Intracranial Hemangiopericytoma—Our Experience in 30

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GEORGE KWOK CHU WONG ET AL. INCIDENCE AND MORTALITY OF SPONTANEOUS SAH IN HONG KONG

study on cognitive dysfunction after spontaneoussubarachnoid haemorrhage: patient profiles andrelationship to cholinergic dysfunction. ActaNeurochir (Wien) 151:1601-1607, 2009.

35. Woodworth GF, Biard CJ, Garces-Ambrossi G,Tornascia J, Tamargo RJ: Inaccuracy of theadministrative database: comparative analysis oftwo databases for the diagnosis and treatment ofintracranial aneurysms. Neurosurgery 65:251-256,2009.

36. Yasuno K, Bilguvar K, Bijlenga P, Low SK,Krischek B, Auburger G, Simon M, Krex D,Arlier Z, Nayak N, Ruigrok YM, Niemela M,Tajima A, von und zu Fraunberg M, Doczi T,Wirjatijasa F, Hata A, Blasco J, Oszvald A,Kasuya H, Zilani G, Schoch B, Singh P, Stuer C,Risselada R, Beck J, Sola T, Ricciardi F,Aromaa A, Illig T, Schreiber S, van Duijn CM, van

Key words- Hemangiopericytoma- Intracranial- Recurrence- Survival- Treatment

Abbreviations and AcronymsEBRT: External beam radiotherapyGTR: Gross total resectionHPC: HemangiopericytomaOS: Overall survivalSTR: Subtotal resectionWHO: World Health Organization

From the Departments of 1Neurosurgery and2Neuroradiology, Sapienza University of Rome;

and the 3Department of Neurosurgery INM—IRCCSNeuromed, Pozzilli (Isernia), Sapienza University of Rome,Rome, Italy

To whom correspondence should be addressed:Angelina Graziella Melone, M.D.[E-mail: [email protected]]

Citation: World Neurosurg. (2014) 81, 3/4:556-562.http://dx.doi.org/10.1016/j.wneu.2013.11.009

Journal homepage: www.WORLDNEUROSURGERY.org

Available online: www.sciencedirect.com

1878-8750/$ - see front matter ª 2014 Elsevier Inc.

556 www.SCIENCEDIRECT.com

den Berg LH, Perret C, Proust C, Roder C,Ozturk AK, Gaal E, Berg D, Geisen C,Friedrich CM, Summers P, Frangi AF, State MW,Wichmann HE, Breteler MM, Wijmenga C,Mane S, Peltonen L, Elio V, Stirkenboom MC,Lawford P, Bryne J, Macho J, Sandalcioglu EI,Mayer B, Raabe A, Steinmetz H, Rufenacht D,Jaaskelainen JE, Hernesniemi J, Rinkel GJ,Zembutsu H, Inoue I, Palotie A, Cambien F,Nakamura Y, Lifton RP, Gunel M: Genome-wideassociation study of intracranial aneurysm iden-tifies three new loci. Nat Genet 42:420-425, 2010.

37. Yasuno K, Bakircioglu M, Low SK, Bilguvar K,Gaal E, Ruigrok YM, Niemela M, Hata A,Bijlenga P, Kasuya H, Jaaskelainen JE, Krex D,Auburger G, Simon M, Krischek B, Zembutsu H,Inoue I, Palotie A, Cambien F, Nakamura Y,Lifton RP, Gunel M: Common variant near the

-OBJECTIVE: Meningeal hemangiopericcentral nervous system tumor that tendsand has a high rate of recurrence.

-METHODS: This study presents a retrofor intracranial HPC at Rome University H

-RESULTS: A total of 43 patients with into 2010. Treatment and follow-up informapatients. The median survival for all padiagnosis, with 1-year, 5-year, and 10-ye72.2%, respectively. Eighteen patients (41.time until recurrence was 72.24 monthprogression-free survival rates of 98%, 51%(11.62%) developed extracranial metastaadjuvant radiation treatment, includingKnife radiosurgery, and/or proton beam tsurvival (OS) (178 vs. 154 months, respectcantly improved recurrence-free interval (with patients who did not undergo radiaassociated with earlier recurrence includsinus invasion (log-rank, P < .05).

-CONCLUSIONS: Strategies combiningtion seemed to hinder tumor progressiodevelopment of metastases. Greater exteincreased OS (log-rank, P < .05). AnaplasOS and with reduced recurrence interval

WORLD NEUROSURGERY, http://

endothelin receptor type A (EDNRA) gene isassociated with intracranial risk. Proc Natl AcadSci USA 108:19707-19712, 2011.

Conflict of interest statement: This study was supported bythe Neurosurgery Research and Training Fund, the ChineseUniversity of Hong Kong.

Received 26 October 2012; accepted 27 July 2013;published online 22 September 2013




1878-8750/$ - see front matter ª 2014 Elsevier Inc.All rights reserved.

Intracranial Hemangiopericytoma—Our Experience in 30 Years: A Series of 43 Cases
and Review of the Literature
Angelina Graziella Melone1, Alessandro D’Elia1, Francesca Santoro2, Maurizio Salvati3, Roberto Delfini1,Giampaolo Cantore3, Antonio Santoro1

ytoma (HPC) is a rare, aggressiveto invade locally and to metastasize,

spective review of patients managedospital.

tracranial HPC were treated from 1980tion was available for analysis on 36tients was 83.5 months after date ofar survival rates of 100%, 94.4%, and86%) had HPC recurrence. The medians, with 1-year, 5-year, and 10-year, and 29%, respectively. Five patients

sis. Patients undergoing any form ofexternal beam radiotherapy, Gammaherapy, had no longer median overallively; P [ .2); but did have a signifi-108 vs. 64 months; P [ .04) comparedtion treatment. Tumor characteristicsed size ‡7 cm (log-rank, P < .05) and

adjuvant radiation with tumor resec-n, but had no effect on OS or thent of resection was associated withtic HPC was associated with reduced(log-rank, P < .05).
INTRODUCTION
Intracranial hemangiopericytoma (HPC) isa rare, vascularized mesenchymal tumor.It develops from malignant transformation

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http://dx.doi.org/10.1016/j.wneu.2013.07.128

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ANGELINA GRAZIELLA MELONE ET AL. TREATMENT AND PROGNOSIS OF INTRACRANIAL HEMANGIOPERICYTOMA

of pericytes, cells that form the walls ofcapillaries and postcapillary venules. HPCexhibits a characteristically monotonousappearance under low-power microscopy,as well as well-developed branching stag-horn vasculature with walls of varyingthickness (16). HPC is almost alwaysattached to the dura, and has a strongtendency to recur locally and to metasta-size outside the central nervous system(5, 7, 27, 35, 36, 38). Recurrence usuallyprecedes metastasis (14, 26).Although found in similar arachnoid

locations as generally benign meningi-omas, HPC has a more aggressive biologyfacilitating local recurrence and distantmetastasis (9). HPC is considered a WorldHealth Organization grade II neoplasm,with anaplastic variants classified as gradeIII (25).HPC remains a rare entity thought to

represent 0.4% of all primary centralnervous system tumors, making menin-gioma approximately 50 to 60 times morecommon (25, 28). Extracranial location ismore frequent in HPCs than in menin-giomas (4). These extremely vascular tu-mors are challenging to treat, especiallyin pediatric cases because of the smalleroverall blood volume in children. Thesurgeon must be careful when interrupt-ing the blood supply to the tumor, andpreoperative endovascular embolizationis recommended. Infantile HPC is char-acterized by a more benign course, withresponsiveness to chemotherapy andeven a tendency to spontaneous regres-sion. In contrast, the behavior of HPCin children older than 1 year does notseem to differ from that of HPC inadults (24).Because HPC closely mimics meningi-

oma in clinical and radiographic presenta-tion, histological confirmation is the onlydefinitive means of distinguishing theselesions (32). In terms of histologicalgrading, Zhou et al. (37) found that intra-cranial anaplastic HPC tended to havemoremarked lobulated and cross-leaf growthand was more likely to bleed. Featuresuseful in distinguishing anaplastic HPCfrom its lower-grade counterparts includenecrosis and cystic components, andanaplastic HPC is more likely to show het-erogeneous signal on neuroimaging andless likely to exhibit the dural tail sign.Destruction of adjacent skull and

WORLD NEUROSURGERY 81 [3/4]: 556-5

peritumoral edema also are morecommonly seen in anaplastic HPC, as arerecurrence and extracranial metastasis.

PATIENTS AND METHODS

The series presented herein was generatedby reviewing the histopathological recordsat Sapienza University of Rome Hospital,Department of Neurosurgery, to identify allpatients diagnosed with HPC since 1980(comprising some cases retrospectivelyreviewed after 1993). Some patients under-went initial treatment for the primary dis-ease at another institution and were laterseen at our institution for follow-up ormanagement of recurrent disease. In thesecases, primary treatment was determinedvia review of medical and radiological re-cords. All patients had pathologicallyconfirmed HPC reviewed by the neuropa-thology department at our institution.Pathology reports were reviewed to confirmdiagnosis; in addition, all specimens weregraded according to the Mena classification(26) into low or high grade. According tothese criteria, high-grade meningeal HPCwas characterized by the presence of ne-crosis and/or more than 5 mitoses for 10microscopic fields (magnification 400�),and at least 2 of the following microscopicfeatures: hemorrhage, moderate to highnuclear atypia, and moderate to highcellularity.Data on demographics, tumor charac-

teristics, treatment modalities, survival,and length of follow-up also were recorded.All cases of HPC recurrence and metastasiswere documented radiographically, andmortality data were confirmed by chart re-view. Patient data were analyzed by sex,age, length of survival, and mortality. Tu-mor data were analyzed by size, location,recurrence, and metastasis. Tumor size wasdetermined by measuring the maximumradiographic diameter. Recurrence wasdefined as reappearance of tumor withinthe cranial cavity, or an increase in size ofresidual tumor. Metastasis was defined asthe presence of HPC tissue in any extra-cranial location. Treatment data wereanalyzed by modality, extent of resection,use of adjuvant radiotherapy, use ofGamma Knife radiosurgery, and amount ofradiation received; none of our patientswere treated with chemotherapy. All treat-ment analyses performed were based on

62, MARCH/APRIL 2014 ww

the primary intervention that a patientreceived, whether that occurred at anoutside institution or at our institution.Follow-up time for overall survival (OS) wasdefined as the time between primary sur-gery and death or the last known date ofposttreatment follow-up. Follow-up timefor recurrence-free survival was defined asthe time between primary surgery withpathological diagnosis of HPC and themost recent imaging study demonstratingradiographic absence or progression of tu-mor. Follow-up time for metastasis wasdefined as the time until radiographic evi-dence of HPC progression extracranially, orthe last known date of follow-up. Univari-ate survival analysis was performed usingthe Kaplan-Meier method to evaluate theprognostic significance of patient and tu-mor characteristics, and the efficacy ofvarious treatment modalities. Significancewas determined using the log-rank test,with the P value considered significant atthe 5% (P ¼ .05) level. A multivariate Coxregression analysis was then performed todetermine the independent impact of fac-tors found to be significant or trendingtoward significance in univariate analysis.All descriptive and statistical analysis wasperformed using SPSS version 12.0.

RESULTS

Forty-three cases of HPC with provenhistological diagnosis were identified be-tween 1980 and 2010. All patients wereinitially treated with microsurgical resec-tion. Subtotal resection (STR) was per-formed when gross total resection (GTR)was not possible. Twenty-six patients werefemale (60.5%) and 17 (39.5%) were male,with a median age at first operation of46.9 years (range 19 to 79 years). Tumorsmost frequently occurred superficially andclosely related to the meninges, in thefalx/parasagittal area (55%). Eight (18.6%)tumors were associated with venous sinuscompression or invasion. Symptoms wererelated to tumor location. The most com-mon symptoms were headache (40%) andupper and/or lower limb weakness (12%).Seven patients who lived overseas werelost to follow-up after the initial surgeryand were excluded from further analysis.The median follow-up period was 118months (range 24 to 384 months). Sevenpatients were diagnosed and underwent

w.WORLDNEUROSURGERY.org 557


Table 1. Treatment Strategy: Subgroups

Treatment Strategy Patients (n) Recurrence (n) Metastasis (n) Mortality (n)

GTR alone 19 5 1 1

GTR þ EBRT 11 4 1 2

STR alone 3 3 1 4

STR þ EBRT 10 6 2 7

EBRT, external beam radiotherapy; GTR, gross total resection; STR, subtotal resection.

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initial treatment at other hospitals. Thesepatients were included in the studybecause subsequent treatment was carriedout at our institution.The median survival for all patients was

83.5 months after date of diagnosis, with1-year, 5-year, and 10-year OS rates of 100%,94.4%, and 72.2%, respectively (Figure 1).Thirty-three tumors (76.7%) were classi-

fied as World Health Organization (WHO)grade II and 10 (23.3%) as WHO grade III.Eighteen (41.86%) of the 43 had at least 1recurrence. Nine of 18 recurrences wereWHO grade III. Extracranial metastasis wasseen in 5 cases, all ofwhichwereWHOgradeIII HPC and were situated in the lung (1),liver (3), and peritoneum (1). For surgicalremoval of the lesions, we used the oper-ating microscope, microsurgical instru-mentation, a neuronavigation system, brainmapping techniques, and neurophysiolog-ical intraoperative monitoring (from 2000).

GTR Is Associated with Longer OS andLonger Recurrence-Free IntervalAt first operation, GTR was achieved in 30(69.76%) cases and STR in 13 (30.23%)cases. Six patients with GTR receivedpreoperative embolization, and 6 patientswho underwent STR also received preop-erative embolization. After GTR, 11 pa-tients (36.67%) received adjuvant externalbeam radiotherapy (EBRT). Most patientsreceiving STR were further treated withadjuvant radiation, including 10 of the 13(76.9%) who received EBRT, 5 (50%) ofwhom received Gamma Knife radiosurgeryand 5 (50%) of whom received protonbeam therapy (Table 1). Eighteen patients(41.86%) had HPC recurrence. The

Figure 1. Overall s


median time until recurrence was 72.24months after the first surgery (mean 64months), with 1-year, 5-year, and 10-yearprogression-free-survival rates of 98%,51%, and 29%, respectively. The recur-rence rate at 1, 5, and 10 years was 2.7%,50%, and 72%, respectively (Figure 2). Allpatients with local recurrence underwentrepeat surgery. Ten patients received GTR,2 of whom received 60 Gy of adjuvantEBRT to the resection bed. Eight patientsreceived STR, 2 of whom received post-operative EBRT to residual tumor, GammaKnife surgery with a 16-Gy marginal dose,as recommended by Kim et al. (22), whofound thatdoseshigher thanpreviously used(around 15 Gy) are desirable for better localtumor control of HPCs and are not associ-ated with severe adverse radiation effects.The median OS after the second operationwas 48 months (range 8 to 60 months). Sixpatients had a second recurrence after theinitial retreatment; they all underwent repeatsurgery and 5 went on to have a thirdrecurrence, at which point no more opera-tions or radiotherapy were performed. The

urvival rate.


average survival period for these patientswas 28.3 months (range 4 to 57 months,median 32 months). In the period afterthe first, second, and third operations,median Karnofsky Performance Statuspassed from 85 to 80 and subsequentlyto 60.The 5 (11.62%) patients in whom extra-

cranial metastases developed, with metas-tases involving the liver (3 patients), lung(1 patient), and peritoneum (1 patient), alldied of systemic disease. Overall, patientsin the complete excision group had both alonger OS (235 vs. 175 months; P ¼ .047)and a longer recurrence-free interval (117vs. 54 months; P ¼ .0025) (Table 2).

Anaplastic HPC Is Associated withReduced OS and Reduced Recurrence-Free IntervalThirty-three (76.7%) patients were classi-fied as having low-grade tumors. Overallmean survival was 256 and 142 months inthe low- and high-grade tumor groupsrespectively, a difference of 114 months(P ¼ .03). Eighteen tumors recurred, 9 ofwhich were high-grade, and the remainderwere low-grade. All patients experiencingrecurrence underwent revision surgery. Of9 low-grade lesions, 4 (44.4%) demon-strated high-grade transformation and hada second recurrence. High-grade tumorsrecurred an average of 36 months earlierthan low-grade tumors (59 vs. 95 months,respectively; P ¼ .1) (Table 3).

Adjuvant Radiation Therapy Does NotConfer a Longer OS but Does Prolong theRecurrence-Free IntervalPatients undergoing any form of adjuvantradiation treatment, including EBRT,Gamma Knife radiosurgery, and/or protonbeam therapy, did not have longer median

dx.doi.org/10.1016/j.wneu.2013.11.009



Figure 2. Recurrence rate.

Table 3. Histological Grade, OverallSurvival, Recurrence-Free Survival

HistologicalGrade

OverallSurvival

Recurrence-FreeInterval

Low grade 256 months 95 months

High grade 114 months 59 months

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OS (178 vs. 154months, respectively; P¼ .2),but did have a significantly improvedrecurrence-free interval (108 vs. 64 months,respectively; P ¼ .04) compared with pa-tients who did not undergo radiosurgery.The 1-, 5-, and 10-year recurrence ratesfor irradiated tumors were 24%, 35%, and65%, respectively; for nonirradiated tumors,the rates were 36%, 48%, and 88%, respec-tively (Figure 3). No adverse effects, such asradiation necrosis or marked peritumoraledema, were observed in any patients.

Adjuvant Radiation Treatment, not Extentof Resection, Increases the Recurrence-Free IntervalWhen considering extent of resection, STRwith adjuvant radiation trended towardindependently predicting increased time torecurrence when compared with GTR alone(log-rank, P¼ .132) (Figure 4). We found nosignificant difference in time until recur-rence among patients receiving GTR withadjuvant radiation vs. patients receivingGTR alone (log-rank, P ¼ .286), in patientsreceiving STR with adjuvant radiation vs.patients receiving STR alone (log-rank, P ¼.299), or in patients receiving GTR withEBRT vs. patients receiving STR with EBRT(log-rank, P ¼ .713).

Table 2. Degree of Resection, Overall Surviv

Grade of Excision Overall Su

Gross total resection (69.76) 235 mo

Subtotal resection (30.23%) 175 mo


Extent of Resection, not AdjuvantRadiation Treatment, Is Associated withIncreased OSThere was a statistical trend toward worseOS among patients receiving any form ofadjuvant radiation treatment with surgicalresection vs. those receiving surgery alone(log-rank, P ¼ .08). When analyzing extentof resection, patients receiving GTRdemonstrated extended OS compared withthose receiving STR with any form ofadjuvant radiation (log-rank, P < .05)(Figure 5); unlike the study by Ghia et al.,which reported that patients undergoingSTR with postoperative EBRT comparedfavorably with those undergoing GTRalone with respect to OS (17).The median survival advantage was 20.4

years for patients receiving GTR comparedwith 9.3 years for patients receiving STRwith adjuvant radiation. We found no sig-nificant difference in OS among patientsreceiving GTR with adjuvant radiation vs.patients receivingGTR alone (log-rank, P¼.315) or in patients receiving STR withadjuvant radiation vs. patients receivingSTR alone (log-rank, P ¼ .631). There wasno evidence that extent of resection oradjuvant radiation could prevent or extendthe development of metastatic HPC (log-rank, P ¼ not significant).

al, Recurrence-Free Survival

rvival Recurrence-Free Interval

nths 117 months

nths 54 months


Tumor Size and Anatomic Location PredictRecurrenceThe maximum tumor diameter rangedfrom 2.5 cm to 12 cm. On univariate anal-ysis, we found that tumors �7 cm recurredsignificantly earlier than those <7 cm (log-rank, P< .05). Tumors>7 cm recurred at amedian time of 3 years, vs. 13.1 years fortumors <7 cm. When analyzed byanatomical location, HPCs invading thecerebral venous sinuses recurred signifi-cantly earlier than those not associated withthe sinuses (log-rank, P< .05). Tumors thatinvaded dural sinuses recurred at a mediantime of 5 years, vs. 10.6 years for tumors notinvading the sinuses.

DISCUSSION

Few cases of HPC have been described inthe literature, and fewer still in the formatof a surgical series examining a suitablenumber of cases with satisfactory follow-up. Statistical analysis of our series pro-vides significant insights, some of whichconcur with data previously published inthe literature, and others that diverge fromthem.In our series, the median age was 46.9

years, with a range of 19 to 79 years,somewhat older than that reported byother investigators (18, 25). With regard toa predilection to one sex or the other,previous studies have reported a malepreponderance in the male-to-female ratioof 1.4:1 (18, 25, 26), whereas our seriesshows the opposite, with a male-to-femaleratio of 2:3. The presenting symptoms inour series mirror those already reported,particularly a median duration of clinicalhistory of 11.5 months, compatible with atumor characterized by relatively slowevolution. Examination findings mimicthose of patients harboring meningiomas,with motor deficits seen in perirolandiclesions and general or partial seizures andheadache seen in the majority of cases.



Figure 3. Recurrence rate in patients undergoing postsurgical externalbeam radiotherapy (Series 1) and in patients not receiving radiationtreatment (Series 2).

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An estimation of the relative incidence ofmeningeal HPC with respect to meningi-oma can be estimated by retrospectivelycomparing the number of meningiomastreated at our institution during the studytime period with the 43 cases of HPCdescribed here. The resultantfigure of 3.0%is higher than previous suggestions of 1HPC for every 40 to 60meningiomas (1, 3, 5,6, 10, 13, 18, 20, 21, 31).Two tumor features were shown to be

important in univariate analysis whenconsidering recurrence of HPC, namelysize �7 cm and dural sinuses invasion.The influence of tumor size on recurrenceis likely multifactorial. Larger tumors aremore difficult to resect due to their largermass and difficulty obtaining adequateexposure, increasing the likelihood of re-sidual tumor and thus recurrence. Larger

Figure 4. STR with adjuvant radiation (Serecurrence compared with GTR alone (SeSTR, subtotal resection.


tumors also are more likely to infiltratenerves, vessels, and critical neuroanatom-ical areas. They may grow near to radio-sensitive structures, decreasing theefficacy of surgical and/or radiation treat-ments (28).More interesting appears to be the role of

extent of surgical resection, which was al-ways defined objectively on postsurgicalneuroimaging with the purpose of mini-mizing the variability in judgment of theoperators. Our parameters defined totalsurgical resection as no sign of residualdisease on postoperative magnetic reso-nance imaging or computed tomography. Anumber of studies have compared out-comes after incomplete and complete tu-mor resection (12, 15, 19, 21, 23, 33). Thosestudies agree that complete excision of thetumor is the most important factor for

ries 2) extends time untilries 1). GTR, gross total resection;


reducing the likelihood of recurrences andincreasing the likelihood of survival.We obtained a total surgical removal in

30 of 43 cases (69.76%), comparing favor-ably with previous surgical series that re-ported total excision rates of 25% to 70%(13). These excellent results are probablydue to the use of intraoperative technolo-gies directed toward minimizing surgicalrisk, such as the operating microscopeand neuronavigation systems. These tech-niques allow an optimal resection whileminimizing the risk of postoperative defi-cits or death. This is of the utmost impor-tance given that extent of resection has beenwell established as a principal prognosticfactor in HPC (13, 18, 26, 34).Other goals of surgical treatment are the

improvement of quality of life and theextension of the interval free from recur-rence, and such objectives were feasible inthe majority of patients of our series. Weobserved improvement in neurologicalstatus in 35 patients, with 8 patientsremaining unchanged. The Karnofskypresurgical mean value was 75, increasingto 85 postoperatively. We had no post-operative mortality.Available follow-up data were sufficient

to allow analysis in 36 patients. Mediantime to recurrence was 72.24 months forthe first recurrence. Pertinent literatureshows a median times to recurrence of 58months (18), and 61 months with 28 re-currences (30). Although time to recur-rence does not seem to be correlated toincreased median survival, it of courseaffects the quality of life of each patient. Ina recent study, median time until recur-rence was 5 years, with 1-year, 5-year, and10-year progression-free survival rates of96%, 49%, and 28%, respectively (28).Other data reported survival of 61% at

15 years fromfirst operation (36),whereas at 5years from the first operation, survival variesbetween 67% to 93%depending on the series(8). In comparison to these data, our studywould confirm the suggestion of some au-thors (13) that good surgical technique con-fers a significant survival advantage.The poor prognosis associated with

intracranial HPC has led several in-vestigators to advocate aggressive treat-ment combining radical surgical resectionwith adjuvant radiotherapy (2). Accordingto Chang et al. (11), resection remains theinitial treatment of choice for HPC becausehistopathological diagnosis is required to

dx.doi.org/10.1016/j.wneu.2013.11.009



Figure 5. GTR alone (Series 2) appears to extend overall survival compared with STR plus adjuvantradiation (Series 1). GTR, gross total resection; STR, subtotal resection.

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distinguish them from meningiomas. Onceresection is achieved, however, they foundthat the high rates of HPC recurrence maybe addressed by performing stereotacticradiosurgery, which has been shown toprovide significant benefit.Our data support those reported by

Guthrie et al. showing that radiation ther-apy may be able to prolong the time beforefirst recurrence (18). However, as otherauthors have found (17, 29), we did not findthat the addition of postoperative adjuvantradiation confers a survival benefit.Finally, the outcomes reported herein

serve to confirm the validity of the histolog-ical parameters for anaplastic HPC definedby Mena et al. (26). In our series, grade III(anaplastic) HPC recurred in 90% of cases,whereas recurrence was only seen in 27% oflow-gradeHPCs. Thisfindingunderlines thereliability of histological prognosis.

CONCLUSIONS

The primary aim of the treatment ofHPC patients is to prolong survival andto improve quality of life. Strategiescombining adjuvant radiation with tumorresection appeared to hinder tumor pro-gression, but had no effect on OS or thedevelopment of metastasis. Greater extentof resection was associated with increasedOS. Anaplastic HPC was associated withreduced OS and with reduced time torecurrence. Long-term clinical and radio-graphic follow-up of these patients isimperative, as recurrence and/or metasta-ses often take several years to develop.Tumors �7 cm recurred significantly


earlier than those <7 cm. When analyzedby anatomic location, HPCs invading thecerebral dural sinuses recurred signifi-cantly earlier than those not associatedwith the sinuses.

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Conflict of interest statement: The authors declare that thearticle content was composed in the absence of anycommercial or financial relationships that could be construedas a potential conflict of interest. This research is supportedby the University of Rome Sapienza as part of a doctoralresearch program.

Received 18 November 2012; accepted 6 November 2013;published online 13 November 2013




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