Fact-sheet: Hemangiopericytoma
Updated on 09/17/2021 at 12:16 PM
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Definition
Hemangiopericytoma is a rare mesenchymal tumor arising from Zimmerman's pericytes, contractile cells surrounding capillaries. It is characterized by its malignant potential and high rate of recurrence and distant metastasis, warranting wide surgical excision and adjuvant radiotherapy. Its location is ubiquitous, most often affecting the musculoskeletal system, the pelvis, and the retroperitoneum. Only 25% involve the head and neck (less than 1% of intracranial tumors and 2 to 2.4% of meningeal tumors). When supratentorial, headache is the main symptom. Seizures, sensorimotor deficits, or visual disturbances are less common.
Clinical features
Depends on its location: when supratentorial, headache is the main symptom.
Seizures, sensorimotor deficits, or visual disturbances are less common.
Posterior fossa tumors are mainly responsible for
balance disorders.
CT
Extra-axial tumor, well-defined, lobulated, iso- or hyperdense.
Heterogeneous before and after contrast, more rarely homogeneous.
Cystic or necrotic areas are often present.
Calcifications are exceptional.
MRI
Broad dural attachment base.
Moderate peritumoral edema.
Bone erosion or lysis, but nonspecific, never hyperostosis.
T2: iso- or hyperintense.
Gadolinium: early, intense, and heterogeneous enhancement depending on the degree of necrosis.
Differential diagnosis
Meningioma+++