Journal of Neuro-Oncology

, Volume 138, Issue 3, pp 447–467 | Cite as

Surveillance for metastatic hemangiopericytoma-solitary fibrous tumors-systematic literature review on incidence, predictors and diagnosis of extra-cranial disease

  • Tarini Ratneswaren
  • Florence Rosie Avila Hogg
  • Mathew Joseph Gallagher
  • Keyoumars Ashkan
Topic Review



Intracranial hemangiopericytomas (HPC) and solitary fibrous tumors (SFTs) (HPC-SFT) are rare vascular tumors that resemble meningioma on imaging and predominantly affect young adults. HPC-SFT have a high rate of local recurrence with well-known propensity for extracranial metastases. This provides clinical dilemmas frequently encountered in oncology: (i) How should these patients be monitored long term? (ii) Which primary tumors are more likely to metastasize?


This systematic review aims to identify the incidence, common locations and time to presentation of extra-cranial metastases of HPC-SFT. We will assess the effect of primary tumor location, treatment, grade, patient age, gender and effect of local recurrence on rates of extra-cranial metastasis and discuss the ideal techniques by which patients with intracranial HPC-SFT should be monitored for extra-cranial metastases.


Using PRISMA guidelines the authors searched Pubmed. Search terms included hemangiopericytoma, HPC, solitary fibrous tumor/ tumour, SFT, HPC-SFT, extra-cranial metastases, metastases, recurrence, monitoring, follow-up. Studies were identified up to 1st February 2018. Reference lists of identified articles were reviewed to detect other relevant citations. Data were extracted using a standard data collection form and results organized into (i) general study/patient characteristics, (ii) location of extra-cranial metastases, (iii) methods by which metastases were detected and followed up and (iv) characteristics of primary tumors.


Seventy-one studies were identified. Mean recorded follow up ranged from 4 to 312 months. Mean age at diagnosis was 42.0 years. The overall rate of extra-cranial metastasis was 28% (n = 251/904). The minimum time to extracranial metastases was 3 months and the maximum time was 372 months. In the 71 studies identified, where site of extra-cranial metastasis was specified, there were 347 metastases in 213 patients. The most common sites for metastases were bone (location not specified) (19.6%) followed by lung and pleura (18.4%), liver (17.6%), and vertebrae (14.1%). Extra-cranial metastatic disease is typically diagnosed following symptomatic presentation. There is little documentation of methods used to monitor patients with extra-cranial HPC-SFT and no clear surveillance paradigm observed. Higher primary tumor grade (WHO Grade III) was associated with a 1.88 (p = 0.016) increased risk of extra-cranial metastasis. Location and treatment of primary tumor, local recurrence, patient age and gender were not.


Patients with intracranial HPC-SFT require periodic, long term monitoring for extra-cranial metastases. Metastases occur in any age group and can occur early and late. They vary in location and are typically diagnosed following symptomatic presentation. There is no suggested imaging modality for surveillance. Higher grade primary tumors have a greater risk of metastasis. Regular clinical review is essential with early imaging for symptoms of recurrence/metastasis with imaging modality dependent on clinical concern. Quality evidence for an imaging surveillance protocol in this heterogeneous group of patients is lacking. A multicenter study on appropriate surveillance may be of benefit.


Hemangiopericytoma Solitary fibrous tumor Extra-cranial metastases Monitoring HPC SFT 


Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest. The authors alone are responsible for the content and writing of the paper.


  1. 1.
    Stout AP, Murray MR (1942) Hemangiopericytoma: a vascular tumor featuring zimmermann’s pericytes. Ann Surg 116:26–33CrossRefPubMedPubMedCentralGoogle Scholar
  2. 2.
    Enzinger FM, Smith BH (1976) Hemangiopericytoma. An analysis of 106 cases. Hum Pathol 7:61–82CrossRefPubMedGoogle Scholar
  3. 3.
    Guthrie BL, Ebersold MJ, Scheithauer BW, Shaw EG (1989) Meningeal hemangiopericytoma: histopathological features, treatment, and long-term follow-up of 44 cases. Neurosurgery 25:514–522CrossRefPubMedGoogle Scholar
  4. 4.
    Rutkowski MJ et al (2012) Intracranial hemangiopericytoma: clinical experience and treatment considerations in a modern series of 40 adult patients. Cancer 118:1628–1636. CrossRefPubMedGoogle Scholar
  5. 5.
    Melone AG et al (2014) Intracranial hemangiopericytoma–our experience in 30 years: a series of 43 cases and review of the literature. World Neurosurg 81:556–562. CrossRefPubMedGoogle Scholar
  6. 6.
    Louis DN et al (2016) The 2016 World Health Organization classification of tumors of the central nervous system: a summary. Acta Neuropathol 131:803–820. CrossRefPubMedGoogle Scholar
  7. 7.
    Bouvier C et al (2012) Solitary fibrous tumors and hemangiopericytomas of the meninges: overlapping pathological features and common prognostic factors suggest the same spectrum of tumors. Brain Pathol 22:511–521CrossRefPubMedGoogle Scholar
  8. 8.
    Schweizer L et al (2013) Meningeal hemangiopericytoma and solitary fibrous tumors carry the NAB2-STAT6 fusion and can be diagnosed by nuclear expression of STAT6 protein. Acta Neuropathol 125:651–658. CrossRefPubMedGoogle Scholar
  9. 9.
    Mena H, Ribas JL, Pezeshkpour GH, Cowan DN, Parisi JE (1991) Hemangiopericytoma of the central nervous system: a review of 94 cases. Hum Pathol 22:84–91CrossRefPubMedGoogle Scholar
  10. 10.
    Kumar N et al (2012) Intracranial meningeal hemangiopericytoma: 10 years experience of a tertiary care Institute. Acta Neurochir 154:1647–1651. CrossRefPubMedGoogle Scholar
  11. 11.
    Sheehan J, Kondziolka D, Flickinger J, Lunsford LD (2002) Radiosurgery for treatment of recurrent intracranial hemangiopericytomas. Neurosurgery 51:905–910 (discussion 910–901)PubMedGoogle Scholar
  12. 12.
    Galanis E et al (1998) Management of recurrent meningeal hemangiopericytoma. Cancer 82:1915–1920CrossRefPubMedGoogle Scholar
  13. 13.
    Ramakrishna R, Rostomily R, Sekhar L, Rockhill J, Ferreira M (2014) Hemangiopericytoma radical resection remains the cornerstone of therapy. J Clin Neurosci 21:612–615. CrossRefPubMedGoogle Scholar
  14. 14.
    Zhou JL, Liu JL, Zhang J, Zhang M (2012) Thirty-nine cases of intracranial hemangiopericytoma and anaplastic hemangiopericytoma: a retrospective review of MRI features and pathological findings. Eur J Radiol 81:3504–3510. CrossRefPubMedGoogle Scholar
  15. 15.
    Anderson C, Rorabeck CH (1980) Skeletal metastases of an intracranial malignant hemangiopericytoma. Report of a case. J Bone Joint Surg Am 62:145–148CrossRefPubMedGoogle Scholar
  16. 16.
    Schiariti M, Goetz P, El-Maghraby H, Tailor J, Kitchen N (2011) Hemangiopericytoma: long-term outcome revisited. Clinical article. J Neurosurg 114:747–755. CrossRefPubMedGoogle Scholar
  17. 17.
    Suzuki H et al (2002) Intracranial hemangiopericytoma with extracranial metastasis occurring after 22 years. Neurol Medico-chir 42:297–300CrossRefGoogle Scholar
  18. 18.
    Hiraide T et al (2014) Pancreatic metastases of cerebellar hemangiopericytoma occurring 24 years after initial presentation: report of a case. Surg Today 44:558–563. CrossRefPubMedGoogle Scholar
  19. 19.
    Fountas KN et al (2006) Management of intracranial meningeal hemangiopericytomas: outcome and experience. Neurosurg Rev 29:145–153. CrossRefPubMedGoogle Scholar
  20. 20.
    Ambrosini-Spaltro A, Eusebi V (2010) Meningeal hemangiopericytomas and hemangiopericytoma/solitary fibrous tumors of extracranial soft tissues: a comparison. Virchows Arch 456:343–354. CrossRefPubMedGoogle Scholar
  21. 21.
    Tanabe S et al (1984) A case report of pancreatic metastasis of an intracranial angioblastic meningioma (hemangiopericytoma) and a review of metastatic tumor to the pancreas. J Surg Oncol 26:63–68CrossRefPubMedGoogle Scholar
  22. 22.
    Teh BS, Lu HH, Jhala DN, Shahab I, Lynch GR (2000) Pancreatic head mass from metastatic meningeal hemangiopericytoma. Sarcoma 4:169–172. CrossRefPubMedPubMedCentralGoogle Scholar
  23. 23.
    Iwamuro M et al (2009) A case of primary intracranial hemangiopericytoma with hepatic metastases: successful treatment with radiofrequency ablation and transcatheter arterial chemoembolization. Clin J Gastroenterol 2:30–35. CrossRefPubMedGoogle Scholar
  24. 24.
    Sun S, Liu A, Wang C (2009) Gamma knife radiosurgery for recurrent and residual meningeal hemangiopericytomas. Stereot Funct Neurosurg 87:114–119. CrossRefGoogle Scholar
  25. 25.
    Nickerson TP, Fahy AS, Bingener J (2015) Laparoscopic resection of intra-abdominal metastasis from intracranial hemangiopericytoma. Int J Surg Case Rep. PubMedPubMedCentralCrossRefGoogle Scholar
  26. 26.
    Eil R, Lu KC, Wettach GR, Tsikitis VL (2012) Intracranial hemangiopericytoma focally recurrent to the pelvis. J Cancer Therapy 3:487CrossRefGoogle Scholar
  27. 27.
    Chang CC, Chang YY, Lui CC, Huang CC, Liu JS (2004) Meningeal hemangiopericytoma with delayed multiple distant metastases. J Chin Med Assoc 67:527–532PubMedGoogle Scholar
  28. 28.
    Wei G et al (2015) Intracranial meningeal hemangiopericytoma: recurrences at the initial and distant intracranial sites and extraneural metastases to multiple organs. Mol Clin Oncol 3:770–774. CrossRefPubMedPubMedCentralGoogle Scholar
  29. 29.
    Chan WS, Zhang J, Khong PL (2010) 18F-FDG-PET-CT imaging findings of recurrent intracranial haemangiopericytoma with distant metastases. Br J Radiol 83:e172–e174. CrossRefPubMedPubMedCentralGoogle Scholar
  30. 30.
    Nair V et al. (2010) Meningeal hemangiopericytoma with delayed extra-neuraxial metastases: diagnostic conundrum and management using high-precision simultaneous multi-target irradiation on helical tomotherapy.Google Scholar
  31. 31.
    Jääskeläinen J, Servo A, Haltia M, Wahlström T, Valtonen S (1985) Intracranial hemangiopericytoma: radiology, surgery, radiotherapy, and outcome in 21 patients. Surg Neurol 23:227–236. CrossRefPubMedGoogle Scholar
  32. 32.
    Olson C, Yen CP, Schlesinger D, Sheehan J (2010) Radiosurgery for intracranial hemangiopericytomas: outcomes after initial and repeat Gamma Knife surgery. J Neurosurg 112:133–139. CrossRefPubMedGoogle Scholar
  33. 33.
    Chang SD, Sakamoto GT (2003) The role of radiosurgery for hemangiopericytomas. Neurosurg Focus 14:e14CrossRefPubMedGoogle Scholar
  34. 34.
    Kano H, Niranjan A, Kondziolka D, Flickinger JC, Lunsford LD (2008) Adjuvant stereotactic radiosurgery after resection of intracranial hemangiopericytomas. Int J Rad Oncol Biol Phys 72:1333–1339. CrossRefGoogle Scholar
  35. 35.
    Ecker RD et al (2003) Hemangiopericytoma in the central nervous system: treatment, pathological features, and long-term follow up in 38 patients. J Neurosurg 98:1182–1187. CrossRefPubMedGoogle Scholar
  36. 36.
    Kim JH et al (2003) Meningeal hemangiopericytomas: long-term outcome and biological behavior. Surg Neurol 59:47–53 (discussion 53–44)CrossRefPubMedGoogle Scholar
  37. 37.
    Hara M et al (1998) Recurrence in meningeal hemangiopericytomas. Surg Neurol 50:586–591. CrossRefPubMedGoogle Scholar
  38. 38.
    Chen L-f et al (2015) Multimodal treatment and management strategies for intracranial hemangiopericytoma. J Clin Neurosci 22:718–725. CrossRefPubMedGoogle Scholar
  39. 39.
    Damodaran O et al (2014) Primary intracranial haemangiopericytoma: comparison of survival outcomes and metastatic potential in WHO grade II and III variants. J Clin Neurosc 21:1310–1314. CrossRefGoogle Scholar
  40. 40.
    Soyuer S, Chang EL, Selek U, McCutcheon IE, Maor MH (2004) Intracranial meningeal hemangiopericytoma: the role of radiotherapy: report of 29 cases and review of the literature. Cancer 100:1491–1497. CrossRefPubMedGoogle Scholar
  41. 41.
    Dufour H et al (2001) Meningeal hemangiopericytoma: a retrospective study of 21 patients with special review of postoperative external radiotherapy. Neurosurgery 48:756–762 (discussion 762–753)PubMedGoogle Scholar
  42. 42.
    Fukuda Y, Watanabe K, Toyama Y, Mikami S, Matsumoto M (2015) Metastasis of intracranial meningeal hemangiopericytoma to thoracic spine 17 years after surgical excision: a case report. J Orthop Sci 20:425–429. CrossRefPubMedGoogle Scholar
  43. 43.
    Noh SH, Lim JJ, Cho KG (2015) Intracranial hemangiopericytomas: a retrospective study of 15 patients with a special review of recurrence. J Korean Neurosurg Soc 58:211–216CrossRefPubMedPubMedCentralGoogle Scholar
  44. 44.
    Begum M, Katabuchi H, Tashiro H, Suenaga Y, Okamura H (2002) A case of metastatic malignant hemangiopericytoma of the ovary: recurrence after a period of 17 years from intracranial tumor. Int J Gynecol Cancer 12:510–514CrossRefPubMedGoogle Scholar
  45. 45.
    Chan JKI, Cheuk W, Ho LC, Wen J-M (2012) Recurrent meningeal hemangiopericytoma with multiple metastasis and hypoglycemia: a case report. Case Rep Med 2012:628756PubMedPubMedCentralGoogle Scholar
  46. 46.
    Cao Y et al (2006) Recurrent intracranial hemangiopericytoma with multiple metastases. Chin Med J Beijing Engl Ed 119:169Google Scholar
  47. 47.
    Spatola C, Privitera G (2004) Recurrent intracranial hemangiopericytoma with extracranial and unusual multiple metastases: case report and review of the literature. Tumori 90:265–268CrossRefPubMedGoogle Scholar
  48. 48.
    Chacko G, Chacko AG, Rajshekhar V, Muliyil JP (2006) Intracranial hemangiopericytomas: correlation of topoisomerase IIalpha expression with biologic behavior. Surg Neurol 65:11–17. CrossRefPubMedGoogle Scholar
  49. 49.
    Heiser MA, Waldron JS, Tihan T, Parsa AT, Cheung SW (2009) Temporal fossa hemangiopericytoma: a case series. Otol Neurotol 30:985–989. CrossRefPubMedGoogle Scholar
  50. 50.
    Yesilkaya Y, Topcuoglu AD, Tanas M, Altundag OK (2012) Primary intracranial hemangiopericytoma with mesenteric and retroperitoneal spread. Biomed Imaging Interv J 8(4):e24Google Scholar
  51. 51.
    Pistolesi S et al (2004) Meningeal hemangiopericytoma metastatic to the adrenal gland with multiple metastases to bones and lungs: a case report. Tumori 90:147–150CrossRefPubMedGoogle Scholar
  52. 52.
    Grunenberger F et al (1999) Hepatic and pulmonary metastases from a meningeal hemangiopericytoma and severe hypoglycemia due to abnormal secretion of insulin-like growth factor: a case report. Cancer 85:2245–2248CrossRefPubMedGoogle Scholar
  53. 53.
    Nonaka M, Kohmura E, Hirata M, Hayakawa T (1998) Metastatic meningeal hemangiopericytoma of thoracic spine. Clin Neurol Neurosurg 100:228–230CrossRefPubMedGoogle Scholar
  54. 54.
    Siegel HJ, Lopez-Ben R, Sutton JH, Siegal GP (2012) Intracranial meningeal hemangiopericytoma metastatic to the scapula. Orthopedics 35:e112–e115. PubMedCrossRefGoogle Scholar
  55. 55.
    Satayasoontorn K et al (2014) Meningeal hemangiopericytoma only diagnosed at the time of late bone metastasis. Skelet Radiol 43:1543–1549. CrossRefGoogle Scholar
  56. 56.
    Woitzik J, Sommer C, Krauss JK (2003) Delayed manifestation of spinal metastasis: a special feature of hemangiopericytoma. Clin Neurol Neurosurg 105:159–166CrossRefPubMedGoogle Scholar
  57. 57.
    Lee JK et al (2006) Spinal metastasis from cranial meningeal hemangiopericytomas. Acta Neurochirurg 148:787–790. CrossRefGoogle Scholar
  58. 58.
    Taniura S, Taniguchi M, Mizutani T, Takahashi H (2007) Metastatic hemangiopericytoma to the cauda equina: a case report. Spine J 7:371–373. CrossRefPubMedGoogle Scholar
  59. 59.
    Kim BS, Kong D-S, Seol HJ, Nam D-H, Lee J-I (2016) Gamma knife radiosurgery for residual or recurrent intracranial hemangiopericytomas. J Clin Neurosci. CrossRefPubMedGoogle Scholar
  60. 60.
    Lo RC, Suriawinata AA, Rubin BP (2016) Liver metastasis of meningeal hemangiopericytoma: a study of 5 cases. Clin Mol Hepatol 22:188–191. CrossRefPubMedPubMedCentralGoogle Scholar
  61. 61.
    Purandare NC et al (2010) Metastatic recurrence of an intracranial hemangiopericytoma 8 years after treatment: report of a case with emphasis on the role of PET/CT in follow-up. Cancer Imaging 10:117–120. CrossRefPubMedPubMedCentralGoogle Scholar
  62. 62.
    Manatakis DK, Delis SG, Ptohis N, Korkolopoulou P, Dervenis C (2015) Multidisciplinary approach to hepatic metastases of intracranial hemangiopericytoma: a case report and review of the literature. Case Rep Oncol Med 2015:214–306. CrossRefGoogle Scholar
  63. 63.
    De Martin E, Coilly A, Guettier C, Samuel D (2015) Liver metastases from meningeal hemangiopericytoma. Liver Int 35:2337. CrossRefPubMedGoogle Scholar
  64. 64.
    Doxtader EE, Mukhopadhyay S, Prayson RA (2015) Solitary lung metastasis from intracranial hemangiopericytoma 18 years after initial resection. J Clin Neurosci 22:1210–1212. CrossRefPubMedGoogle Scholar
  65. 65.
    Nakada S et al (2015) NAB2–STAT6 fusion gene analysis in two cases of meningeal solitary fibrous tumor/hemangiopericytoma with late distant metastases. Brain Tumor Pathol 32:268–274. CrossRefPubMedGoogle Scholar
  66. 66.
    Ramos LR, Marques PP, Loureiro R, Brito MJ, de Freitas J (2014) Pancreatic metastasis of a meningeal hemangiopericytoma: a rare cause of obstructive jaundice. Endoscopy 46(S 01):E135–E136. CrossRefPubMedGoogle Scholar
  67. 67.
    Fabbri A, Grifoni E, Ciuti G, Fedi R, Moggi Pignone A (2014) Central nervous system hemangiopericytoma with bone and lung metastases: a case report. Intern Emerg Med 9:349–350. CrossRefPubMedGoogle Scholar
  68. 68.
    Delgado M et al (2011) Anti-angiogenic treatment (sunitinib) for disseminated malignant haemangiopericytoma: a case study and review of the literature. Case Rep Oncol 4:55–59. CrossRefPubMedPubMedCentralGoogle Scholar
  69. 69.
    Han N et al (2016) Meningeal solitary fibrous tumors with delayed extracranial metastasis. J Pathol Trans Med 50:113CrossRefGoogle Scholar
  70. 70.
    Degnan AJ, Lee KK, Minervini MI, Borhani AA (2017) Metastatic extrapleural malignant solitary fibrous tumor presenting with hypoglycemia (Doege-Potter syndrome). Radiol Case Rep 12:113–119. CrossRefPubMedGoogle Scholar
  71. 71.
    Kim KA, Gonzalez I, McComb JG, Giannotta SL (2004) Unusual presentations of cerebral solitary fibrous tumors: report of four cases. Neurosurgery 54:1004–1009CrossRefPubMedGoogle Scholar
  72. 72.
    Wu Z, Yang H, Weng D, Ding Y (2015) Rapid recurrence and bilateral lungs, multiple bone metastasis of malignant solitary fibrous tumor of the right occipital lobe: report of a case and review. Diagn Pathol 10:91. CrossRefPubMedPubMedCentralGoogle Scholar
  73. 73.
    Ng HK, Choi PC, Wong CW, To KF, Poon WS (2000) Metastatic solitary fibrous tumor of the meninges. Case report. J Neurosurg 93:490–493. CrossRefPubMedGoogle Scholar
  74. 74.
    Ogawa K et al (2004) Malignant solitary fibrous tumor of the meninges. Virchows Arch 444:459–464. CrossRefPubMedGoogle Scholar
  75. 75.
    Gessi M et al (2013) Extracranial metastasizing solitary fibrous tumors (SFT) of meninges: histopathological features of a case with long-term follow-up. Neuropathology 33:68–74. CrossRefPubMedGoogle Scholar
  76. 76.
    Cohen-Inbar O et al (2017) Stereotactic radiosurgery for intracranial hemangiopericytomas: a multicenter study. J Neurosurg 126:744–754. CrossRefPubMedGoogle Scholar
  77. 77.
    Soyuer S, Chang EL, Selek U, McCutcheon IE, Maor MH (2004) Intracranial meningeal hemangiopericytoma: the role of radiotherapy. Cancer 100:1491–1497. CrossRefPubMedGoogle Scholar
  78. 78.
    Greenberg DD, Crawford B (2016) Surveillance strategies for sarcoma: results of a survey of members of the musculoskeletal tumor society. Sarcoma 2016:8289509CrossRefPubMedPubMedCentralGoogle Scholar
  79. 79.
    Huang B, Law MW, Khong PL (2009) Whole-body PET/CT scanning: estimation of radiation dose and cancer risk. Radiology 251:166–174. CrossRefPubMedGoogle Scholar
  80. 80.
    Brenner DJ, Elliston CD (2004) Estimated radiation risks potentially associated with full-body CT screening. Radiology 232:735–738. CrossRefPubMedGoogle Scholar
  81. 81.
    Schmidt GP, Reiser MF, Baur-Melnyk A (2007) Whole-body imaging of the musculoskeletal system: the value of MR imaging. Skelet Radiol 36:1109–1119. CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Radiology, Addenbrooke’s HospitalCambridge University Hospitals NHS TrustCambridgeUK
  2. 2.Department of Neurosurgery, King’s College HospitalKing’s College Hospital NHS Foundation TrustLondonUK
  3. 3.Academic Neurosurgery UnitSt. George’s University of LondonLondonUK

Personalised recommendations