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Vol. 19. Issue 5.
Pages 446-451 (January 2008)
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Vol. 19. Issue 5.
Pages 446-451 (January 2008)
Hemangiopericitoma de la fosa posterior: a propósito de un caso
Hemangiopericytoma of the posterior fossa: case report
R. Gutiérrez-González
, G.R. Boto, Á. Pérez-Zamarrón, M. Rivero-Garvía
Servicio de Neurocirugía. Hospital Clínico San Carlos. Madrid. España
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Resumen

El hemangiopericitoma es un tumor mesenquimal infrecuente originado de los pericitos de Zimmerman, que habitualmente asienta en tejidos blandos. Su variante meníngea supone menos del 1% de todos los tumores intracraneales. Característicamente presenta un comportamiento agresivo, con marcada tendencia a recurrir localmente y a lo largo del neuroeje, así como a metastatizar en localizaciones extraneurales.

Presentamos el caso de una paciente de 74 años que debutó con clínica inespecífica, observándose durante la exploración física una masa retroauricular no dolorosa a la palpación, adherida a la piel. El estudio de neuroimagen demostró una lesión de gran tamaño localizada en la fosa posterior que realzaba intensamente tras la administración de contraste, asociada a llamativa osteolisis occípito-mastoidea y cuya vascularización provenía exclusivamente de ramas de la arteria carótida externa. La paciente se sometió a la resección quirúrgica completa de la lesión, administrándose radioterapia inicial adyuvante una vez confirmado histológicamente el diagnóstico de hemangiopericitoma. Dieciséis meses después de la intervención, la paciente se encuentra libre de enfermedad.

El tratamiento de elección del hemangiopericitoma intracraneal es su resección quirúrgica completa, siempre que sea técnicamente posible, seguida de radioterapia adyuvante con dosis superiores a los 50 Gy. Esta combinación ha demostrado aumentar el intervalo de tiempo libre de recurrencia. Un estrecho seguimiento a largo plazo de estos pacientes es esencial para detectar recurrencias o metástasis a distancia precozmente, pudiendo aparecer incluso décadas después del adecuado tratamiento inicial.

Palabras clave:
Hemangiopericitoma
Meningioma
Meningioma angioblástico
Metástasis
Neoplasia cerebral
Recurrencia
Abreviaturas:
EMA
HPC
RM
TC
WHO
Summary

Hemangiopericytoma is an uncommon mesenchymal neoplasm arising from Zimmerman's pericytes, which usually locates in soft tissues. Meningeal hemangiopericytoma accounts for less than 1% of all intracranial tumours. Typically, it behaves aggressively, showing distinct tendency to recur locally or distantly along the neural axis and to present extraneural metastases.

We describe a 74-year-old patient who presented unspecific symptoms and whose physical exam revealed a painless retroauricular mass which was adhered to skin. Neuroimaging studies showed a large posterior fossa tumour with intense enhancement after contrast infusion that caused striking occipital-mastoid osteolisis and which was exclusively fed by external carotid artery branches. The patient underwent gross total resection of the tumour, and once the histological diagnosis of hemangiopericytoma was confirmed, she underwent initial adjuvant radiotherapy. Sixteen months after surgery, the patient remains recurrence free.

The treatment of choice of intracranial hemangiopericytoma is gross total resection, which must be attempted when technically feasible, followed by adjuvant radiotherapy providing total doses over 50 Gy. This combination has demonstrated increasing recurrence-free interval in these patients. Close and longterm follow-up is mandatory in order to achieve early diagnosis of recurrence or metastases in these patients, since they can appear several years, even decades, after initial proper treatment.

Key words:
Angioblastic meningioma
Brain neoplasm
Hemangiopericytoma
Meningioma
Metastasis
Recurrence

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