Hemangiopericytoma
BASIC DESCRIPTION
- Highly vascular, hypercellular meningeal mesenchymal tumor
- Fibroblastic sarcoma
- Synonymous with “solitary fibrous tumor, hemangiocytoma type”
PATHOLOGY
- WHO grade II or III (anaplastic)
- Arises from smooth muscle pericytes around meningeal capillaries
- “Staghorn” pattern of malignant cells surrounding blood vessels
- Hypercellular, increased mitotic index (Ki-67, 5%–10%)
- Markedly increased mitoses, necrosis in WHO grade III tumors
- More aggressive than meningiomas
- Anaplastic tumors have a tendency for systemic metastases
CLINICAL FEATURES
- Adults most commonly afflicted (fouth to sixth decades of life)
- Slight male gender predilection
- Common presenting signs/symptoms: headache
- Treatment: surgical resection ± presurgical embolization due to risk of intraoperative hemorrhage, chemotherapy, radiation therapy
- Long-term patient monitoring due to delayed local recurrence, metastases
- Prognosis: 5-year survival, >90%
IMAGING FEATURES
- General
- Lobulated, enhancing extra-axial mass with dural attachment (dural tail)
- Mimics meningioma but lacks calcification or adjacent calvarial hyperostosis
- Dural sinus invasion, calvarial erosion may be seen
- CT
- Hyperdense extra-axial mass
- Hypodense peritumoral edema
- ±Calvarial erosion
- Heterogeneous but avid enhancement on contrast-enhanced CT imaging
- MRI
- T1WI: heterogeneous signal, ±flow voids
- T2WI: heterogeneous signal, hyperintense peritumoral edema, ±flow voids
- T1WI+C: heterogeneous but avid enhancement, ±dural tail
- MRV: ±dural venous sinus invasion/occlusion
IMAGING RECOMMENDATIONS
- MRI without and with intravenous contrast; CT to help characterize calvarial invasion
For more information, please see the corresponding chapter in Radiopaedia.
Contributor: Rachel Seltman, MD
References
Damodaran O, Robbins P, Knuckey N, et al. Primary intracranial haemangiopericytoma: comparison of survival outcomes and metastatic potential in WHO grade II and III variants. J Clin Neurosci 2014;21:1310–1314. doi.org/10.1016/j.jocn.2013.11.026.
Fountas KN, Kapsalaki E, Kassam M, et al. Management of intracranial meningeal hemangiopericytomas: outcome and experience. Neurosurg Rev 2006;29:145–153. doi.org/10.1007/s10143-005-0001-9.
Liu G, Chen Z-Y, Ma L, et al. Intracranial hemangiopericytoma: MR imaging findings and diagnostic usefulness of minimum ADC values. J Magn Reson Imaging 2013;38:1146–1151. doi.org/10.1002/jmri.24075.
Louis DN, et al. WHO Classification of Tumours of the CNS: Haemangiopericytoma. IARC Press, Lyon, France; 2007:178–180.
Osborn AG, Salzman, KL, Jhaveri MD. Diagnostic Imaging (3rd ed). Elsevier, Philadelphia, PA; 2016.
Sibtain NA, Butt S, Connor SEJ, et al. Imaging features of central nervous system haemangiopericytomas. Eur Radiol 2007;17:1685–1693. doi.org/10.1007/s00330-006-0471-3.
Smith AB, Horkanyne-Szakaly I, Schroeder JW, et al. From the radiologic pathology archives: mass lesions of the dura: beyond meningioma-radiologic-pathologic correlation. Radiographics 2014;34:295–312. doi.org/10.1148/rg.342130075.
Wu W, Shi J, Cheng H, et al. Hemangiopericytomas in the central nervous system. J Clin Neurosci 2009;16:519–523. doi.org/10.1016/j.jocn.2008.06.011.
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