Diagnosis And Treatment Of Solitary Fibrous Tumor/hemangiopericytoma Of Central Nervous System

Background and purpose:The diagnosis of SFT(solitary fibrous tumor)and HPC(Hemangiopericytoma)have been combined in one diagnosis named SFT/ HPC(Solitary Fibrous tumor/Hemangiopericytoma)by WHO(world health organization)in the year of 2016.SFT and HPC both were the rare tumors of CNS(Center Nerve System),however because of significant differences of the prognosis,they were regarded as two disparate lesions for a long time,in the year of 2007 CNS WHO specially highlighted their differences in pathology and immunohistochemstry.But with the rising of reported cases and development of molecular pathology,they were proved to share the same genetic inversion.For this reason,the 2016 CNS WHO has created the combined term SFT/HPC to describe such lesions.Because the new diagnosis has been created for less than 1 year and its low occurance rate,relevant literatures are scarce.In order to bring conducive information for diagnosis and treatment of SFT/HPC,we summarize the clinical data of SFT/HPC of China-Japan union hospital and analyze features of radiology,treatment,prognosis.Clinical date and Methods:15 patients with SFT/HPC of CNS hospitalized in China-Japan Union hospital neurosurgery department from the December of 2010 to the June of 2016 were analyzed and the pathological diagnosis were reevaluated according to the classification of tumors of the central nervous system proposed by WHO in 2016.Among the 15 cases,8 was female,7 was male,the age range from 33~78 years old and 47.8 in mean.13 cases occurred primarily and 2 cases recurred firstly.The disease duration was 2 weeks to 60 months(median in 8.5 months)and the reccurrance time was 8 years and 14 years respectively.3 cases was located in middle cranial fossa,3 in parietal lobe,2 in frontal para-flax,1 in anterior cranial fossa,1 in frontal lobe,1 in tentorium,1 in occipital transverse sinus,1 was intracranial multiple and the last one was in the atla.The size of lesion ranges from 1.5cm×2.0cm×2.4cm to 2.5cm×5.5cm×12.0cm.Results:All patients underwent operations,total resection was achieved in 9 cases,subtotal resection in 4 cases and partial resection was achieved in 2 cases.1 elderly patient(78 years old)died from post-operative pulmonary complications,1 patient’s relatives refused to further treatment at the 3rd day after operation,due to the hemorrhage of remnant tumor.1 patient occur operative region hemorrhage in the 1st day after operation,hematoma evacuation drainage was performed.2 patients occurred delayed post-operative hemorrhage,one was in the operative region and received conservative treatment,the other was in remote intracranial region and received hematoma evacuation drainage.After pathological reevaluation,all cases were diagnosed as SFT/HPC,WHO I was in 2,WHO II was in 12 and WHO III was in 1.Except for 1 death case and 1 case refusing treatment,3 patients lost to follow-up and totally 10 patients were in follow-up,which last from 2 months to 67 months,average in 21 months.During the follow-up period,7 patients with total resection were asymptomatic,2 of 3 patients with subtotal resection or partial resection died,the other one did not express clinical progression.Conclusion:1.SFT/HPC rarely occurs in CNS,Its clinical and radiological manifestations are lack of specificity,and has a high preoperative misdiagnosis rate.The lobulated,cystic and necrotic appearance on MRI and heterogeneous enhancement signal are relative imaging features of SFT/HPC.2.The extent of resection is still the crucial factor to the prognosis.The postoperative radiotherapy doesn’t bring authentic benefits and should be selected cautiously on the basis of the extent of resection and pathologic grade.