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Hemangiopericytoma invading the craniovertebral junction: First reported case and review of the literature

Occurrence of hemangiopericytoma (HPC) in the central nervous system is rare. Spinal HPCs with intramedullary involvement are even more unusual. We present a case of a craniovertebral intradural HPC with both extra- and intra-medullary extensions. Though the patient presented with vague cervical sym...

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Detalles Bibliográficos
Autores principales: Drazin, Doniel, Shweikeh, Faris, Bannykh, Serguei, Johnson, J. Patrick
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3872659/
https://www.ncbi.nlm.nih.gov/pubmed/24381454
http://dx.doi.org/10.4103/0974-8237.121622
Descripción
Sumario:Occurrence of hemangiopericytoma (HPC) in the central nervous system is rare. Spinal HPCs with intramedullary involvement are even more unusual. We present a case of a craniovertebral intradural HPC with both extra- and intra-medullary extensions. Though the patient presented with vague cervical symptoms, imaging was indicative of an intradural lesion from the occiput to C4 and a second smaller, subclinical lesion, at the T2-3 level. He underwent gross total surgical resection of the craniovertebral lesion and did well post-operatively. The thoracic lesion was treated with radiosurgery and the patient is neurologically at baseline 5 years later. Gross total resection of HPCs is the recommended treatment when possible. Histopathology is crucial for diagnosis due to both its rarity and similar characteristics to other tumors on physical and radiographic evaluations. Recognizing that these uncommon tumors can occur with both extra-medullary and intra-medullarly locations are important for diagnosis and treatment recommendations. Future studies using national surgical databases that contain histology will be needed to understand the long-term clinical outcomes.