Journal Information
Vol. 29. Issue 6.
Pages 309-313 (November - December 2018)
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Vol. 29. Issue 6.
Pages 309-313 (November - December 2018)
CASO CLÍNICO
Tumor fibroso solitario/hemangiopericitoma de la médula espinal
Solitary fibrous tumour/haemangiopericytoma of the spinal cord
Ana Flores-Justaa,
Corresponding author
annaflores36@gmail.com

Autor para correspondencia.
, Elena López-Garcíaa, Alfredo García-Alluta, Rosa María Reyes-Santíasb
a Servicio de Neurocirugía, Hospital Clínico Universitario de Santiago de Compostela , Santiago de Compostela, A Coruña, España
b Servicio de Anatomía Patológica, Hospital Clínico Universitario de Santiago de Compostela, Santiago de Compostela, A Coruña, España
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Los tumores fibrosos solitarios/hemangiopericitomas (TFS/HPC) son tumores mesenquimales fibroblásticos caracterizados por una fusión genética NAB2-STAT6 con una escasa incidencia en el sistema nervioso central (SNC), de los cuales los espinales son una minoría. Presentamos el caso de una mujer de 63 años con paraparesia progresiva y nivel sensitivo D6 que presentaba una lesión intradural extramedular a nivel D9-D10. Realizamos laminectomía con estudio ecográfico intraoperatorio en que visualizamos una lesión heterogénea con patrón infiltrativo afectando a la médula en varios puntos y realizamos resección de la misma. La immunohistoquímica reveló positividad para vimentina, CD34 y STAT6, Ki67 del 2%, diagnosticándose TFS/HPC fenotipo TFS. Los TFS/HPC del SNC son neoplasias raras que deberían tenerse en cuenta en el diagnóstico diferencial de este tipo de lesiones, la clínica y la resonancia magnética suelen llevar a errores en el enfoque diagnóstico. El apoyo de la ecografía intraoperatoria puede ayudar en la toma de decisiones quirúrgicas.

Palabras clave:
Dorsal
Tumor fibroso solitario
Hemangiopericitoma
Tumor espinal
Resonancia magnética
Abstract

Solitary fibrous tumours/haemangiopericytomas (SFT/HPC) are fibroblastic mesenchymal neoplasms that harbour a genetic fusion of NAB2/STAT6. There are few reported cases in the central nervous system (CNS), of which spinal tumours are minority. We present a case of a 63-year-old woman with progressive paraparesis and a sensory level of T6. On the MRI we detected an intradural extramedullary lesion on T9-T10. We performed a laminectomy with an intraoperative ultrasound study in which we observed a heterogeneous lesion with an infiltrating pattern affecting the medulla at several points, and resection of the lesion. Immunohistological findings revealed positivity for vimentin, CD34 and STAT6; Ki-67 was 2%. A final diagnosis of SFT/HPC with SFT phenotype was made. SFT/HPC of the CNS is a rare neoplasm that should be taken into account in the differential diagnosis of these kinds of lesions, as clinical and MRI findings often lead to errors in diagnostic approach. The support of intraoperative ultrasound can assist in surgical decision-making.

Keywords:
Thoracic
Solitary fibrous tumour
Haemangiopericytoma
Spinal tumour
MRI

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