Prognostic Factors of Survival for Grade 3 Solitary Fibrous Tumor/Hemangiopericytoma: A Population-Based Retrospective Surveillance, Epidemiology, and End Results Database Analysis.

INTRODUCTION: Grade 3 solitary fibrous tumor, previously known as anaplastic hemangiopericytoma, is a rare and highly malignant intracranial tumor with a limited understanding of its natural history and treatment outcomes. METHODS: We conducted a retrospective analysis using the Surveillance, Epidemiology, and End Results (SEER) database spanning 2000-2019 to evaluate the clinical characteristics and treatment modalities that influence overall survival in this tumor entity. A cohort of 249 patients with intracranial grade 3 solitary fibrous tumors was identified. Univariate and multivariable Cox proportional hazard models were employed to determine significant prognostic factors for overall survival. Kaplan-Meier models were used to visualize survival curves, and a nomogram was constructed to predict survival probabilities at 6- and 12-month following diagnosis. RESULTS: Our findings indicated that patient age (<65 years), localized or regional disease burden, surgical resection, and radiation therapy were significant predictors of better overall survival. Combination therapies showed improved survival, with surgery and radiation therapy having the most significant impact. However, chemotherapy alone or in combination did not demonstrate a significant survival benefit, likely due to the limited sample size. The nomogram provided personalized prognostic predictions based on significant clinical factors. CONCLUSIONS: These data emphasize the importance of surgical resection and radiation therapy in the management of grade 3 solitary fibrous tumors, supporting the use of combination therapies to improve overall survival in this rare and aggressive intracranial neoplasm.

Does post-operative radiotherapy improve the treatment outcomes of intracranial hemangiopericytoma? A retrospective study.

BACKGROUND: Intracranial hemangiopericytoma is a rare disease and surgery is the mainstay treatment. Although postoperative adjuvant radiotherapy is often used, there are no reports comparing different radiotherapy techniques. The purpose of this study is to analyze the impact of post-operative radiotherapy and different radiotherapy technique on the results in patients with intracranial hemangiopericytoma (HPC). METHODS: We retrospectively reviewed 66 intracranial HPC patients treated between 1999 and 2019 including 29 with surgery followed by radiotherapy (11 with intensity-modulated radiotherapy (IMRT) and 18 with stereotactic radiosurgery (SRS)) and 37 with surgery alone. Chi-square test was used to compare the clinical characteristic between the groups. The Kaplan-Meier method was used to analyze overall survival (OS) and recurrence-free survival (RFS). Multivariate Cox proportional hazards models were used to examine prognostic factors of survival. We also underwent a matched-pair analysis by using the propensity score method. RESULTS: The crude local control rates were 58.6% in the surgery plus post-operative radiotherapy group (PORT) and 67.6% in the surgery alone group (p = 0.453). In the subgroup analysis of the PORT patients, local controls were 72.7% in the IMRT group and 50% in the SRS group (p = 0.228). The median OS in the PORT and surgery groups were 122 months and 98 months, respectively (p = 0.169). The median RFS was 96 months in the PORT group and 72 months in the surgery alone group (p = 0.714). Regarding radiotherapy technique, the median OS and RFS of the SRS group were not significantly different from those in the IMRT group (p = 0.256, 0.960). The median RFS were 112 and 72 months for pathology grade II and III patients, respectively (p = 0.001). Propensity score matching did not change the observed results. CONCLUSION: In this retrospective analysis, PORT did not improve the local control rates nor the survivals. The local control rates after IMRT and SRS were similar even though the IMRT technique had a much higher biological dose compared with the SRS technique.

Tumor Biology Impacts Survival in Surgically Managed Primary Hepatic Vascular Malignancies.

BACKGROUND: Hepatic angiosarcoma (AS) and hepatic epithelioid hemangioendothelioma (HEHE) are rare primary hepatic vascular malignancies (PHVM) that remain poorly understood. To guide management, we sought to identify factors and trends predicting survival after surgical intervention using a national database. MATERIALS AND METHODS: In a retrospective analysis of the National Cancer Database patients with a diagnosis of PHVM were identified. Clinicopathologic factors were extracted and compared. Overall survival (OS) was estimated and predictors of survival were identified. RESULTS: Three hundred ninty patients with AS and 216 with HEHE were identified. Only 16% of AS and 36% of HEHE patients underwent surgery. The median OS for patients who underwent surgical intervention was 97 months, with 5-year OS of 30% for AS versus 69% for HEHE patients (P< 0.001). Tumor biology strongly impacted OS, with AS histology (Hazard Ratio [HR] of 3.61 [1.55-8.42]), moderate/poor tumor differentiation (HR = 3.86 [1.03-14.46]) and tumor size (HR = 1.01 [1.00-1.01]) conferring worse prognosis. The presence of metastatic disease in the surgically managed cohort (HR = 5.22 [2.01-13.57]) and involved surgical margins (HR = 3.87 [1.59-9.42]), were independently associated with worse survival. CONCLUSIONS: In this national cohort of PHVM, tumor biology, in the form of angiosarcoma histology, tumor differentiation and tumor size, was strongly associated with worse survival after surgery. Additionally, residual tumor burden after resection, in the form of positive surgical margins or the presence of metastasis, was also negatively associated with survival. Long-term clinical outcomes remain poor for patients with the above high-risk features, emphasizing the need to develop effective forms of adjuvant systemic therapies for this group of malignancies.

A review of solitary fibrous tumor/hemangiopericytoma tumor and a comparison of risk factors for recurrence, metastases, and death among patients with spinal and intracranial tumors.

Meningeal solitary fibrous tumors (SFTs) and hemangiopericytomas (HPCs) had been combined into a single classification until 2016. Recurrence and metastases rates are still understudied, especially for spinal SFT/HPCs. Here, we describe CNS SFT/HPCs and predictors for recurrence, metastases, and death, in spinal and intracranial SFT/HPCs, separately. We collected data from studies with patient-level data available on primary SFT/HPCs from multiple online databases. Clinico-demographic data, surgical outcomes, recurrence, metastases, and death rates were abstracted. We used logistic and Cox regression models to identify predictors for recurrence, metastases, and death for spinal and intracranial SFT/HPCs. Twenty-nine studies (368 patients) were included. Higher histological grade and subtotal resection were associated with recurrence (p values < 0.05), while higher histological grade and recurrence (p values < 0.005) were associated with metastases formation. Time to recurrence (p < 0.005) and metastases (p < 0.001) formation were shorter for spinal SFT/HPCs. Death rates were higher among intracranial SFT/HPC patients (p value = 0.001). Among patients with higher histological grade, rates of metastases formation were different between intracranial and spinal SFT/HPCs. Risk of metastases was higher in the first 5 years from surgery for both intracranial and spinal SFT/HPCs. Meningeal SFT/HPCs patients have high rates of recurrence and metastasis, which occur mostly within the first 5 years after diagnosis. Spinal and intracranial SFT/HPCs show similar behavior, but spinal SFT/HPCs tend to develop metastases and recurrences in a shorter interval of time. Careful follow-up for spinal SFT/HPCs should be considered because spinal cases seem to be slightly more aggressive and require more attention.

Spinal location is prognostic of survival for solitary-fibrous tumor/hemangiopericytoma of the central nervous system.

BACKGROUND: Prior studies have highlighted infratentorial tumor location as a prognostic factor for solitary fibrous tumor (SFT) and hemangiopericytoma (HPC) of the central nervous system (CNS), and spinal location is considered a positive prognostic factor for other tumors of the CNS. While SFT/HPC of the CNS is known to frequently arise from the spinal meninges, there are no case series that report outcomes for spinally located CNS tumors, and their prognosis in relation to intracranial and other CNS-located tumors is unknown. OBJECTIVE: To investigate outcomes for patients with SFT/HPC of the spinal meninges. METHODS: The Surveillance, Epidemiology, and End-Results Program was used to identify patients with SFT/HPC within the CNS from 1993-2015. We retrospectively analyzed the relationship between tumor location (spinal vs. Brain and other CNS) and survival. RESULTS: We identified 551 cases of CNS SFT/HPC, 64 (11.6%) of which were primary tumors of the spinal meninges. Spinal tumors were more likely than brain and other CNS tumors to be SFT vs. HPC (37.5 vs. 12%, p < 0.001), benign (42.2 vs. 20.3%, p < 0.001), and less than 5 cm (53.1 vs. 35.7%, p < 0.001). The 10-year survival rates for spinal and brain/other CNS tumors were 85 and 58%, respectively. Median survival time was significantly longer for spinal tumors (median survival not reached vs. 138 months, p = 0.03, HR = 0.41 [95% CI 0.18-0.94]). On multivariable analysis, spinal tumor location was associated with improved survival over tumors located in the brain and other CNS (HR = 0.36 [95% CI 0.15-0.89], p = 0.03). CONCLUSION: Spinal tumor location is associated with improved survival in patients with SFT/HPC of the CNS. Larger institutional studies are necessary to characterize the relationship between tumor location and other relevant factors such as presentation and amenability to gross-total resection and adjuvant radiotherapy. Future studies exploring optimal management of spinally located tumors are also needed.

Solitary Fibrous Tumor/Hemangiopericytoma of Spinal Cord: A Retrospective Single-Center Study of 16 Cases.

OBJECTIVE: In this study, we retrospectively reviewed our experience in the surgical management of solitary fibrous tumor (SFT)/hemangiopericytomas (HPCs) of the spinal cord. METHODS: Sixteen patients with SFT/HPCs of the spinal cord were enrolled in this study. Data on clinical presentation, radiologic findings, histopathologic features, surgical treatment, adjuvant therapy, and prognosis were retrospectively reviewed. Kaplan-Meier curves and log-rank tests were used to identify the prognostic factors for recurrence and overall survival (OS). RESULTS: Our series included 6 men and 10 women, with a male/female ratio of 1:1.7. Magnetic resonance imaging (MRI) showed slightly hyperintense lesions on T2-weighted images for all 16 patients. All tumors showed positive immunohistochemical staining for signal transducer and activator of transcription 6. Statistical analysis of clinical data showed that age, gender, tumor location, tumor size, medullary compartment location, and Ki-67 index were not associated with recurrence and OS (P > 0.05). However, World Health Organization grade III was significantly associated with recurrence (P < 0.01). Gross total resection (GTR) and postoperative radiotherapy significantly reduced recurrence (P < 0.01 and P < 0.05), but only GTR showed remarkable benefits to improve OS (P < 0.05). CONCLUSIONS: SFT/HPCs of spinal cord are rare neoplasms with a propensity to recur. Hyperintensity on T2-weighted magnetic resonance imaging combined with positive immunohistochemical staining for signal transducer and activator of transcription 6 are important clues for classification and differentiation of these tumors. The extent of resection, World Health Organization grade, and postoperative radiotherapy might be predictive factors for recurrence. Complete tumor resection should be sought whenever possible, and adjuvant radiotherapy is recommended after surgical resection. Moreover, regular and long-term follow-up is mandatory to monitor recurrence.

Association between programmed cell death ligand-1 expression and extracranial metastasis in intracranial solitary fibrous tumor/hemangiopericytoma.

BACKGROUND: Intracranial solitary fibrous tumor/hemangiopericytoma (SFT/HPC) often shows extracranial metastasis, and treatment options are very limited. Immune-checkpoint molecules have not been studied well in SFT/HPCs, and their role in intracranial SFT/HPCs remains unclear. METHODS: We investigated the expression of programmed cell death-1 (PD-1), programmed cell death ligand-1 (PD-L1), and tumor-infiltrating lymphocytes (TIL) in 16 patients of intracranial SFT/HPC by immunohistochemistry to determine if correlation with prognosis exists. RESULTS: Median overall survival (OS) of 16 patients was 9.2 years, and median follow-up of alive patients was 9.9 years. Recurrence was observed in 13 (81.3%) patients, and extracranial metastasis were observed in 6 (37.5%). PD-L1 expression was observed in all 16 tumors, whereas PD-1 expression was observed in 2. CD3 and CD8 expressions were observed in TILs in 12 and 13 patients respectively. Although the ratio of PD-L1 positive-tumor cells was not associated with OS, progression-free survival, or metastasis-free survival (MFS), diffuse staining of PD-L1 showed a trend toward shorter time to treatment failure (TTF: time to either extracranial metastasis or death) (p = 0.072). Similarly, the intense staining of PD-L1 was associated with shorter MFS (p = 0.0084) and TTF (p = 0.033). CD3 or CD8 expression was not associated with any of the prognostic parameters. In the combined analysis of PD-L1 and CD8, diffuse PD-L1 staining coupled with no or sparse CD8 expression was significantly associated with a shorter TTF (p = 0.005) and showed a trend toward shorter MFS (p = 0.0611). CONCLUSIONS: PD-L1 is frequently expressed in intracranial SFT/HPCs, and diffuse or intense PD-L1 expression might be associated with the early occurrence of extracranial metastases.

Solitary fibrous tumours and haemangiopericytoma of the meninges. A retrospective study for outcome and prognostic factor assessment.

BACKGROUND: To report on the outcome of patients diagnosed with central nervous system haemangiopericytoma (HPC) or solitary fibrous tumours (SFT) and identify factors that may influence recurrence and survival. MATERIAL AND METHODS: Between January 1977 and December 2016, a retrospective search identified 22 HPCs/SFTs. The patients underwent a total of 40 surgical resections and 63.6% received radiotherapy. Median follow-up was 7.8 years. RESULTS: Six patients (27.3%) were re-operated for tumour recurrence. At the end of the study, 15 patients (68.2%) had no residual tumour on the last imaging. Surgical recurrence-free survival at 5 years was 77.4%, [95% CI: 60.1-99.8]. None of the investigated variables was associated with recurrence. At the end of the study, 5 patients were deceased (22.7%) and only 10 patients (45.5%) had no residual tumour on the last imaging and were alive. Overall survival at 5 years was 95%, [95% CI: 85.9-100]. None of the investigated variables was associated with overall survival. Patients who received radiotherapy demonstrated neither a reduced risk of surgical recurrence (P=0.378) nor a longer overall survival (P=0.405). CONCLUSION: SFTs/HPCs are associated with a significant risk of recurrence that may reduce the survival. Even if we could not demonstrate their benefit in this limited series, we believe that tailored maximal tumour resection on initial surgery is beneficial and that adjuvant RT is useful for tumours displaying grade II or III, even in case of complete removal.

Efficacy of adjuvant radiotherapy in the intracranial hemangiopericytoma.

We retrospectively evaluated an efficacy of adjuvant radiotherapy (RT) in the intracranial hemangiopericytoma (HPC) and analyzed prognostic factors influencing treatment outcomes. Among 49 patients diagnosed as localized intracranial HPC between 1995 and 2016, 31 patients received adjuvant RT after surgery; 26 with fractionated RT and 5 with stereotactic radiosurgery using Gamma Knife. After gross total resection (GTR) (n = 32) and subtotal resection (STR) (n = 17), histopathological grade was confirmed to be grade II (n = 9) or grade III (n = 40). The median follow-up period was 50 months (range 3-216 months). The local recurrence was defined as intracranial relapse within 15 mm and regional recurrence as beyond 15 mm from the margin of surgical bed. The 10-year overall survival (OS) and progression-free survival (PFS) were 69.9 and 34.4%, respectively. The 10-year local, regional, and distant failure-free rates were 56.6, 88.2, and 73.3%, respectively. Local tumor control was better with GTR followed by RT than GTR alone (p = 0.056), while there was no difference in OS. Local tumor control and OS after STR plus RT were equivalent to those after GTR alone. There were no differences in distant metastasis-free survival (DMFS) among GTR plus RT, GTR alone, and STR plus RT. Tumor volume > 40 cm3 was associated with poor PFS (p = 0.024). The local tumor recurrence was reduced by adjuvant RT after surgery. But OS or DMFS was not improved with adjuvant RT. PFS was better in the tumor with small volume at diagnosis.

Surgical management of spinal solitary fibrous tumor/hemangiopericytoma: a case series of 20 patients.

PURPOSE: Spinal solitary fibrous tumor/hemangiopericytoma (SFT/HPC), a rare mesenchymal tumor that arises from pericytes of Zimmerman, comprises only 0.08% of all primary bone tumors and 0.1% of primary malignant bone tumor and rarely occurs in the spine. We attempt to correlate the clinical factors and different treatment options with the recurrence rate and overall survival of SFT/HPC over time. METHODS: A retrospective study of 20 patients with spinal osseous SFT/HPCs who were surgically treated in our center between 2003 and 2015 was performed. Kaplan-Meier curves and log-rank tests were used to compare the survival probability or recurrence-free probability between groups, and P values < 0.05 were considered statistically significant. RESULTS: Three surgical management strategies, including subtotal resection, piecemeal total resection, and total en bloc spondylectomy (TES) were applied. Postoperative radiotherapy was carried out in 14 cases. The mean follow-up period was 38.3 (median 35, range 7-93) months, and 6 patients passed away with the mean follow-up time of 47.7 (median 41, range 24-77) months. Relapse was detected in 9 patients (45%) with the mean time from surgery to recurrence being 36.6 (median 28, range 12-73) months. Our results indicate that grade III is an adverse prognostic factor for both recurrence and over survival (OS) for spinal osseous SFT/HPC, while total resection, especially TES, is a positive prognostic factor. CONCLUSIONS: Spinal osseous SFT/HPC is a challenging clinical entity given its high local recurrence rate. Surgical management plays a crucial role in the whole treatment of spinal SFT/HPCs and total excision, especially TES, should be strived for whenever possible. Postoperative radiotherapy is recommended to lower the recurrent rate. This study also confirms that pathology grade III is an adverse prognostic factor for spinal osseous SFT/HPCs.

Genetic alterations of IDH1 and Vegf in brain tumors.

BACKGROUND: This study evaluates the presence of R132H mutation in isocitrate dehydrogenase (IDH1) gene and the vascular endothelial growth factor (VEGF) +936 C/T polymorphism in brain tumors. The impact of these genetic alterations on overall survival (OS) and progression free survival (PFS) was evaluated. METHODS: A cohort of 80 patients surgically treated at Hospital Clínico San Carlos, Madrid, between March 2004 and November 2012, was analyzed. Tumors were distributed in 73 primary brain tumors (gliomas, meningiomas, hemangiopericytomas and hemangioblastomas) and seven secondary tumors evolved from a low grade glioma, thus providing a mixed sample. RESULTS: IDH1R132H gene mutation was found in 12 patients (15%) and appears more frequently in secondary tumors (5 (71.4%) whereas in 7 (9.7%) primary tumors (p < .001)). The mutation is related to WHO grade II in primary tumors and a supratentorial location in secondary tumors. The OS analysis for IDH1 showed a tendency towards a better prognosis of the tumors containing the mutation (p = .059).The IDH1R132H mutation confers a better PFS (p = .025) on primary tumors. The T allele of VEFG +936 C/T polymorphism was found in 16 patients (20%). No relation was found between this polymorphism and primary or secondary tumor, neither with OS or PFS. CONCLUSIONS: IDH1R132H gene mutation is exclusive in supratentorial tumors and more frequent in secondary ones, with a greater survival trend and better PFS in patients who carry it. The T allele of VEGF +936 C/T polymorphism is more common in primary tumors, although there is no statistical relation with survival.

Long-Term Outcome and Prognostic Factors After Repeated Surgeries for Intracranial Hemangiopericytomas.

OBJECTIVE: The goals of the present study were to identify predictors of better survival and to propose appropriate management strategies for recurrent hemangiopericytomas (HPC) and anaplastic hemangiopericytomas (AHPC). METHODS: Between 2008 and 2016, 191 patients underwent surgeries for HPC and/or AHPC at our institute, and during follow-up the tumors recurred in 57 patients, including 31 males (54.4%). RESULTS: At the first recurrence, 30 patients (52.6%) underwent surgery, 25 patients (43.9%) declined surgery, and 2 patients (3.5%) received Gamma Knife treatment. The 1-year, 3-year, and 5-year actuarial rates of second progression-free survival in the HPC group were 73.3%, 46.7%, and 24.9%, respectively; the rates in the AHPC group were 66.7%, 66.7%, and 0%, respectively. The actuarial 1-year, 3-year, and 5-year overall survival rates of HPC after the first recurrence were 87.4%, 69.2%, and 39.5%, respectively; in the AHPC group, the rates were 85.2%, 45.9%, and 24.5%, respectively. Each 1-month increase in the time interval from first surgery to first recurrence (first recurrence-free survival) (hazard ratio, 0.972; 95% confidence interval, 0.952-0.993; P = 0.010) was strongly associated with better overall survival. Patients who received surgery with or without radiation at their first recurrence survived longer than patients who did not (estimated median survival time, 53.0 months vs. 35.7 months; P = 0.028). CONCLUSIONS: Treatment is imperative for the first recurrence of HPC or AHPC. More attention should be paid to patients with shorter first recurrence-free survival. Surgery is the first choice for their first recurrence and radiotherapy should be administered if there is no history of radiotherapy.

Surgical Management and Adverse Factors for Recurrence and Long-Term Survival in Patients with Hemangiopericytoma.

OBJECTIVE: Intracranial hemangiopericytoma is a rare tumor with high recurrence rate. We analyzed adverse factors for recurrence and survival of patients with hemangiopericytoma. METHODS: We retrospectively reviewed clinical data of 120 patients (mean age, 42 years; 60 male patients) with hemangiopericytoma who were surgically treated in our hospital from December 2008 to January 2016. RESULTS: Gross total resection (GTR) rate was 71.7%. Postoperative adjuvant radiotherapy (PRT) was administered to 63 patients. After median follow-up period of 46.9 months, 35 (29.1%) recurrences and 17 (14.1%) deaths were observed. Progression-free survival (PFS) at 1, 3, and 5 years was 90.8%, 78.5%, and 68.0%, and corresponding overall survival rate was 99.2%, 93.7%, and 82.4%. Higher preoperative Karnofsky performance scale scores (hazard ratio [HR] = 0.896, 95% confidence interval [CI] = 0.845-0.950, P < 0.001), convex surface location (HR = 2.151, 95% CI = 1.042-4.443, P = 0.038), and PRT (HR = 0.339, 95% CI = 0.159-0.724, P = 0.005) were independent favorable factors for PFS. For overall survival, higher preoperative Karnofsky performance scale scores (HR = 0.914, 95% CI = 0.854-0.978, P = 0.009), GTR (HR = 0.291, 95% CI = 0.109-0.777, P = 0.014), and PRT (HR = 0.210, 95% CI = 0.060-0.734, P = 0.015) were independent favorable factors. In patients undergoing non-GTR, PRT significantly improved PFS (HR = 0.252, 95% CI = 0.070-0.906, P = 0.035). CONCLUSIONS: This study revealed risk factors for PFS and overall survival to predict outcomes and determine treatments. GTR was attempted as frequently as possible, and PRT was recommended for patients with non-GTR or recurrence to improve tumor control.

Characteristics and prognosis of glomangiopericytomas: A systematic review.

BACKGROUND: Glomangiopericytoma belongs to the category of borderline/low-malignant-potential tumors of the sinonasal tract, but no studies about prognosis have been reported. METHODS: To define the characteristics of glomangiopericytoma and to identify its prognostic factors, a systematic review was performed. A total of 337 cases of glomangiopericytomas were analyzed. RESULTS: Patients with glomangiopericytoma demonstrating resection margin involvement and receiving radiotherapy/chemotherapy had a significantly worse disease-free survival time (P = .014 and .006, respectively). Patients with glomangiopericytoma had a tendency toward longer overall survival when they were diagnosed at a younger age (≤60 years; P = .001), did not demonstrate marginal involvement (P = .032), recurrence/metastasis (P = .002), or radiotherapy/chemotherapy (P = .010), and had a right-sided tumor (P < .001), actin-immunopositivity (P < .001), and CD34-/BCL2-immunonegativities (P = .002 and .019, respectively). By multivariate analysis, actin (P < .001) and CD34 (P = .002) immunostaining were significantly related to the overall survival of patients with glomangiopericytoma. CONCLUSION: Actin and CD34 immunostaining could be used as independent prognostic indicators of glomangiopericytomas.

Analysis of Prognostic Factors, Survival Rates, and Treatment in Anaplastic Hemangiopericytoma.

OBJECTIVE: In this study, we aimed to identify prognostic factors in anaplastic hemangiopericytoma (AHPC) and clinical behaviors that differentiate primary and secondary AHPC. METHODS: The clinical data associated with 52 cases of AHPC that were surgically treated between 2008 and 2015 were reviewed. The patients were classified into the following 2 groups: primary AHPC (AHPC diagnosed at the first surgery) and secondary AHPC (malignant transformation from a lower-grade tumor). RESULTS: The study included 27 men and 25 women. The participants had a mean age of 43 years old. The 3- and 5-year progression-free survival (PFS) rates were 63.4% and 53.5%, respectively, and the corresponding overall survival rates were 78.7% and 70.9%, respectively. At the final follow-up, there were 22 (42.3%) recurrences, 4 (7.7%) extracranial metastases, and 11 (21.2%) deaths. On the basis of multivariate analysis, primary AHPC (hazard ratio [HR] = 0.293, 95% CI 0.122-0.705) and postoperative radiotherapy (PRT) (HR = 0.372, 95% confidence interval [CI] 0.148-0.932; P = 0.035) were significantly associated with increased PFS, and gross total resection (HR = 3.512, 95% CI 1.060-11.634; P = 0.040) and PRT (HR = 0.165, 95% CI 0.035-0.771; P = 0.022) were independent favorable factors for overall survival. CONCLUSION: Gross total resection and PRT following surgery are recommended in AHPC. Identifying clinical behaviors that differentiate primary and secondary AHPC improved our understanding of this type of tumor and guided treatment strategies.

Invasiveness is associated with metastasis and decreased survival in hemangiopericytoma of the central nervous system.

Meningeal hemangiopericytoma (m-HPC) is a rare tumor of the central nervous system (CNS), which is distinguished clinically from meningioma by its tendency to recur and metastasize. The histological classification and grading scheme for m-HPC is still evolving and few studies have identified tumor features that are associated with metastasis. All patients at our institution with m-HPC were assessed for patient, tumor, and treatment characteristics associated with survival, recurrence, and metastasis. New findings were validated using the SEER database. Twenty-seven patients were identified in our institutional records with m-HPC with a median follow-up time of 85 months. Invasiveness was the strongest predictor of decreased overall survival (OS) and decreased metastasis-free survival (MFS) (p = 0.004 and 0.001). On subgroup analysis, bone invasion trended towards decreased OS (p = 0.056). Bone invasion and soft tissue invasion were significantly associated with decreased MFS (p = 0.001 and 0.012). An additional 315 patients with m-HPC were identified in the SEER database that had information on tumor invasion and 263 with information on distant metastasis. Invasion was significantly associated with decreased survival (HR = 5.769, p = 0.007) and metastasis (OR 134, p = 0.000) in the SEER data. In this study, the authors identified a previously unreported tumor characteristic, invasiveness, as the strongest factor associated with decreased survival and metastasis. The association of invasion with decreased survival and metastasis was confirmed in a separate, larger, publicly available database. Invasion may be a useful parameter in the histological grading and clinical management of hemangiopericytoma of the CNS.

Analyses of prognosis-related factors of intracranial solitary fibrous tumors and hemangiopericytomas help understand the relationship between the two sorts of tumors.

Increasing evidence has suggested a close relationship between solitary fibrous tumors (SFTs) and hemangiopericytomas (HPCs) in the central nervous system (CNS). However, CNS SFTs differentiate from HPCs in their clinical behavior and patient prognoses. Analyses of prognosis-related factors can help clarify the relationship between SFT and HPC. The intracranial SFT and HPC cases treated in our departments from January 2002 to December 2012 were retrospectively reviewed. The SFT and HPC cases were also combined into an SFT/HPC group. The factors associated with patient progression-free survival (PFS) and overall survival (OS) were statistically analyzed using uni- and multivariate analyses. Fifty-eight intracranial SFT/HPC patients including 38 SFT patients and 20 HPC patients were treated during this period. The "Marseille grading" evaluated upon the histological aggressive phenotypes was applied in this study. The grading reflected a malignant progression ranging from "conventional" SFTs (grade I) to WHO III HPCs (grade III), and grade was negatively correlated with the PFS and OS of the SFT, HPC and SFT/HPC patients (P < 0.05).The multivariate analyses revealed that gross total resection (GTR) was significantly positively correlated with PFS and OS in the SFT, HPC and SFT/HPC patients and that radiotherapy was significantly positively correlated with PFS in the HPC and SFT/HPC patients (P < 0.05). In conclusion, the intracranial SFTs and HPCs share common prognostic factors including extent of surgery and pathology, moreover, the histological grading of the aggressive phenotypes supports the unifying of the CNS SFT and HPC into one tumor entity of SFT/HPC.

Gamma knife radiosurgery for residual or recurrent intracranial hemangiopericytomas.

Residual or recurrent hemangiopericytoma (HPC) has been treated with radiosurgery; however, its long-term outcome is not well known. This study is to investigate the long-term outcome of gamma knife radiosurgery (GKS) for residual or recurrent HPCs. We conducted a retrospective analysis of 18 patients who underwent gamma knife radiosurgery for residual or recurrent HPCs. Of the 18 patients, 10 patients had high-grade HPCs (27 tumors) and 8 had low-grade HPCs (13 tumors). Median overall survival (OS) after the first GKS was 134.7months and actuarial survival rate at 1, 5, and 10years was 85.6%, 85.6%, and 37.4%, respectively. At the last follow-up, local tumor control was achieved in 32 (80.0%) of the 40 GKS-treated tumors. New lesions developed out of initial GKS target in 8 patients (44.4%). They were also treated with additional GKS. The actuarial local control rate of 40 tumors at 1-, 3-, and 5-years was 89.3%, 60.9%, and 37.5%, respectively. The median local recurrence-free interval of 40 tumors after initial GKS for each lesion was 86.1months for low-grade and 40.5months for high-grade tumors (p=0.010). Extracranial metastases developed in 7 (38.9%) patients with high-grade pathology and became a cause of death in 3 patients. Intracranial tumor control can be achieved over the long term, though additional GKS is frequently necessary. Extracranial metastasis is common in HPC of high-grade pathology. Close surveillance and aggressive treatment is recommended not only for intracranial tumor but also for possible extracranial metastases.

hTERT promoter methylation in meningiomas and central nervous hemangiopericytomas.

In meningiomas, prognostic impact of mutations in the human telomerase reverse transcriptase (hTERT) promoter region was recently shown, while studies of promoter methylation and analyses of hemangiopericytomas are lacking. hTERT promoter methylation was analyzed in 78 meningioma and 38 meningeal hemangiopericytoma samples by methylation-specific polymerase chain reaction (MS-PCR) and compared with histopathological and clinical variables and with immunohistochemical hTERT expression. Promoter methylation was found in 62 samples (53 %) and tended to be higher in meningiomas (N = 19/41, 46 %) than in hemangiopericytomas (N = 8/33, 24 %, p = .057). In meningiomas, methylation was 16, 60 and 77 % in grade I, II and III tumors (p < .001) and higher in recurrent (N = 33/37, 89 %) than in primary diagnosed (N = 19/41, 46 %) tumors (OR 5.14, 95 % CI 1.34-19.71, p = .017). Univariate analyses showed shorter mean progression free and overall survival in methylated than in unmethylated individuals (26 vs. 100 months; p = .045 and 110 vs. 113 months; p = .025, respectively). Moreover, hTERT expression was found in 70 % (N = 53) and was more frequent in methylated than in unmethylated samples (78 vs. 52 %, OR 3.36, 95 % CI 1.20-9.40, p = .021). In hemangiopericytomas, methylation was similar in grade II (24 %) and III (25 %, p > .05) and in primary (24 %) and recurrent tumors (40 %, p > .05). hTERT expression was similar as compared to meningiomas (74 %, N = 28, p > .05) but was independent of promoter methylation (OR 4.26, 95 % CI 0.47-39.0, p = .199). In meningeal tumors, hTERT promoter methylation is more common than mutations and in meningiomas but not in hemangiopericytomas positively correlated with WHO grade and hTERT expression.