Abstract
Background: The 2016 central nervous system (CNS) World Health Organisation (WHO) Update has merged the entities of meningeal solitary fibrous tumor (SFT) and hemangiopericytoma (HPC) into a single entity based on the presence of the nerve growth factor 1A (NGFI-A) binding protein 2 (NAB2) - signal transducer and activator of transcription 6 (STAT6) gene fusion in these tumors. Immunohistochemical (IHC) staining with STAT6 results in a strong nuclear positivity confirming the diagnosis. Meningeal SFT/HPCs are currently histologically graded according to a three-tiered system. Grade I (SFT phenotype) is benign, whereas grades II and III (HPC phenotype) are malignant and require radiotherapy in addition to gross total resection.
Objectives: The objectives were to review the cases diagnosed as meningeal SFT or HPC between 2010 and 2017 and classify them into SFT (grade I) or HPC (grades II and III) phenotypes; to confirm the diagnosis by performing STAT6 immunohistochemistry; and to observe and record the histological features in detail and correlate the tumor grades with their behavior. The published literature on the subject was also reviewed.
Materials and Methods: A total of 21 cases diagnosed between 2010 and 2017 as meningeal SFT or HPC were included in the study. All cases were reviewed by the authors and were categorized and graded according to histologic phenotype and mitotic count. STAT6 immunohistochemistry was performed in all the cases. The epidemiological data and histologic findings in each case were recorded in detail. The follow-up of patients was obtained.
Results: Fifteen patients were males and six were females. The mean age was 43.5 years. The mean tumor size was 6.8 cm. The tumor specimens in 20 out of 21 cases corresponded to the HPC phenotype, of which 6 were in grade II while 14 were in grade III. Thus, over 95% cases had malignant lesions. The tumor in all the 21 cases recruited for the study showed immunohistochemical positivity for SAT6, while CD34 was positive in all the 18 tumor in which it was performed. The follow-up was available in 14 of the patients. Recurrence occurred in six patients who had either a grade II or a grade III tumor and three patients died (including one patient with a grade III tumor. This patient died a month after initial resection although there was no evidence of recurrence). Radiotherapy was given to only 4 out of 14 patients in whom follow-up was available.
Conclusion: These rare tumors need to be accurately diagnosed and optimally treated (gross total resection and radiotherapy) to improve the prognosis.