Hemangiopericytoma: long-term outcome revisited

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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.


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.


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.


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.

Abbreviations used in this paper: EBRT = external beam radiation therapy; GKS = Gamma Knife surgery; GTR = gross-total resection; OS = overall survival.

Article Information

Address correspondence to: Marco Schiariti, M.D., Department of Neurosurgery, Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena, Via Francesco Sforza, 35 – 20100, Milan, Italy. email: mp_schiariti@hotmail.com.

Please include this information when citing this paper: published online July 30, 2010; DOI: 10.3171/2010.6.JNS091660.

© AANS, except where prohibited by US copyright law.



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    Probability of recurrence (y axis) in relation to the number of months (x axis) after the first operation. Recurrence was dated at the identification of local tumor growth identified on MR imaging. Rates were calculated using the Kaplan-Meier method. The average period before the first recurrence was 80 months (median 61 months).

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    Probability of developing metastases in relation to the number of months after the first operation for the primary tumor. Rates were calculated using the Kaplan-Meier method. Nine patients suffered metastases at an average of 123 months after initial surgery.

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    Probability of survival in relation to the number of months after the first operation. Rates were calculated with the Kaplan-Meier method. Tumor-related deaths were used as end points. The average OS was 216 months.

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    Probability of survival in patients with low- and high-grade tumors in relation to the number of months after the first operation. Rates were calculated with the Kaplan-Meier method. The average survival was 142 and 256 months in the low- and high-grade tumor groups, respectively (a difference of 114 months; p = 0.03).

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    Probability of recurrence in patients with complete and incomplete excision in relation to the number of months after the first operation. Rates were calculated using the Kaplan-Meier method. The average period before the first recurrence was 117 and 54 months in the complete and incomplete resection groups, respectively (p = 0.0045).

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    Probability of survival in patients with and without radiotherapy (EBRT) in relation to the number of months after the first operation. Rates were calculated using the Kaplan-Meier method. The average length of survival was 254 and 154 months in the EBRT and non-EBRT groups, respectively (p = 0.2).

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    Probability of recurrence in patients who underwent EBRT adjusting for surgical excision. Rates were calculated using Cox regression analysis (p = 0.03).



Adegbite ABKhan MIPaine KWETan LK: The recurrence of intracranial meningiomas after surgical treatment. J Neurosurg 58:51561983


Alén JFLobato RDGómez PABoto GRLagares ARamos A: Intracranial hemangiopericytoma: study of 12 cases. Acta Neurochir (Wien) 143:5755862001


Bastin KTMehta MP: Meningeal hemangiopericytoma: defining the role for radiation therapy. J Neurooncol 14:2772871992


Brunori ADelitala AOddi GChiappetta F: Recent experience in the management of meningeal hemangiopericytomas. Tumori 83:8568611997


Chang SDSakamoto GT: The role of radiosurgery for hemangiopericytomas. Neurosurg Focus 14:5Article 142003


Coffey RJCascino TLShaw EG: Radiosurgical treatment of recurrent hemangiopericytomas of the meninges: preliminary results. J Neurosurg 78:9039081993


Cox DR: Regression models and life tables. J R Stat Soc Ser B 34:1872201972


Cushing HEisenhardt L: Meningiomas: Their Classification Regional Behavior Life History and Surgical End Results Springfield, ILCharles C Thomas1938


Dardick IHammar SPScheithauer BW: Ultrastructural spectrum of hemangiopericytoma: a comparative study of fetal, adult, and neoplastic pericytes. Ultrastruct Pathol 13:1111541989


Dufour HMétellus PFuentes SMurracciole XRégis JFigarella-Branger D: Meningeal hemangiopericytoma: a retrospective study of 21 patients with special review of postoperative external radiotherapy. Neurosurgery 48:7567632001


Ecker RDMarsh WRPollock BEKurtkaya-Yapicier OMc-Clelland RScheithauer BW: Hemangiopericytoma in the central nervous system: treatment, pathological features, and long-term follow up in 38 patients. J Neurosurg 98:118211872003


Galanis EBuckner JCScheithauer BWKimmel DWSchomberg PJPiepgras DG: Management of recurrent meningeal hemangiopericytoma. Cancer 82:191519201998


Guthrie BLEbersold MJScheithauer BWShaw EG: Meningeal hemangiopericytoma: histopathological features, treatment, and long-term follow-up of 44 cases. Neurosurgery 25:5145221989


Habermann CRNicolas VSteiner P: [Magnetic resonance tomography diagnosis of plexiform fibrohistiocytic tumor.]. Aktuelle Radiol 5:2432451995. (Ger)


Henn WWullich BThönnes MSteudel WIFeiden WZang KD: Recurrent t(12;19)(q13;q13.3) in intracranial and extracranial hemangiopericytoma. Cancer Genet Cytogenet 71:1511541993


Jääskeläinen JServo AHaltia MWahlström TValtonen S: Intracranial hemangiopericytoma: radiology, surgery, radiotherapy, and outcome in 21 patients. Surg Neurol 23:2272361985


Joseph JTLisle DKJacoby LBPaulus WBarone RCohen ML: NF2 gene analysis distinguishes hemangiopericytoma from meningioma. Am J Pathol 147:145014551995


Kaplan ELMeier P: Nonparametric estimation from incomplete observations. J Am Stat Assoc 53:4574811958


Kim JHJung HWKim YSKim CJHwang SKPaek SH: Meningeal hemangiopericytomas: long-term outcome and biological behavior. Surg Neurol 59:47542003


Kleihues PBurger PCScheithauer BW: Tumours of the meninges. Histological Typing of Tumours of the Central Nervous System ed 2BerlinSpringer-Verlag1993. 3341


Mena HRibas JLPezeshkpour GHCowan DNParisi JE: Hemangiopericytoma of the central nervous system: a review of 94 cases. Hum Pathol 22:84911991


Ramsey HJ: Fine structure of hemangiopericytoma and hemangio-endothelioma. Cancer 19:200520181966


Sheehan JKondziolka DFlickinger JLunsford LD: Radiosurgery for treatment of recurrent intracranial hemangiopericytomas. Neurosurgery 51:9059112002


Someya MSakata KIOouchi ANagakura HSatoh MHareyama M: Four cases of meningeal hemangiopericytoma treated with surgery and radiotherapy. Jpn J Clin Oncol 31:5485522001


Soyuer SChang ELSelek UMcCutcheon IEMaor MH: Intracranial meningeal hemangiopericytoma: the role of radiotherapy: report of 29 cases and review of the literature. Cancer 100:149114972004


Staples JJRobinson RAWen BCHussey DH: Hemangiopericytoma—the role of radiotherapy. Int J Radiat Oncol Biol Phys 19:4454511990


Stout APMurray MR: Hemangiopericytoma: a vascular tumor featuring Zimmerman's pericytes. Ann Surg 116:26331942


Tsou HWang YYang DWei S: Intra-extracranial hemangiopericytoma: clinical manifestations, histopathological features, diagnosis, treatment, and outcomes. Chin Med J (Taipei) 65:3143192002




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