Abstract
The hemangiopericytomas (HPCs) are rare tumors of the dural base and correspond of less than 1% of all primary Central Nervous System (CNS) tumors. It usually affects adult males with 40 to 50 years old and has a supratentorial location (70%), followed by posterior fossa (15%) and spine (15%). Generally, it has fast progression and manifests clinically through headache, focal neurological deficits and epileptic seizures. The tumor has high incidence of local recurrence, from 34% to 90%, and it is estimated that the follow-up of these patients should be done for at least 7, 5 years. The percentage of long-distance metastases is lower, 12% to 55%, affecting especially bones, lungs and liver, in decreasing order. The objective of this study is to evaluate the contradictions when approaching these tumors and to analyze a rare case of pediatric involvement. It was prepared a case report and a literature review was made using the data bases MEDLINE®, PubMed and Cochrane, where 31 relevant articles were selected related to the proposed study. The outcome of HPC treatment can change according to the tumor’s location in the CNS or extracranial, the tumor’s size and the histopathological classification. Therefore, many studies have been proposed observing the answer of the surgical resection, the adjuvant radiotherapy, the radiosurgery and the antiangiogenic therapies.
Highlights
The hemangiopericytoma (HPC) is considered a variant from the extra pleural solitary fibrous tumor, a rare tumor of dural base, which has a large histological spectrum
The distribution inside the Central Nervous System (CNS) is similar to the meningioma, in which 70% are supratentorial, 15% are in the posterior fossa and 15% are in the spine
Reports suggests that the average time for local recurrence is about 5 years, while the average for long
Summary
The hemangiopericytoma (HPC) is considered a variant from the extra pleural solitary fibrous tumor, a rare tumor of dural base, which has a large histological spectrum. It is responsible for less than 1% of all primary tumors of the CNS (Central Nervous System), and only 2, 1% are HPC grade II, mainly occurring in adults between 40 and 50 years old with a small predominance on males [1, 2]. Reports suggests that the average time for local recurrence is about 5 years, while the average for long
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