Presentation
Vertigo and headache.
Patient Data
There is a 17*22 mm abnormal signal (iso on T1 and low onT2 sequences) solid, avid enhancing mass lesion at the anatomical region of the pineal gland and superior cerebellar cistern with pressure effect on the tectal plate.
Multiple and confluent high signal foci on T2 and FLAIR sequences in the subcortical and periventricular white matter of both cerebral hemispheres (Fazekaskas III) depict microvascular ischemic events.
VP shunt inserted to the trigone of right lateral ventricle with adjacent gliosis also is seen in the right cerebral hemisphere.
Case Discussion
A pathologically proven case of hemangiopericytoma.
Histopathology
A vascular neoplasm is composed of mild atypical cells with vesicular nuclei, visible nucleoli, eosinophilic and focal clear cytoplasm and stag-horn like intratumoral blood vessels arranged in sheet-like features. No necrosis is seen. Few mitotic figures (up to 3/10 HPF) are noted. Foci of eosinophilic collagenous bands are evident.
IHC study revealed:
- CD34: Positive
- CD117: Positive
- CD99: Positive
- Vimentin: Non specific
- EMA: Negative
- PR; Negative
- S100: Negative
- GFAP: Negative
- Ki 67: Up to 2-3%
FINAL DIAGNOSIS: solitary fibrous tumor of the dura (previously hemangiopericytoma)
Note: Due to molecular/genetic similarities hemangiopericytomas are no longer recognized as distinct entities. Instead, they represent cellular higher-grade solitary fibrous tumors. In the 2016 revised 4th Edition of the WHO classification of CNS tumors, they were grouped together under one diagnosis (solitary fibrous tumor/hemangiopericytoma), and in the 2021 5th edition the term hemangiopericytoma was dropped entirely.
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