Turkish Neurosurgery
Intracranial Solitary Fibrous Tumor/Hemangiopericytoma: A Clinicoradiological Poorly Recognized Entity- An Institutional Experience
Nibedita Sahoo1, Debahuti Mohapatra1, Souvagya Panigrahi2, Anasuya Lenka 1, Prateek Das1, Satya S. Mohapatra3
1IMS and SUM Hospital, Pathology, BHUBANESWAR,
2IMS and SUM Hospital, Neurosurgery, BHUBANESWAR,
3IMS and SUM Hospital, Radiology, BHUBANESWAR,
DOI: 10.5137/1019-5149.JTN.31204-20.2

Aim:Intracranial solitary fibrous tumors (SFTs) and hemangiopericytomas (HPCs) are rare nonmeningothelial mesenchymal tumors sharing fusion of NGF1-A binding protein 2 (NAB2) and signal transducers and activators of transcription (STAT6.).The WHO classification of central nervous system (CNS) tumors (2016) highlights that molecular confirmation of NAB2/STAT6 fusion or immunohistochemical nuclear expression of STAT6 is mandatory for the diagnosis of SFT/HPC.Herein, we present a series of four cases of SFT/HPC of the brain, which mimicked other CNS tumors both clinically and radiologically.Material and Methods:This is a retrospective study over a period of two and a half years. Out of the 156 operated cases of brain tumors, four patients (2.56%) were diagnosed with SFT/HPC. The clinicoradiological details with the surgical procedure were retrieved from the archived hospital records.Results:All cases were males, of which three were in their 5th decade while one was a 14-month-old baby. Two cases were primary and the rest were recurrent.The location of tumors was extra-axial left cerebellotentorial,clivaldural-based,left cerebellar, and in the left frontoparietal region,respectively. The clinical impression was meningioma in three cases,while it was primitive neuroectodermal tumor (PNET) /atypical teratoid/rhabdoid tumor (ATRT) in one case.With the detailed histomorphology and immunohistochemistry, the final diagnosis was anaplastic hemangiopericytoma(WHO grade III) for all the cases. During our follow-up, one patient died with the disease, while the rest are doing well.Conclusion:SFT/HPC should be kept in the differential diagnosis of all dura-based hypervascular masses, especially in recurrent cases, due to its aggressiveness and high recurrence rate.

Corresponding author : Debahuti Mohapatra