Meningeal hemangiopericytoma (historical)
Citation, DOI & article data
Hemangiopericytomas of the meninges are rare tumors of the meninges, now considered to be an aggressive form of solitary fibrous tumors of the dura. They often present as large and locally aggressive dural masses, frequently extending through the skull vault. They are difficult to distinguish on imaging from the far more common meningioma but are treated similarly with surgical resection with or without radiotherapy to reduce the risk of recurrence, which is high.
The remainder of the article presents a historical perspective of this entity. For a current discussion please refer to solitary fibrous tumors of the dura.
Hemangiopericytomas have been enigmatic tumors with a long and checkered history of changing name and classification.
They were previously classified as angioblastic sub-type meningiomas, then considered to arise from smooth muscle perivascular pericytes of dural capillaries (pericytes of Zimmerman) 3.
More recent studies suggest that these lesions are actually arising from fibroblast and are in the spectrum of the solitary fibrous tumors of the dura 4. This is further supported by the fact that both entities share a similar genetic alteration: genomic inversion of 12q13 locus resulting in the fusion of NAB2 and STAT6 genes, the latter expressed and able to be assessed using immunohistochemistry techniques 6.
This resulted in the term being abandoned throughout the body, but for some time it tenaciously persisted in the CNS classification on account of what was felt to be distinctive imaging features.
In the revised 4th edition (2016) of the WHO classification of CNS tumors, it ceased to be a distinct entity but the term remained in use under the compound diagnosis "solitary fibrous tumor/hemangiopericytoma" 6.
In the 5th edition (2021) the term was finally officially retired 7, although it is likely to persist for some time in everyday use. Solitary fibrous tumors grade 2 or 3 are equivalent to hemangiopericytomas 7.
Hemangiopericytomas accounted for less than 1% of all intracranial tumors 1. They were typically encountered in younger adults (30-50 years) with up to 10% being diagnosed in children 3. There is a slight male predilection (M: F 1.4:1) 3,6.
Clinical presentation was usually due to mass effect and will vary depending on location. Headache, seizures, focal neurological dysfunction may all be presenting features 3. Additionally, in up to 20% of cases, these tumors can metastasize systemically, typically to liver, lung, and bone 1,3,6.
Solitary fibrous tumors of the dura can be graded from WHO grade 1 to 3 with what traditionally has been termed hemangiopericytomas being grade 2 or 3 6.
Hemangiopericytomas were highly cellular tumors with frequent mitoses (grade II <5 per 10 HPF; grade III ≥5 per 10 HPF) and often with areas of necrosis 6. The cells are separated by a limited amount of delicate reticulin fibers and have numerous "staghorn" vessels, the latter a feature shared by solitary fibrous tumors of the dura 6.
Ideally, the diagnosis is confirmed by assessing for STAT6 expression by immunohistochemistry or identifying NAB2-STAT6 fusion 6. Hemangiopericytomas had a number of useful immunohistochemical markers 6:
- STAT6: positive
- CD34: positive
- vimentin: positive
Ki-67 proliferation index is typically around 10% 6.
Hemangiopericytomas were almost always solitary, usually supratentorial masses, often lobulated in contour. They were highly vascular and had a tendency to erode adjacent bone 3.
Another common location was the posterior fossa in the posterior occipital region.
- vivid enhancement
- erosion of adjacent bone
- no hyperostosis
- no calcification
Features on various sequences included:
- T1: isointense to grey matter
T1 C+ (Gd)
- vivid enhancement
- may have a narrow base of dural attachment
- dural tail sign is seen, more commonly in grade II tumors
- isointense to grey matter
- multiple flow voids on MRI (need to distinguish from the spoke-wheel appearance of meningioma)
- adjacent brain edema frequently present
- MR spectroscopy
- intermediate restricted diffusion (less than meningioma)
- minimum ADC ~1100 (+/- 130) x 10-6 mm2/s
- ECA, ICA and vertebral supply common
- highly vascular
- corkscrew arteries
- fluffy tumor stain
- lack of early draining veins 3
- useful for pre-operative embolization
- assessment of dural venous sinus involvement
Treatment and prognosis
Total surgical excision was recommended, with pre-operative catheter embolization helpful in limiting blood loss 3. Adjuvant radiotherapy to reduce the incidence of recurrence has also been advocated 1,3.
The main differential diagnosis was that of meningioma although all other dural masses should be considered. Distinguishing a hemangiopericytoma from a meningioma was difficult as they have similar appearances on both CT and MRI.
- older patients (>50 years of age)
- central vascular spoke-wheel vascular supply
- less likely to erode adjacent bone
- more likely to cause hyperostosis
- more likely to be multiple
- very unlikely to metastasize
- usually, have a broad dural attachment and dural tail
- MRS: alanine peak, absent myoinositol peak
- immunohistochemistry: EMA positive, CD34 and STAT6 negative
- 1. Chiechi MV, Smirniotopoulos JG, Mena H. Intracranial hemangiopericytomas: MR and CT features. AJNR Am J Neuroradiol. 1996;17 (7): 1365-71. AJNR Am J Neuroradiol (abstract) - Pubmed citation
- 2. Cosentino CM, Poulton TB, Esguerra JV et-al. Giant cranial hemangiopericytoma: MR and angiographic findings. AJNR Am J Neuroradiol. 14 (1): 253-6. AJNR Am J Neuroradiol (abstract) - Pubmed citation
- 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 (2): 295-312. doi:10.1148/rg.342130075 - Pubmed citation
- 4. Schweizer L, Koelsche C, Sahm F et-al. Meningeal hemangiopericytoma and solitary fibrous tumors carry the NAB2-STAT6 fusion and can be diagnosed by nuclear expression of STAT6 protein. Acta Neuropathol. 2013;125 (5): 651-8. doi:10.1007/s00401-013-1117-6 - Pubmed citation
- 5. Louis DN, Perry A, Reifenberger G et-al. The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. Acta Neuropathol. 2016;131 (6): 803-20. doi:10.1007/s00401-016-1545-1 - Pubmed citation
- 6. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK "WHO Classification of Tumours of the Central Nervous System. 4th Edition Revised" ISBN: 9789283244929
- 7. Louis D, Perry A, Wesseling P et al. The 2021 WHO Classification of Tumors of the Central Nervous System: A Summary. Neuro-Oncology. 2021;23(8):1231-51. doi:10.1093/neuonc/noab106 - Pubmed