 24. Issue 4.
Pages 178-182 (July - August 2013)
Caso clínico
Fibrosarcoma epitelioide esclerosante a nivel paraespinal. Caso clínico y revisión de la literatura
Sclerosing epithelioid fibrosarcoma of the paravertebral column. Case report and literature review
Patricia Puerta Roldána,
Corresponding author
puertaroldan82@gmail.com

Autor para correspondencia.
, Rodrigo Rodríguez Rodrígueza, Silvia Bagué Rossellb, Manel de Juan Delagoc, Joan Molet Teixidóa
a Servicio de Neurocirugía, Hospital de la Santa Creu i Sant Pau, Barcelona, España
b Servicio de Anatomía Patológica, Hospital de la Santa Creu i Sant Pau, Barcelona, España
c Servicio de Radiología, Hospital de la Santa Creu i Sant Pau, Barcelona, España
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Tabla 1. Casos de fibrosarcoma epitelioide esclerosante publicados en la literatura con afectación del sistema nervioso (2012)
Resumen

El fibrosarcoma epitelioide esclerosante (SEF) es una variante poco frecuente de fibrosarcoma de bajo grado con características histológicas e inmunohistoquímicas bien definidas, caracterizado por su mal pronóstico. Presentamos un caso de SEF a nivel paraespinal en un paciente varón de 49 años con un tumor que se extiende hacia el foramen L4-L5 e invade la raíz L5. La histología de la pieza quirúrgica y el estudio inmunohistoquímico fueron compatibles con SEF. Este caso es particularmente inusual por su origen a nivel paraespinal e ilustra, a pesar de su bajo grado, el potencial de malignidad del SEF.

Palabras clave:
Fibrosarcoma epitelioide esclerosante
Tumor paraespinal
Vimentina
Metástasis tardías
Pronóstico
Sistema nervioso
Abstract

Sclerosing epithelioid fibrosarcoma (SEF) is a rare variant of low-grade fibrosarcoma, with specific histological and immunohistochemical features and a poor prognosis. We report a case of SEF of the paravertebral column in a 49-year old male who presented a paraspinal mass with extension into the L4-L5 neural foramen and invasion of the L5 nerve root. Histology of the tumourectomy specimen and its immunohistochemical study led to the diagnosis of SEF. This case was particularly unusual due to its paravertebral column location and, despite its low grade, illustrates the malignant potential of SEF.

Keywords:
Sclerosing epithelioid fibrosarcoma
Paraspinal tumour
Paravertebral column tumour
Vimentin
Late metastases
Prognosis
Nervous system

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