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Vol. 33. Issue 4.
Pages 199-203 (July - August 2022)
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Vol. 33. Issue 4.
Pages 199-203 (July - August 2022)
Case Report
Primary central nervous system angiosarcoma with recurrent acute subdural hematoma
Angiosarcoma cerebral primario del sistema nervioso central con hematoma subdural agudo recurrente
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Marc Valera-Meléa,
Corresponding author
marcvaleram@gmail.com

Corresponding author.
, Juan Vicente Darriba Allésa, Fernando Ruiz Juretschkea, Emma Sola Vendrellb, José Manuel Hernández Povedaa, Antonio Montalvo Afonsoc, Vicente Casitas Hernandoa, Roberto García Leala
a Department of Neurosurgery, Hospital General Universitario Gregorio Marañón, Calle Doctor Esquerdo 46, 28007 Madrid, Spain
b Department of Neuropathology, Hospital General Universitario Gregorio Marañón, Calle Doctor Esquerdo 46, 28007 Madrid, Spain
c Department of Neurosurgery, Hospital Universitario de Burgos, Avenida Islas Baleares 3, 09006 Burgos, Spain
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Abstract

Angiosarcoma is an infrequent tumor among sarcomas, especially presenting as a primary tumor within the central nervous system, which can lead to a rapid neurological deterioration and death in few months. We present a 41-year old man with a right frontal enhancing hemorrhagic lesion. Surgery was performed with histopathological findings suggesting a primary central nervous system angiosarcoma. He was discharged uneventfully and received adjuvant chemotherapy and radiotherapy. At 5 months, the follow-up MRI showed two lesions with an acute subdural hematoma, suggesting a relapse. Surgery was again conducted finding tumoral membranes attached to the internal layer of the duramater around the right hemisphere. The patient died a few days later due to the recurrence of the subdural hematoma.

This case report illustrates a rare and lethal complication of an unusual tumor. The literature reviewed shows that gross-total resection with adjuvant radiotherapy seems to be the best treatment of choice.

Keywords:
Angiosarcoma
Central nervous system
Recurrence
Subdural hematoma
Resumen

El angiosarcoma es un tumor infrecuente entre los sarcomas, especialmente cuando se trata de una lesión primaria en el sistema nervioso central, que puede conducir a un rápido deterioro neurológico y a la muerte en pocos meses. Se presenta a un varón de 41 años con una lesión hemorrágica frontal derecha. Se realiza cirugía con resultados histopatológicos que sugieren un angiosarcoma cerebral primario. El paciente recibió tratamiento complementario con quimioterapia y radioterapia. A los cinco meses, el seguimiento con RM mostró dos lesiones y un hematoma subdural agudo, sugiriendo recaída. Se realizó una nueva cirugía hallando membranas tumorales unidas a la cara interna de la duramadre, alrededor del hemisferio derecho. El paciente acabó falleciendo debido a la recurrencia del hematoma subdural.

Este caso ilustra una complicación rara y letal de un tumor infrecuente. La literatura revisada muestra que su tratamiento de elección es la resección completa y la radioterapia adyuvante.

Palabras clave:
Angiosarcoma
Sistema nervioso central
Recurrencia
Hematoma subdural

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