Solitary fibrous tumor/hemangiopericytoma of central nervous system: a clinicopathologic analysis of 71 cases
10.3760/cma.j.issn.0529-5807.2017.07.004
- VernacularTitle: 中枢神经系统孤立性纤维性肿瘤/血管外皮瘤71例临床病理分析
- Author:
Xiaoling LI
1
;
Weiwei FU
2
;
Sheng ZHANG
;
Dayang CHEN
;
Yupeng CHEN
;
Jie WU
;
Xueyong LIU
;
Guoping LI
;
Shengming LIN
;
Sheng LUO
;
Shanshan CAI
;
Wei GUO
;
Xingfu WANG
Author Information
1. Department of Pathology, the First Affiliated Hospital of Fujian Medical University, Fuzhou 350005, China
2. Department of Pathology, the Affiliated Hospital of Qingdao University, Qingdao 266003, China
- Publication Type:Journal Article
- Keywords:
Solitary fibrous tumors;
Hemangiopericytoma;
Activating transcription factors;
Immunohistochemistry;
Prognosis
- From:
Chinese Journal of Pathology
2017;46(7):465-470
- CountryChina
- Language:Chinese
-
Abstract:
Objective:As solitary fibrous tumor (SFT) and hemangiopericytoma (HPC) share the same molecular genetics features, the 2016 WHO classification of central nervous system (CNS) tumors had created the combined term SFT/HPC and assigns three grades. This study aims to investigate the clinicopathologic characteristics, diagnosis, differential diagnosis and prognosis of CNS SFT/HPC.
Methods:Seventy-one cases of CNS SFT and HPC were retrospectively reclassified and studied. Histopathological, immunohistochemical and imaging features were analyzed. The follow-up data were analyzed.
Results:There were 37 male and 34 female patients. The median age was 48 years (range, 3-77 years). Twelve cases (17%) were WHO grade Ⅰ, 26 (37%) were WHO grade Ⅱ and 33 (46%) were WHO grade Ⅲ. Microscopically the tumor could show traditional SFT phenotype, HPC phenotype or mixed phenotype. Immunochemically, 97%(69/71) were positive for STAT6, with 96%(66/69)showing diffuse strong staining. Approximately 90% were diffusely positive for bcl-2, CD99 and vimentin. The expression rate of CD34 decreased with increasing tumor grade, and the mean expression rate was 78%. SSTR2a was variably expressed in 10% (7/71) of cases including one case showing strong cytoplasmic staining. A few cases expressed EMA, CD57 and S-100 focally. The Ki-67 index ranged from 1% to 50%. Thirty four patients were followed up for 8-130 months; 12 patients(35%)had recurrences, and two (6%) had liver metastases.
Conclusions:CNS SFT/HPC is relatively uncommon. There was significant morphological overlap or transition between different grades. STAT6 is a specific marker for the diagnosis of this tumor. Surgical resection is the preferred treatment. WHO grade Ⅱ and Ⅲ SFT/HPC show rates of local recurrence and systemic metastasis, with liver being the most common site of extracranial metastasis.
