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Background: Solitary fibrous tumors (SFT) of the central nervous system are rare and treatment options are not well established. The aim of this study was to evaluate the clinical outcomes of radiotherapy (RT) and re-radiotherapy (re-RT) for de novo intracranial SFT and recurrent intracranial SFT., Methods: This retrospective study analyzed efficacy and toxicity of different RT modalities in patients who received radiotherapy (RT) for intracranial SFT at Heidelberg University Hospital between 2000 and 2020 following initial surgery after de novo diagnosis ("primary group"). We further analyzed the patients of this cohort who suffered from tumor recurrence and received re-RT at our institution ("re-irradiation (re-RT) group"). Median follow-up period was 54.0 months (0-282) in the primary group and 20.5 months (0-72) in the re-RT group. RT modalities included 3D-conformal RT (3D-CRT), intensity-modulated RT (IMRT), stereotactic radiosurgery (SRS), proton RT, and carbon-ion RT (C12-RT). Response rates were analyzed according to RECIST 1.1 criteria., Results: While the primary group consisted of 34 patients (f: 16; m:18), the re-RT group included 12 patients (f: 9; m: 3). Overall response rate (ORR) for the primary group was 38.3% (N = 11), with 32.4% (N = 11) complete remissions (CR) and 5.9% (N = 2) partial remissions (PR). Stable disease (SD) was confirmed in 5.9% (N = 2), while 41.2% (N = 14) experienced progressive disease (PD). 14% (N = 5) were lost to follow up. The re-RT group had 25.0% CR and 17.0% PR with 58.0% PD. The 1-, 3-, and 5-year progression-free survival rates were 100%, 96%, and 86%, respectively, in the primary group, and 81%, 14%, and 14%, respectively, in the re-RT group. Particle irradiation (N = 11) was associated with a lower likelihood of developing a recurrence in the primary setting than photon therapy (N = 18) (OR = 0.038; p = 0.002), as well as doses ≥ 60.0 Gy (N = 15) versus < 60.0 Gy (N = 14) (OR = 0.145; p = 0.027). Risk for tumor recurrence was higher for women than for men (OR = 8.07; p = 0.014) with men having a median PFS of 136.3 months, compared to women with 66.2 months., Conclusion: The data suggests RT as an effective treatment option for intracranial SFT, with high LPFS and PFS rates. Radiation doses ≥ 60 Gy could be associated with lower tumor recurrence. Particle therapy may be associated with a lower risk of recurrence in the primary setting, likely due to the feasibility of higher RT-dose application., (© 2024. The Author(s).)

Background: Radiation exposure is a known risk factor for meningioma but there are no data regarding hemangiopericytoma and radiation exposure., Case Description: We report a 29-year-old pineoblastoma patient diagnosed with a hemangiopericytoma at a different location, after a successful surgical excision and adjuvant radiotherapy for the original tumor 4-year prior., Conclusion: Hemangiopericytoma emergence can be seen after radiotherapy.

Background: Solitary fibrous tumor/hemangiopericytoma (SFT/HPCT) is a rare tumor characterized by high recurrence rate and metastatic potential, even after surgical resection. We report on the clinical outcomes and risk factors for metastasis and progression-free survival (PFS) of patients diagnosed with SFT/HPCT., Methods: We retrospectively identified patients with intracranial or spinal SFT/HPCT who underwent surgical resection and/or radiation therapy at our institution between 1995 and 2021. Baseline demographics, tumor characteristics, and outcome data were collected, and factors associated with PFS and metastasis were analyzed., Results: Thirty-four subjects (mean age, 46.4 years; 44% female) with a histopathologically proven diagnosis of SFT/HPCT were included; the median follow-up was 89.7 months. Twenty-two tumors were supratentorial (67%), 6 (18%) were infratentorial, and 5 (15%) were spinal. Eleven patients had documented occurrence of metastasis (32%). Detailed preoperative and postoperative data were available for 25 patients (74%) who received treatment at our institution after their initial diagnosis. Of those, 20 (80%) underwent gross total resection (GTR), and 12 (48%) received either adjuvant or salvage radiotherapy. Univariate analyses revealed that males had a shorter mean PFS compared with females (25 months vs. 78 months; P = 0.01), and that patients who underwent GTR had a longer mean PFS compared with those who underwent subtotal resection (54 months vs. 23 months; P = 0.02). Male sex was the sole risk factor for metastasis (odds ratio, 6.75; 95% confidence interval, 1.19-38.02)., Conclusions: Our data demonstrate a strong association between male sex and the outcomes of shorter PFS and higher risk for metastases. Further research is warranted to understand the clinical characteristics and outcomes of this rare tumor., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Purpose: This study aimed to evaluate the role of postoperative radiotherapy (PORT) in intracranial solitary fibrous tumor/hemangiopericytoma (SFT/HPC)., Materials and Methods: A total of 133 patients with histologically confirmed HPC were included from eight institutions. Gross total resection (GTR) and subtotal resection (STR) were performed in 86 and 47 patients, respectively. PORT was performed in 85 patients (64%). The prognostic effects of sex, age, performance, World Health Organization (WHO) grade, location, size, Ki-67, surgical extent, and PORT on local control (LC), distant metastasis-free survival (DMFS), progression-free survival (PFS), and overall survival (OS) were estimated by univariate and multivariate analyses., Results: The 10-year PFS, and OS rates were 45%, and 71%, respectively. The multivariate analysis suggested that PORT significantly improved LC (p < 0.001) and PFS (p < 0.001). The PFS benefit of PORT was maintained in the subgroup of GTR (p=0.001), WHO grade II (p=0.001), or STR (p < 0.001). In the favorable subgroup of GTR and WHO grade II, PORT was also significantly related to better PFS (p=0.028). WHO grade III was significantly associated with poor DMFS (p=0.029). In the PORT subgroup, the 0-0.5 cm margin of the target volume showed an inferior LC to a large margin with 1.0-2.0 cm (p=0.021). Time-dependent Cox proportion analysis showed that distant failures were significantly associated with poor OS (p=0.003)., Conclusion: This multicenter study supports the role of PORT in disease control of intracranial SFT/HPC, irrespective of the surgical extent and grade. For LC, PORT should enclose the tumor bed with sufficient margin.

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., (© 2021. The Author(s).)

Introduction: A meningeal solitary fibrous tumor (SFT), also called hemangiopericytoma, is a rare mesenchymal malignancy. Due to anatomic constrains, even after macroscopic complete surgery with curative intent, the local relapse risk is still relatively high, thus increasing the risk of dedifferentiation and metastatic spread. This study aims to better define the role of postoperative radiotherapy (RT) in meningeal SFTs., Patients and Methods: A retrospective study was performed across seven sarcoma centers. Clinical information was retrieved from all adult patients with meningeal primary localized SFT treated between 1990 and 2018 with surgery alone (S) compared to those that also received postoperative RT (S + RT). Differences in treatment characteristics between subgroups were tested using independent samples t -test for continuous variables and chi-square tests for proportions. Local control (LC) and overall survival (OS) rates were calculated as time from start of treatment until progression or death from any cause. LC and OS in groups receiving S or S + RT were compared using Kaplan-Meier survival curves., Results: Among a total of 48 patients, 7 (15%) underwent S and 41 (85%) underwent S + RT. Median FU was 65 months. LC was significantly associated with treatment. LC after S at 60 months was 60% versus 90% after S + RT ( p  = 0.052). Furthermore, R1 resection status was significantly associated with worse LC (HR 4.08, p  = 0.038). OS was predominantly associated with the mitotic count (HR 3.10, p  = 0.011)., Conclusion: This retrospective study, investigating postoperative RT in primary localized meningeal SFT patients, suggests that combining RT to surgery in the management of this patient population may reduce the risk for local failures.

Background and Purpose: The role of radiotherapy (RT) in the treatment of hemangiopericytoma/solitary fibrous tumor (HPC/SFT) is still under debate. We aimed at investigating whether radiotherapy can improve the results in patients operated for extracranial HPC/SFT., Materials and Methods: Data from patients with HPC/SFT, treated from 1982 to 2012, were retrospectively reviewed within the Rare Cancer Network framework. Actuarial local control (LC), disease-free survival (DFS), metastasis-free survival (MFS) and overall survival (OS) were calculated with Kaplan-Meyer method. Patient and tumor parameters were analyzed by univariate and multivariate analysis., Results: Of 114 HPC/SFT, 58 (50.9%) occurred in the extremities/superficial trunk and 56 (49.1%) in intra-thoracic/retroperitoneum. Seventy-eight patients (68.4%) underwent surgery only (Sx), and 36 (31.6%) Sx and RT (Sx + RT). Median RT dose was 60 Gy (range 45-68.4 Gy) in 1.6-2.2 Gy fractions. In the extremities/superficial trunk group of patients, actuarial 5-year LC rates were 50.4% after Sx and 91.6% after Sx + RT (p < 0.0001) for LC, and 50.4% after Sx and 83.1% after Sx + RT (p = 0.008) for DFS. In the intra-thoracic/retroperitoneum group of patients, actuarial 5-year rates were 89.3% after Sx and 77.8% after Sx + RT (p = 0.99) for LC, and 73.8% after Sx and 77.8% after Sx + RT (p = 0.93) for DFS. At multivariate analysis, the addition of RT resulted in better LC and DFS in the whole series. The advantage was confirmed for LC in the group of patients affected by extremity/superficial trunk tumors., Conclusion: Addition of RT to Sx could improve the prognosis, in terms of LC and DFS, essentially in patients with extremities/superficial trunk tumor locations., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Hemangiopericytoma and Solitary Fibrous Tumor are tumors with low incidence. They have a tendency to recur locally and to metastasize. The WHO integrated both tumors into a new entity but one of the pending issues is to demonstrate the effectiveness of surgery plus complementary radiotherapy (RT) and standardize the use of it. We reviewed the data from 10 years. We assessed pathologic and radiologic characteristics. The operation records were evaluated to determine the features and extent of tumor resection. We compared the outcomes in patients using or not RT. The mean follow-up was 74.8 months, with a range of 12 and 210 months. The population included 3 males (30%) and 7 females (70%). The most common location was brain convexity (30%), the remaining were cervical and lumbar spine, sacrum, intraventricular, torcular, sphenoid ridge and intraorbital. Postoperative external beam radiotherapy was delivered in 7 patients (70%), the criteria were a partial resection or WHO II and III histological grades. 2 patients developed local recurrences at 12 and 19 months after initial surgery. 1 patient underwent 2 surgeries, and the other, 4 surgeries. The mean recurrence free survival rate was 15.5 months. Distant metastases were found in 4 patients. 3 of the 10 patients died. Five-year overall survival rate was 66% and mean overall survival was 76 months. A safe and complete resection in the first surgery is the most important prognostic factor. Complementary RT can be helpful, even in cases of complete resection in WHO low-grade., (Copyright © 2019 Sociedad Española de Neurocirugía. Publicado por Elsevier España, S.L.U. All rights reserved.)

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., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Hemangiopericytomas (HPCs) are rare vascular tumors that resemble meningiomas on imaging and have a high rate of local recurrence and metastases. There remains a paucity of data to guide management decisions of intraspinal dissemination of HPCs in the literature, and none specifically related to anaplastic HPCs., Case Description: We report a case of a 34-year-old woman with locally and distantly recurrent anaplastic HPC (World Health Organization grade III). She initially presented with tinnitus in her right ear. A well-circumscribed, contrast-enhancing lesion was identified in the right cerebellopontine angle. Treatment consisted of subtotal resection and postoperative radiation therapy (RT) to a dose of 60 Gy in 30 fractions. After a 3-year disease-free interval, 7 lesions recurred intra- and extracranially. The extracranial lesions were drop metastases of the original HPC through the cerebrospinal fluid into the spinal canal. Of note, fluorodeoxyglucose positron emission tomography (PET)/computed tomography scan was not sensitive enough to detect these new lesions. The intracranial recurrence was on the edge of the prior radiotherapy field, representing a marginal failure having received less than 50 Gy. The intracranial recurrences were treated with salvage gamma knife stereotactic radiosurgery (SRS) with local control. She underwent intradural extramedullary hemilaminectomy of a thoracic spine metastasis followed by fractionated proton beam therapy (PBT) with a boost to unresected lesions. Within a few months of PBT, she became pregnant. Pregnancy did not affect recurrence or ameliorate tumor growth., Conclusions: This case report discusses the role genetics, adjuvant RT, SRS, magnetic resonance imaging, and PET scan played in this unique clinical scenario of anaplastic HPC., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Leksell stereotactic radiosurgery has proven to be effective for less common tumors encountered in the brain, including hemangiomas of the orbit or cavernous sinus, recurrent hemangiopericytomas, and both sporadic hemangioblastomas as well as those encountered in the context of von Hippel-Lindau (VHL) disease. While all three tumors are responsive to single-session radiosurgery, hemangiomas and hemangiopericytomas are the most likely to demonstrate tumor regression. Hemangiopericytomas that recur after initial resection can be lower grade or anaplastic and have both higher local as well as distant recurrence risks. Sporadic hemangioblastomas undergo Leksell radiosurgery at the time of recurrence after initial surgery. In the context of VHL, growing or recurrent tumors are treated with tumor control rates exceeding 90%. Tumor control improves with higher dose delivery, typically >15 Gy at the margin. Dose-limiting structures may include the optic apparatus for hemangiomas and brain stem locations for hemangioblastomas., (© 2019 S. Karger AG, Basel.)

Hemangiopericytomas are rare mesenchymal tumors with propensity to recur locally and metastasize. We report the unique case of a fifty-five-year-old male with recurrence of a previously resected craniocervical hemangiopericytoma presenting with obstructive hydrocephalus secondary to new metastatic cerebellar deposits. Emergent surgical resection of the cerebellar hemangiopericytomas was performed prior to adjuvant radiotherapy. Hemangiopericytomas are rare but important differentials for craniocervical junction lesions. Gross total resection remains the cornerstone of management with post-operative radiotherapy and chemotherapy as potential adjuncts. Tumors located in deep regions pose complex management challenges as safe maximal excision may be limited by proximal eloquent structures., (Crown Copyright © 2018. Published by Elsevier Ltd. All rights reserved.)

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 cm 3 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.

Objective: Several small (<100 patients) studies have been suggestive of a survival benefit associated with postoperative radiation for intracranial hemangiopericytoma. Given the rarity of this disease, we sought to examine the patterns of care and outcomes of this tumor in the National Cancer Database (NCDB)., Patients and Methods: We accessed the NCDB to identify patients with intracranial hemangiopericytoma between 2004-2012. Patients were categorized by surgical status, postoperative radiation usage, and location (meningeal or brain parenchyma). Multivariable Cox regression was used to identify covariables associated with differences in survival., Results: There were 588 patients included in this study, of which 323 (54.9%) received postoperative radiation. The median age at diagnosis was 51 years and the median follow up was 55.1 months. There were no differences in overall survival between the two groups. The 5-year overall survival was 77.1% for those receiving postoperative radiation and 83.8% for those who did not (p = .14). On subgroup analysis by tumor location there remained no significant differences between groups. For those with tumors in the brain parenchyma, the 5-year overall survival was 77.6% for postoperative radiation compared to 85.8% for no postoperative radiation (p = .08). For those with tumors identified as being meningeal in origin, the 5-year overall survival was 75.7% for those who received postoperative radiation compared to 81.6% for those who did not receive postoperative radiation (p = .86). On multivariable analysis, postoperative radiation was not associated with any differences on survival (HR 1.35. 95% CI 0.90-2.02, p = .15)., Conclusions: More than 50% of patients with hemangiopericytoma received postoperative radiation but there was no survival benefit associated with this treatment. This is a hypothesis-generating study and further studies are necessary to better determine how best to treat this rare disease., (Copyright © 2018 Elsevier B.V. All rights reserved.)

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., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

Intracranial hemangiopericytomas (HPC) are chemotherapy- and radiotherapy (RT)-resistant. Here, we report on a novel stereotactic radiosurgery (SRS) technique-"Cor Occidere" (Latin), as a potential strategy of overcoming radioresistance of HPC. A 36-year old female presented to our clinic for consideration of a 3rd-course of RT for her recurrent cavernous sinus HPC, following previous cranial RT at 13 and 5 years prior, and a failed 9 months trial of bevacizumab/temozolomide. The tumor-adjacent brain stem and carotid artery risked substantial damage given the cumulative RT doses to these organs. We therefore designed an SRS plan targeting only the tumor core with 16 Gy single-fraction. Despite underdosing the tumor margin, we achieved stable disease over 25 months, contrasting her responses to systemic therapies. Achieving tumor control despite a suboptimal treatment that utilized high dose ablation of the tumor core suggests novel biological mechanisms to overcome radioresistance of HPC.

Haemangiopericytomas (HPCs) in the spinal canal are extremely rare and have only been infrequently reported. Severe and sustained spinal cord compression resulting from these intraspinal tumours may cause potentially irreversible neurological impairment. We described a rare case of primary intradural extramedullary HPC recovered from serious compression of thoracic spinal cord in a 65-year-old man suffered from a rapidly progressive disease with complete paralysis of the lower limbs. Magnetic resonance imaging (MRI) and spinal angiography exhibited an unusual hypervascular mass, which was confirmed as HPC by pathological examination, in the dorsal aspects of T7 to T8 spine. Total surgical resection was performed followed by adjuvant radiation. Moreover, this patient had been undergoing specific training of lower limbs from the onset of paralysis and continuous rehabilitation was conducted after discharge. Increased strength of his lower limb muscles was shown in the period of follow-up. Remarkably, four years after surgery, this patient was able to walk with sticks. No signs of local recurrence or metastasis occurred. Conclusively, surgical removal is still the preferred treatment for HPC, and adjuvant radiotherapy and/or chemotherapy may help control tumour recurrence or metastasis. Additionally, it is possible that rehabilitation training potentially promotes neurological function recovery to some extent.

Purpose/objectives: Hemangiopericytomas are rare central nervous system (CNS) tumors. We sought to investigate existing clinical management strategies and overall survival (OS) among patients with hemangiopericytomas of the CNS., Methods/materials: All patients diagnosed with CNS hemangiopericytoma from 2004 to 2014 in the National Cancer Database were included. Clinical and treatment-related characteristics were analyzed for an association with OS following diagnosis using univariable and multivariable analyses., Results: Nine-hundred and eighty-one patients were included (0.22% of all CNS tumors). At diagnosis, 22 patients had spinal tumors (2%), 21 patients had multifocal tumors (2%) 28 had disseminated disease (3%), and the remainder were unifocal intracranial tumors. Patients either underwent surgical resection and radiation (48%), surgery alone (37%), radiation alone (6%), or biopsy alone (9%). Of patients with known extent of resection, 53% underwent gross total resection, and, of patients with known radiation modality, 15% received stereotactic radiosurgery. Among the total cohort, 3 and 10year OS was 87% and 59%, respectively. On multivariable analysis, factors associated with inferior OS included age (HR=1.05, p<0.001), WHO grade (p<0.001), multifocal disease (HR=2.59, p=0.04), disseminated disease (HR=2.67, p=0.01), and chemotherapy (HR=2.66, p=0.01). Patients receiving surgery alone or surgery and radiation demonstrated improved OS compared to biopsy alone (HR=0.45, p=0.01 and HR=0.47, p=0.02, respectively). However radiation utilization did not impact OS (p=0.691)., Conclusions: The present data provide large-scale prognostic information from a contemporary cohort of patients with hemangiopericytoma and support an initial attempt at surgical extirpation. The benefits of ionizing radiation are likely limited to improved local control and neurologic function., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

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., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: Central nervous system (CNS) hemangiopericytomas are rare mesenchymal tumors of the brain. In the absence of randomized clinical trials or large studies, the only information we have about the natural history and the management is from isolated clinical case series. They have suggested that surgery is beneficial, with conflicting results on the role of complete resection and adjuvant radiation. We have conducted a systematic review of clinical case series of CNS hemangiopericytoma analyzing the biology of the tumor and the best follow-up and management strategy., Methods: Fifteen pertinent clinical case series on newly diagnosed CNS hemangiopericytoma were selected by a review of literature. A total of 523 patients were analyzed for age, sex, mode of recurrence and metastases, and survival after complete/incomplete resection with or without radiation., Results: The mean age was found to be 44.17 (±3.59) years. The incidence was higher in male individuals younger than 45 years and in older female individuals. Complete resection and adjuvant radiation significantly improved survival in comparison with incomplete resection and no radiation, respectively (P<0.0001). Furthermore, a significant trend of the tumor to recur locally compared with extraneural and neural axis metastases was noted (P<0.0001). The mean time for distant metastases was seen to be 91.33 (±12.66) months., Conclusions: Complete resection followed by adjuvant radiation improves survival. Extraneural metastases, especially to lung, bone, and liver, are not uncommon and can occur late in the disease course for which continued follow-up is required. There is also a need to establish a systemic treatment regimen to control the distant metastases.

OBJECTIVE Hemangiopericytomas (HPCs) are rare tumors widely recognized for their aggressive clinical behavior, high recurrence rates, and distant and extracranial metastases even after a gross-total resection. The authors report a large multicenter study, through the International Gamma Knife Research Foundation (IGKRF), reviewing management and outcome following stereotactic radiosurgery (SRS) for recurrent or newly discovered HPCs. METHODS Eight centers participating in the IGKRF participated in this study. A total of 90 patients harboring 133 tumors were identified. Patients were included if they had a histologically diagnosed HPC managed with SRS during the period 1988-2014 and had a minimum of 6 months' clinical and radiological follow-up. A de-identified database was created. The patients' median age was 48.5 years (range 13-80 years). Prior treatments included embolization (n = 8), chemotherapy (n = 2), and fractionated radiotherapy (n = 34). The median tumor volume at the time of SRS was 4.9 cm 3 (range 0.2-42.4 cm 3 ). WHO Grade II (typical) HPCs formed 78.9% of the cohort (n = 71). The median margin and maximum doses delivered were 15 Gy (range 2.8-24 Gy) and 32 Gy (range 8-51 Gy), respectively. The median clinical and radiographic follow-up periods were 59 months (range 6-190 months) and 59 months (range 6-183 months), respectively. Prognostic variables associated with local tumor control and post-SRS survival were evaluated using Cox univariate and multivariate analysis. Actuarial survival after SRS was analyzed using the Kaplan-Meier method. RESULTS Imaging studies performed at last follow-up demonstrated local tumor control in 55% of tumors and 62.2% of patients. New remote intracranial tumors were found in 27.8% of patients, and 24.4% of patients developed extracranial metastases. Adverse radiation effects were noted in 6.7% of patients. During the study period, 32.2% of the patients (n = 29) died. The actuarial overall survival was 91.5%, 82.1%, 73.9%, 56.7%, and 53.7% at 2, 4, 6, 8, and 10 years, respectively, after initial SRS. Local progression-free survival (PFS) was 81.7%, 66.3%, 54.5%, 37.2%, and 25.5% at 2, 4, 6, 8, and 10 years, respectively, after initial SRS. In our cohort, 32 patients underwent 48 repeat SRS procedures for 76 lesions. Review of these 76 treated tumors showed that 17 presented as an in-field recurrence and 59 were defined as an out-of-field recurrence. Margin dose greater than 16 Gy (p = 0.037) and tumor grade (p = 0.006) were shown to influence PFS. The development of extracranial metastases was shown to influence overall survival (p = 0.029) in terms of PFS; repeat (multiple) SRS showed additional benefit. CONCLUSIONS SRS provides a reasonable rate of local tumor control and a low risk of adverse effects. It also leads to neurological stability or improvement in the majority of patients. Long-term close clinical and imaging follow-up is necessary due to the high probability of local recurrence and distant metastases. Repeat SRS is often effective for treating new or recurrent HPCs.

Objective: Hemangiopericytoma (HPC) is a rare mesenchymal tumor that tends to affect the central nervous system and is associated with distant metastasis and a high recurrence rate. The purpose of this study was to analyze the prognostic factors in patients with primary HPC who received surgical treatment., Methods: This retrospective study reviewed all adult patients with primary HPC of the central nervous system treated from 2001 to 2009 at our institution. Clinical information, adjuvant radiation, and expression levels of Ki-67 and p53 were correlated with patient outcomes., Results: The final analysis included 103 patients. The mean follow-up period was 75.9 months ± 36.5 (range, 1-165 months). There was a significant difference in progression-free survival (PFS) (P < 0.001) and overall survival (P = 0.014) between patients who underwent gross total resection versus subtotal resection. Expression of p53 was found in 48.5% of patients and showed utility as an independent unfavorable prognostic factor for PFS (P = 0.006). Multivariate analysis revealed that only extent of tumor resection (P = 0.004) and p53 expression (P = 0.024) were independent prognostic factors for PFS. Adjuvant radiation was found to extend PFS only in the p53-negative expression group (P = 0.044)., Conclusions: Gross total resection significantly improves the outcome of patients with primary HPCs, whereas adjuvant radiation contributes significantly to PFS only in patients with negative p53 expression and in patients with incomplete resections. Extent of resection and p53 expression may serve as prognostic markers for the outcome of patients with primary HPC., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Haemangiopericytomas (HPCs) are rare tumours characterised by aggressive behaviour with tendency to local recurrence and to metastasise. WHO grade II and grade III tumours show different progression-free survival and overall survival rates. Gross total tumour resection is still considered the treatment of choice. Adjuvant radiation therapies represent an option in the treatment strategy regardless the extent of resection. Based on this consideration, Gamma Knife radiosurgery has been introduced either as a primary treatment or as an adjuvant treatment for residual or recurrent tumours., Method: A systematic search was performed on PubMed, Web of Science and Google Scholar for clinical series reporting Gamma Knife radiosurgery, Cyberknife and Linear Accelerator (LINAC) for the management of intracranial HPCs., Results: Fourteen studies focusing on the effects of Gamma Knife radiosurgery for intracranial HPCs were included. Four studies reported data on Cyberknife radiosurgery and LINAC. A total of 208 patients harbouring 366 tumours have been reported. Patient's features, radiosurgical treatment characteristics and follow-up data of the pertinent literature have been critically revised., Conclusions: Gamma Knife radiosurgery and the other radiosurgical techniques represent a feasible and effective therapy in the management of HPCs. Tumour control and survival rate are comparable to those reported for radiotherapy. Further studies should be focused to define the exact role of Gamma Knife radiosurgery in the management of HPCs.

Because of the rarity of intracranial hemangiopericytomas (HPCs), the role of postoperative radiation therapy (PORT) in the management of HPC remains unclear. This study therefore analyzed the effects of PORT on patterns of failure and survival improvement in patients with HPC. Fifty-two patients surgically treated for intracranial HPC at our institution between 1992 and 2013 were retrospectively analyzed. Patterns of failure were subdivided into local recurrence, regional metastasis, and distant metastasis. Multivariate Cox proportional hazards models were used to assess factors prognostic of treatment failure and survival, and a time-dependent Cox proportional hazards models were used to investigate the correlations between patterns of failure and death. Of the 52 patients, 45 (87 %) underwent gross total resection, and 39 (75 %) received PORT. PORT significantly lengthened local control (LC) and overall survival (OS), by 14 and 13 months, respectively, independent of the extent of resection. Patients who did and did not receive PORT had 5 year LC rates of 97 and 44 %, respectively (HR .05, P = .002); and 10 year OS rates of 83 and 25 %, respectively (hazard ratio (HR) .20, P = .008). PORT, however, did not show preventive effects on regional and distant metastases. The main patterns of failure were local recurrence in patients who did not receive PORT and distant metastasis in those who received PORT. Regional metastasis was a main immediate cause of death (P < .001), and tended to occur more frequently and earlier in patients not receiving PORT.

We report a 29-year-old woman who presented with severe headache, nausea and vomiting. A lesion was found in the left petrous ridge and near-total resection was performed. Pathologic examination showed anaplastic hemangiopericytoma (World Health Organization Grade III). Hemangiopericytoma is an uncommon mesenchymal tumor that rarely occurs in an intracranial location. Prior studies have reported a surprisingly high rate of late recurrence and extracranial metastases from intracranial hemangiopericytomas, including metastases to the lungs. Resection was followed by external beam radiation. The tumor recurred intracranially 6 and 13 years later and was treated with gamma knife stereotactic radiosurgery. At year 14, she noticed a lump in her neck and underwent parotidectomy for a mucoepidermoid carcinoma. This new diagnosis prompted a staging chest CT scan which showed a 4mm right upper lobe lung nodule along with additional < 5 mm indeterminate nodules. Over the next 3 years, the nodule increased to 8mm. Wedge biopsy of the lung nodule showed metastatic hemangiopericytoma, histologically similar to the intracranial hemangiopericytoma. Both the primary and the lung metastasis were positive for CD34 and STAT-6. To the best of our knowledge, this is the longest reported interval between a resected intracranial hemangiopericytoma and a histologically confirmed solitary metastasis to the lung., (Copyright © 2015 Elsevier Ltd. All rights reserved.)

Intracranial hemangiopericytoma (HPC) is a rare malignant meningothelial tumor. The authors retrospectively reviewed the long-term clinical outcomes of patients with HPC with regard to treatment modalities and histopathological grades. Eighteen women and 20 men (mean age 38.5 years, range, 18-62 years) were observed over an average follow-up period of 61 months (range, 15-133 months) between 2003 and 2013. The initial treatment modalities included total tumor resection followed by conventional radiotherapy (RT) (n=27), and subtotal tumor resection followed by stereotactic radiosurgery (n=11). One patient (3%) had permanent neurological deficits, and six patients (16%) died. Thirteen patients (34%) suffered recurrence. One year, 5 year, and 10 year recurrence-free survival rates were 100%, 70%, and 39%, respectively. Five patients (13%) developed metastasis. One year, 5 year, and 10 year metastasis-free survival rates were 100%, 89%, 74%, respectively. Low grade tumors were associated with longer overall survival, recurrence-free interval and metastasis-free interval (log-rank, p<0.05). Radical resection with RT was associated with longer overall survival and recurrence-free interval (log-rank, p<0.05), but had no effect on the metastasis-free interval (log-rank, p=0.245). Thus, radical surgery followed by adjuvant RT is the primary treatment of HPC, but recurrence and metastasis remain a common treatment outcome regardless of initial strategy. It is necessary to maintain long-term follow-up and serial imaging for all patients with intracranial HPC after treatment, regardless of extent of resection., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

Hemangiopericytomas (HPC) are mesenchymal tumors with a propensity towards chronicity and metastasis. This study aimed to reflect a single institution experience with both World Health Organization (WHO) grade II and III HPC. Pathology records from the years 1990-2013 at the University of Washington were searched to identify tumors unequivocally classified as HPC. Electronic chart review was then utilized to collect pertinent patient data. Of the WHO grade II HPC, there were four men and two women (average age 52 years) while the grade III HPC group had eight men and two women (average age 51 years). Sixty-six percent of WHO grade II tumors were located in the middle or posterior fossa as compared to none of the grade III tumors. Survival analysis revealed a significant survival benefit for patients who underwent complete resection (223 months) versus those with subtotal resection (138 months, p<0.05). Factors such as age, sex, the use of up-front radiation, and whether the patient had a recurrence did not show statistical significance related to overall survival or progression free survival. Radiation in the form of external beam radiotherapy given at the time of the first recurrence did trend towards improved progression free survival (56 months) compared to those patients who were not radiated (22 months, p=0.09) All patients with radical resection went on to never have a recurrence. Our results indicate that HPC are tumors with limited response to radiation and best treated with aggressive resection. Future studies will determine whether molecular-based therapies may provide added adjuvant benefit., (Copyright © 2013 Elsevier Ltd. All rights reserved.)

Objective: Meningeal hemangiopericytoma (HPC) is a rare, aggressive central nervous system tumor that tends to invade locally and to metastasize, and has a high rate of recurrence., Methods: This study presents a retrospective review of patients managed for intracranial HPC at Rome University Hospital., Results: A total of 43 patients with intracranial HPC were treated from 1980 to 2010. Treatment and follow-up information was available for analysis on 36 patients. The median survival for all patients was 83.5 months after date of diagnosis, with 1-year, 5-year, and 10-year survival rates of 100%, 94.4%, and 72.2%, respectively. Eighteen patients (41.86%) had HPC recurrence. The median time until recurrence was 72.24 months, with 1-year, 5-year, and 10-year progression-free survival rates of 98%, 51%, and 29%, respectively. Five patients (11.62%) developed extracranial metastasis. Patients undergoing any form of adjuvant radiation treatment, including external beam radiotherapy, Gamma Knife radiosurgery, and/or proton beam therapy, had no longer median overall survival (OS) (178 vs. 154 months, respectively; P = .2); but did have a significantly improved recurrence-free interval (108 vs. 64 months; P = .04) compared with patients who did not undergo radiation treatment. Tumor characteristics associated with earlier recurrence included size ≥7 cm (log-rank, P < .05) and sinus invasion (log-rank, P < .05)., Conclusions: Strategies combining adjuvant radiation with tumor resection seemed to hinder tumor progression, but had no effect on OS or the development of metastases. Greater extent of resection was associated with increased OS (log-rank, P < .05). Anaplastic HPC was associated with reduced OS and with reduced recurrence interval (log-rank, P < .05)., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Object: Central nervous system (CNS) hemangiopericytomas are relatively uncommon and unique among CNS tumors as they can originate from or develop metastases outside of the CNS. Significant difference of opinion exists in the management of these lesions, as current treatment paradigms are based on limited clinical experience and single-institution series. Given these limitations and the absence of prospective clinical trials within the literature, nationwide registries have the potential to provide unique insight into the efficacy of various therapies., Methods: The authors queried the Surveillance Epidemiology and End Results (SEER) database to investigate the clinical behavior and prognostic factors for hemangiopericytomas originating within the CNS during the years 2000-2009. The SEER survival data were adjusted for demographic factors including age, sex, and race. Univariate and multivariate analyses were performed to identify characteristics associated with overall survival., Results: The authors identified 227 patients with a diagnosis of CNS hemangiopericytoma. The median length of follow-up was 34 months (interquartile range 11-63 months). Median survival was not reached, but the 5-year survival rate was 83%. Univariate analysis showed that age and radiation therapy were significantly associated with survival. Moreover, young age and supratentorial location were significantly associated with survival on multivariate analysis. Most importantly, multivariate analysis using the Cox proportional hazards model showed a statistically significant survival benefit for patients treated with gross-total resection (GTR) in combination with adjuvant radiation treatment (HR 0.31 [95% CI 0.01-0.95], p = 0.04), an effect not appreciated with GTR alone., Conclusions: The authors describe the epidemiology of CNS hemangiopericytomas in a large, national cancer database, evaluating the effectiveness of various treatment paradigms used in clinical practice. In this study, an overall survival benefit was found when GTR was accomplished and combined with radiation therapy. This finding has not been appreciated in previous series of patients with CNS hemangiopericytoma and warrants future investigations into the role of upfront adjuvant radiation therapy.

The prognosis of the treatment of brain tumours depends on two main factors: biological nature and localisation of the neoplasm. Requirements of oncologic surgery can be met only partially if at all in neurological surgery of brain tumours. Resectability depends primarily on localisation of the neoplasms. The leading principle is preservation of fine neural structures, minimising morbidity from tissue resection with the goal of maximal tumour resection. As nervous structures and the target volume do not move in the intracranial space, large radiation doses unusual in traditional radiotherapy can be given either in one or in fractionated sessions to small targets (point-radiation) and a well-controlled radiation necrosis of the pathological tissue can be achieved. Management principles of treatment of skull-base related tumours are very similar due to high risks of functional morbidity evoked by surgical injury to the cranial nerves, brainstem structures, vessels of the Willis circle and those of the substantia perforata anterior and posterior, etc. Such tumours are neoplasms arising from the skull base, those infiltrating the cavernous sinuses, invasive pituitary tumours, those arising from the glomus jugulare, or located within the cerebello-pontine angle, etc. This manuscript intends to illustrate and prove the hypothesis by means of 4 cases that fractionated stereotactic radiotherapy (fSRT) is an important part of treatment armamentarium in the latter cases, as it is capable of exploiting both the advantages of traditional fractionated irradiation and that of the high conformality and selectivity of radiosurgery. It is capable of administering appropriate quantity of total target dose with a lower than limit dose on surrounding structures. The presentation proves that fSRT can be planned already in the phase of surgical indication as a "microsurgery-assisted radiotherapy".

Background: Meningeal hemangiopericytoma (M-HPC) is a rare entity., Objective: To characterize our institutional experience in treating M-HPC., Methods: We reviewed the medical records of patients with M-HPC evaluated at The University of Texas M.D. Anderson Cancer Center between 1979 and 2009., Results: We identified 63 patients diagnosed between 1979 and 2009 with M-HPC treated with surgery alone or with postoperative radiotherapy (PORT). The majority were male (59%) and with a median age of 40.9 years (range, 0-71). Gross total resection (GTR) predominated (n = 31, 49%) followed by subtotal resection (n = 23, 37%) and unknown status (n = 9, 14.3%). PORT was delivered to 39 of the 63 patients (62%). The 5-, 10-, and 15-year overall survival were 90%, 68%, and 28%, respectively. The 5-, 10-, and 15-year local control (LC) were 70%, 37%, and 20%, respectively. The 5-, 10-, and 15-year metastasis-free survival were 85%, 39%, and 7%. PORT resulted in improved LC (hazard ratio [HR] 0.38, P = .008). Radiotherapy (RT) dose ≥60 Gy correlated with improved LC relative to <60 Gy (HR 0.12, P = .045). GTR correlated with improved LC (HR 0.40, P = .03). On multivariate analysis, PORT (HR 0.33, P = .003), GTR (HR = 0.33, P = .008), and RT dose ≥60 Gy (HR 0.33, P = .003) correlated with improved LC. Among those with GTR, PORT resulted in improved LC (HR 0.18, P = .027). Extent of resection and PORT did not correlate with improved overall survival., Conclusion: In M-HPC, both PORT and GTR independently correlate with improved LC. PORT improves LC following GTR. We recommend RT dose ≥60 Gy to optimize LC.

Central nervous system hemangiopericytoma (HPC) is a malignant vascularized mesenchymal tumor with a high rate of recurrence. Because of its rarity, few clinical characteristics and prognostic analysis information regarding recurrent HPC exist for doctors to pursue optimal outcomes. Forty-six recurrent HPC cases treated at our hospital between 2004 and 2012 were compiled into a single database based on a retrospective review of patient records, which were used to summarize the clinical characteristics. The mean survival of the recurrent HPC patients in our cohort was 41.6 ± 4.4 months, with 1-, 2-, 3-, and 4-year survival rates of 80.4, 65.2, 59.2, and 53.8 %, respectively. Thirty patients (65.2 %) suffered their first tumor recurrence, with a mean survival of 36.9 ± 4.1 months. Sixteen patients (34.8 %) suffered a second or further tumor recurrence, with a mean survival of 39.7 ± 7.0 months. Eighteen patients (39.1 %) died of all causes during the follow-up period, with a mean survival of 14.2 ± 5.6 months. Univariate and multivariate regression analyses showed that factors associated with good prognosis included recurrence age over 35 years, an interval between the first and second recurrence of more than 1 year and a clear boundary of the recurrent tumor. Gross total resection with adjuvant external beam radiotherapy could independently delay tumor recurrence of the second or more times and prolong the postoperative survival; thus, this strategy should be pursued as the initial treatment.

Purpose: The aim of this study was to examine the effect of postoperative radiation therapy (RT) on cause-specific survival in patients with meningeal hemangiopericytomas., Methods and Materials: The Surveillance, Epidemiology, and End Results database from 1990-2008 was queried for cases of surgically resected central nervous system hemangiopericytoma. Patient demographics, tumor location, and extent of resection were included in the analysis as covariates. The Kaplan-Meier product-limit method was used to analyze cause-specific survival. A Cox proportional hazards regression analysis was conducted to determine which factors were associated with cause-specific survival., Results: The mean follow-up time is 7.9 years (95 months). There were 76 patients included in the analysis, of these, 38 (50%) underwent gross total resection (GTR), whereas the other half underwent subtotal resection (STR). Postoperative RT was administered to 42% (16/38) of the patients in the GTR group and 50% (19/38) in the STR group. The 1-year, 10-year, and 20-year cause-specific survival rates were 99%, 75%, and 43%, respectively. On multivariate analysis, postoperative RT was associated with significantly better survival (HR = 0.269, 95% CI 0.084-0.862; P=.027), in particular for patients who underwent STR (HR = 0.088, 95% CI: 0.015-0.528; P<.008)., Conclusions: In the absence of large prospective trials, the current clinical decision-making of hemangiopericytoma is mostly based on retrospective data. We recommend that postoperative RT be considered after subtotal resection for patients who could tolerate it. Based on the current literature, the practical approach is to deliver limited field RT to doses of 50-60 Gy while respecting the normal tissue tolerance. Further investigations are clearly needed to determine the optimal therapeutic strategy., (Published by Elsevier Inc.)

Background: Intracranial hemangiopericytoma (HPC) is a rare malignancy for which treatment recommendations vary., Objective: We sought to characterize outcomes of HPC patients treated with postoperative external beam radiotherapy (PORT)., Methods: A retrospective analysis was conducted using the Surveillance, Epidemiology and End Results (SEER) Program of the US National Cancer Institute. We identified patients with intracranial hemangiopericytoma who underwent surgery alone or PORT., Results: We identified 88 patients with a diagnosis of HPC between 1982 and 2009 treated with surgery alone or PORT. The majority of patients were female (53%) and white (84%) with a median age of 50.5 years (range, 0-92 years). Gross total resection (GTR) was achieved in 55%, and PORT was delivered to 48% of the entire cohort. The median overall survival (OS) and cause-specific survival (CSS) were 111 months and 161 months, respectively. On univariate analysis, age older than 50 years correlated with poor OS (hazard ratio [HR]: 3.43; 95% confidence interval [CI]: 1.70-6.95; P = .001) and CSS (HR: 2.77; 95% CI: 1.18-6.48; P = .019). On multivariate analysis (MVA), age >50 years correlated with poor OS (HR: 3.69; 95% CI: 1.72-7.93; P = .001) and CSS (HR: 2.67; 95% CI: 1.08-6.59; P = .034). On MVA, GTR correlated with improved OS (HR: 0.28; 95% CI: 0.11-0.71; P = .007) and CSS (HR: 0.23; 95% CI: 0.07-0.76; P = .016). In addition, PORT correlated with improved OS (MVA HR: 0.02; 95% CI: 0.00-0.31; P = .005) and CSS (MVA HR: 0.02; 95% CI: 0.00-0.45; P = .015). Patients undergoing STR with PORT compared favorably with those undergoing GTR alone with respect to OS (HR: 0.43; 95% CI: 0.15-1.26; P = .13) and CSS (HR: 0.51; 95% CI: 0.15-1.78; P = .29)., Conclusion: In intracranial HPC, both PORT and GTR independently correlate with improved OS and CSS.

Background: Intracranial meningeal hemangiopericytoma (HPC) represents a rare and aggressive intracranial neoplasm located along the dural sinuses. It constitutes less than 1 % of all intracranial tumors and approximately 2-4 % of all meningeal tumors. The authors present our institute's experience in combined modality management of 15 successive patients of HPC., Methods: We retrospectively reviewed 15 patients of HPC treated in our institute from 2001 to 2011. Clinical characteristics and treatment modality, in the form of surgery and radiotherapy, were noted. Statistical analysis was done with regards to recurrence free survival (RFS) and overall survival (OS) using Kaplan-Meier survival analysis., Results: The median age of the patients was 40.0 years. Nine patients were males and six patients were females. The median duration of symptoms was six months. Headache was the most common presenting symptom followed by vomiting, motor weakness and seizures. Twelve patients underwent total excision while three had subtotal excision. Seven patients had WHO grade II histology tumors and eight patients had grade III histology. Thirteen patients received adjuvant radiotherapy (RT). Median RT dose delivered was 50 Gy. Five patients developed local recurrence. One patient had distant metastases. Median duration of RFS was 68 months., Conclusions: HPCs are aggressive tumors. The mainstay of therapy is gross total resection at the initial surgery. Postoperative adjuvant RT should be offered to all patients, regardless of the degree of resection achieved. Long-term follow-up is important as local recurrences and distant metastases can develop years after the initial treatment.

Hemangiopericytoma (HPC) is a rare tumor of uncertain malignant potential arising from mesenchymal cells with pericytic differentiation. It accounts for 3-5% of soft tissue sarcomas and 1% of vascular tumors. It usually presents in 5th to 6th decade of life. Most common sites are limbs, pelvis and head and neck. About 20% of all hemangiopericytomas are seen in head and neck, mostly in adults. Usually it presents in orbit, nasal cavity, oral cavity, jaw, parotid gland, parapharyngeal space, masticator space and jugular foramen. Long term follow up is important because of imprecise nature of the histological criteria for prediction of biologic behavior.We report herein a case of HPC in 66-year-old man, who presented in our department with headache, nasal obstruction and dysphagia. A neck computer tomography scan and magnetic resonance imaging showed a large left parapharyngeal mass bulging into nasopharynx and oropharynx with extension to pharyngeal mucosal surface and causing narrowing of airways and total obstruction of left posterior nostril. Angiography showed a highly vascular neoplasm. Initially he was managed as a case of schwannoma and embolization was done but with no response. An attempt to do complete surgical resection was made, but due to its critical position, it was not possible. During surgery, highly vascularised tumor was found. The histopathologic examination revealed a vascular tumor consistent with hemangiopericytoma G-II. The patient had normal postoperative course of healing and was given adjuvant radiation. He is on regular follow up without signs of recurrence or metastases.In summary, parapharyngeal space is a rare site of presentation for hemangiopericytoma which is highly vascular tumor, requiring extensive work up including magnetic resonance imaging, computed tomography scan and angiography. Complete surgical excision should be attempted. Postoperative radiation is indicated in cases of incomplete resection.

Intracranial hemangiopericytoma (HPC) is an aggressive meningothelial neoplasm. A particularly challenging aspect of management of patients with HPC is optimizing treatment for recurrence, progression, and extracranial metastasis. Here we describe a modern cohort of patients with recurrent HPC to better understand treatment strategies that may improve outcome. Patients managed at UCSF for recurrent intracranial HPC were compiled into a single database based on a retrospective review of patient records, including operative, radiologic, and clinic reports. Cox regression was performed to determine factors that independently predicted treatment outcomes. At UCSF, 14 patients with available treatment and follow-up data were seen for management of HPC recurrence. Eight patients underwent repeat surgical resection, of whom four received adjuvant external beam radiotherapy (EBRT), one received additional Gamma Knife radiosurgery (GKS), and one received brachytherapy. Radiosurgical intervention alone was utilized for recurrence in six patients, with four receiving GKS and two receiving CyberKnife. Nine patients suffered a second recurrence at a median time of 3.5 years following reintervention. Nine patients died following reintervention, with a median survival of 7.9 years following intervention for recurrence. In univariate analysis, factors associated with increased time to second recurrence included non-posterior fossa location (log rank, p < 0.05) and surgical resection with adjuvant EBRT (log rank, p < 0.05). The addition of adjuvant EBRT to surgical resection similarly extended overall survival compared to surgical resection alone (log rank, p < 0.05). GKS was associated with earlier second recurrence compared to surgically based strategies (log rank, p < 0.05). We conclude that when combined with surgical resection, EBRT appears promising in the extension of second recurrence-free survival and overall survival. This multimodality approach also appears to outperform GKS in extending time to second recurrence. Accordingly, when safe and feasible, surgical resection of recurrent HPC with adjuvant EBRT should be the first steps in management., (Copyright © 2011 Elsevier Ltd. All rights reserved.)

A 77-year-old man presented for evaluation of a recurrent right orbital hemangiopericytoma. It had been excised twice over the past 6 years. After the second resection, the tumor rapidly recurred and was insensitive to systemic chemotherapy, and the patient was thus referred to the authors' institution. The patient had proptosis, restricted ocular movement, and binocular diplopia on presentation. Orbital imaging confirmed a well-circumscribed right extraconal mass in the medial orbit. Preoperative radiation therapy was given, which reduced the tumor volume considerably and allowed a successful complete surgical excision of the mass.

Object: Hemangiopericytomas are rare tumors that behave aggressively with a high rate of local recurrence and distant metastases. With the aim of determining the outcome and response to various treatment modalities, a series of 39 patients who underwent microsurgical resection for primary meningeal hemangiopericytoma over a 24-year period is presented., Methods: Patients with hemangiopericytoma were identified from histopathology records and their medical records were analyzed retrospectively by 2 independent reviewers to collect data on surgical treatment, adjuvant therapy, postoperative course, local or distant recurrence, and follow-up., Results: Of the 39 patients, 19 were male and 20 were female. Mean patient age was 44.1 years. Thirty-four tumors were intracranial and 5 were spinal. The mean follow-up period was 123 months. Twenty-eight patients developed local recurrence. The recurrence rate at 1, 5, and 15 years was 3.5%, 46%, and 92%, respectively. Extraneural metastasis occurred in 8 patients (26%) at an average of 123 months after initial surgery. Recurrences and metastases were treated by surgical excision, external beam radiation therapy (EBRT), chemotherapy, and/or stereotactic radiosurgery. Adjuvant EBRT following initial surgery was found to extend the disease-free interval from 154 months to 254 months, although it did not prevent the development of metastasis. In those patients with EBRT and complete resection, the mean recurrence-free interval was found to be 126.3 months longer (p = 0.04) and overall survival 126 months longer than without EBRT. Furthermore, adjusting for resection, patients undergoing EBRT had 0.33 times increased risk of recurrence compared with those who did not (p = 0.03). A majority of patients remained able to live independently despite revision surgery for recurrence., Conclusions: The mean follow-up of this patient series represents the longest follow-up duration published to date and demonstrates extended survival in a significant number of patients with hemangiopericytoma. Gross-total resection followed by adjuvant EBRT provides patients with the highest probability of an increased recurrence-free interval and overall survival. Prolonged survival justifies long-term follow-up and aggressive treatment of initial, recurrent, and metastatic disease.

Hemangiopericytoma of the pineal region is exceedingly rare. We describe a patient with a large pineal region hemangiopericytoma who underwent a third ventriculostomy followed by a gross total resection by a unilateral interhemispheric approach with adjuvant radiotherapy. The patient remains recurrence free 4 years after treatment., (Copyright 2010 Elsevier Ltd. All rights reserved.)

Object: Intracranial hemangiopericytoma (HPC) is a rare and malignant extraaxial tumor with a high proclivity toward recurrence and metastasis. Given this lesion's rarity, little information exists on prognostic factors influencing mortality rates following treatment with surgery or radiation or both. A systematic review of the published literature was performed to ascertain predictors of death following treatment for intracranial HPC., Methods: The authors identified 563 patients with intracranial HPC in the published literature, 277 of whom had information on the duration of follow-up. Statistical analysis of survival was performed using Kaplan-Meier and Cox regression analysis., Results: Hemangiopericytoma was diagnosed in 246 males and 204 females, ranging in age from 1 month to 80 years. Among patients treated for HPC, overall median survival was 13 years, with 1-, 5-, 10-, and 20-year survival rates of 95%, 82%, 60%, and 23%, respectively. Gross-total resection alone (105 patients) was associated with superior survival rates overall, with a median survival of 13 years, whereas subtotal resection alone (23 patients) resulted in a median survival of 9.75 years. Subtotal resection plus adjuvant radiotherapy led to a median survival of 6 years. Gross-total resection was associated with a superior survival benefit to patients regardless of the addition or absence of radiation, and patients receiving > 50 Gy of radiation had worse survival outcomes (median survival 4 vs 18.6 years, p < 0.01, log-rank test). Patients with tumors of the posterior fossa had a median survival of 10.75 versus 15.6 years for those with non-posterior fossa tumors (p < 0.05, log-rank test)., Conclusions: Treatment with gross-total resection provides the greatest survival advantage and should be pursued aggressively as an initial therapy. The addition of postoperative adjuvant radiation does not seem to confer a survival benefit.