Skip to main content
SpringerLink
Log in

Orbit Solitary Fibrous Tumor: A Proposed Risk Prediction Model Based on a Case Series and Comprehensive Literature Review

  • Original Paper
  • Published:
Head and Neck Pathology Aims and scope Submit manuscript

Abstract

Solitary fibrous tumors (SFTs) of the orbit are rare. In order to further characterize the clinical and pathologic features of solitary fibrous tumor arising at this anatomic site, 12 cases of orbital SFTs were analyzed in conjunction with a review of 263 cases reported from the English literature in order to develop a risk prediction model. SFTs of the orbit were equally distributed between males (n = 5) and females (n = 7) with a mean patient age of 46.8 years (median 44.5 years; range 18–76 years) at initial diagnosis. The patients typically presented with swelling or mass around the orbit, with proptosis (n = 10), ptosis (n = 5), and visual changes (n = 6). Tumors were orbital (n = 10) or upper eyelid (n = 2). Mean tumor size was 2.5 cm (median 2.6 cm). Microscopically, the tumors were characterized by cytologically bland spindle cells with patternless growth, hypocellular and hypercellular areas, variable amounts of collagen, and ectatic, branching blood vessels. By immunohistochemistry, all cases had a strong nuclear STAT6 expression. All patients were initially managed with excision or biopsy, three with presurgical embolization. The two patients with biopsy only had persistent disease (mean 37.2 months), but a third patient developed distant bone metastasis at 86.9 months. Overall mean follow-up was 73.1 months: 9 patients are alive or dead without disease (mean 77.9 months), two patients with persistent disease, and one patient with metastatic disease at last follow-up (102 months). Incorporating cases sufficiently reported in the literature, a risk prediction model based on age > 45 years, tumor size > 3 cm, tumor necrosis, mitoses of > 4/2 mm2, moderate to high cellularity, and moderate to severe pleomorphism allows for risk stratification for the development of local recurrence and distant metastasis. In conclusion, orbital SFTs are rare, but can be reliably diagnosed based on the presence of characteristic morphologic features and STAT6 immunohistochemistry. Orbital tumors tend to show a higher frequency of local recurrence than distant metastasis, which can be predicted by a risk stratification model unique to orbital tumors. With late disease common, long term clinical follow-up is recommended.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4

Similar content being viewed by others

References

  1. Klemperer P, Rabin CB. Primary neoplasms of the pleura: a report of five cases. Arch Pathol. 1931;11:385–412.

    Google Scholar 

  2. Stout AP, Murray MR. Hemangiopericytoma: a vascular tumor featuring Zimmermann's pericytes. Ann Surg. 1942;116(1):26.

    CAS  PubMed  PubMed Central  Google Scholar 

  3. Gengler C, Guillou L. Solitary fibrous tumour and haemangiopericytoma: evolution of a concept. Histopathology. 2006;48(1):63–74.

    CAS  PubMed  Google Scholar 

  4. Chmielecki J, Crago AM, Rosenberg M, et al. Whole-exome sequencing identifies a recurrent NAB2-STAT6 fusion in solitary fibrous tumors. Nat Genet. 2013;45(2):131–2.

    CAS  PubMed  PubMed Central  Google Scholar 

  5. Mohajeri A, Tayebwa J, Collin A, et al. Comprehensive genetic analysis identifies a pathognomonic NAB2/STAT6 fusion gene, nonrandom secondary genomic imbalances, and a characteristic gene expression profile in solitary fibrous tumor. Genes Chromosomes Cancer. 2013;52(10):873–86.

    CAS  PubMed  Google Scholar 

  6. Robinson DR, Wu YM, Kalyana-Sundaram S, et al. Identification of recurrent NAB2-STAT6 gene fusions in solitary fibrous tumor by integrative sequencing. Nat Genet. 2013;45(2):180–5.

    CAS  PubMed  PubMed Central  Google Scholar 

  7. Kao YC, Lin PC, Yen SL, et al. Clinicopathological and genetic heterogeneity of the head and neck solitary fibrous tumours: a comparative histological, immunohistochemical and molecular study of 36 cases. Histopathology. 2016;68(4):492–501.

    PubMed  Google Scholar 

  8. Demicco EG, Harms PW, Patel RM, et al. Extensive survey of STAT6 expression in a large series of mesenchymal tumors. Am J Clin Pathol. 2015;143(5):672–82.

    CAS  PubMed  PubMed Central  Google Scholar 

  9. Demicco EG, Park MS, Araujo DM, et al. Solitary fibrous tumor: a clinicopathological study of 110 cases and proposed risk assessment model. Mod Pathol. 2012;25(9):1298–306.

    PubMed  Google Scholar 

  10. Rice CD, Kersten RC, Mrak RE. An orbital hemangiopericytoma recurrent after 33 years. Arch Ophthalmol. 1989;107(4):552–6.

    CAS  PubMed  Google Scholar 

  11. Dorfman DM, To K, Dickersin GR, Rosenberg AE, Pilch BZ. Solitary fibrous tumor of the orbit. Am J Surg Pathol. 1994;18(3):281–7.

    CAS  PubMed  Google Scholar 

  12. Westra WH, Gerald WL, Rosai J. Solitary fibrous tumor. Consistent CD34 immunoreactivity and occurrence in the orbit. Am J Surg Pathol. 1994;18(10):992–8.

    CAS  PubMed  Google Scholar 

  13. Fukunaga M, Ushigome S, Nomura K, Ishikawa E. Solitary fibrous tumor of the nasal cavity and orbit. Pathol Int. 1995;45(12):952–7.

    CAS  PubMed  Google Scholar 

  14. Lucas DR, Campbell RJ, Fletcher CDM, et al. Solitary fibrous tumor of the orbit. Int J Surg Pathol. 1995;2:193–8.

    Google Scholar 

  15. DeBacker CM, Bodker F, Putterman AM, Beckmann E. Solitary fibrous tumor of the orbit. Am J Ophthalmol. 1996;121(4):447–9.

    CAS  PubMed  Google Scholar 

  16. McElvanney AM, Noble JL, O'Donovan DG, Bonshek RE, Banerjee SS. Solitary fibrous tumour: an atypical presentation within the orbit. Eye (Lond). 1996;10(Pt 3):396–9.

    Google Scholar 

  17. Ramdial PK, Nadvi S. An unusual cause of proptosis: orbital solitary fibrous tumor: case report. Neurosurgery. 1996;38(5):1040–3.

    CAS  PubMed  Google Scholar 

  18. Ruska KM, Westra WH. Pathologic quiz case 1. Solitary fibrous tumor (SFT) of the orbit. Arch Otolaryngol Head Neck Surg. 1996;122(10):1130, 2.

  19. Sciot R, Goffin J, Fossion E, Wilms G, Dom R. Solitary fibrous tumour of the orbit. Histopathology. 1996;28(2):188–91.

    CAS  PubMed  Google Scholar 

  20. Char D, Weidner N, Ahn J, Harbour JW. Solitary fibrous tumor of the orbit. Orbit. 1997;16:113–8.

    Google Scholar 

  21. Fukunaga M, Naganuma H, Nikaido T, Harada T, Ushigome S. Extrapleural solitary fibrous tumor: a report of seven cases. Mod Pathol. 1997;10(5):443–50.

    CAS  PubMed  Google Scholar 

  22. Karcioglu ZA, Nasr AM, Haik BG. Orbital hemangiopericytoma: clinical and morphologic features. Am J Ophthalmol. 1997;124(5):661–72.

    CAS  PubMed  Google Scholar 

  23. Cho NH, Kie JH, Yang WI, Jung WH. Solitary fibrous tumour with an unusual adenofibromatous feature in the lacrimal gland. Histopathology. 1998;33(3):289–90.

    CAS  PubMed  Google Scholar 

  24. Ing EB, Kennerdell JS, Olson PR, Ogino S, Rothfus WE. Solitary fibrous tumor of the orbit. Ophthalmic Plast Reconstr Surg. 1998;14(1):57–61.

    CAS  PubMed  Google Scholar 

  25. Lanuza A, Lazaro R, Salvador M, et al. Solitary fibrous tumour of the orbit. Report of a new case. Int Ophthalmol. 1998;22(5):265–8.

    CAS  PubMed  Google Scholar 

  26. Miyagi N, Sugita Y, Terasaki M, et al. Solitary fibrous tumor of the orbit: a case report. Jpn J Neurosurg. 1998;7:243–7.

    Google Scholar 

  27. de Saint Aubain Somerhausen N, Rubin BP, Fletcher CD. Myxoid solitary fibrous tumor: a study of seven cases with emphasis on differential diagnosis. Mod Pathol. 1999;12(5):463–71.

    PubMed  Google Scholar 

  28. Festa S, Lee HJ, Langer P, Klein KM. Solitary fibrous tumor of the orbit: CT and pathologic correlation. Neuroradiology. 1999;41(1):52–4.

    CAS  PubMed  Google Scholar 

  29. Hasegawa T, Matsuno Y, Shimoda T, et al. Extrathoracic solitary fibrous tumors: their histological variability and potentially aggressive behavior. Hum Pathol. 1999;30(12):1464–73.

    CAS  PubMed  Google Scholar 

  30. Hayashi N, Borodic G, Karesh JW, et al. Giant cell angiofibroma of the orbit and eyelid. Ophthalmology. 1999;106(6):1223–9.

    CAS  PubMed  Google Scholar 

  31. Kim HY, Lee SY, Kang SJ, Kim HJ. Solitary fibrous tumor of the orbit: a poorly-recognized orbital lesion. Acta Ophthalmol Scand. 1999;77(6):704–8.

    CAS  PubMed  Google Scholar 

  32. Alexandrakis G, Johnson TE. Recurrent orbital solitary fibrous tumor in a 14-year-old girl. Am J Ophthalmol. 2000;130(3):373–6.

    CAS  PubMed  Google Scholar 

  33. Guillou L, Gebhard S, Coindre JM. Orbital and extraorbital giant cell angiofibroma: a giant cell-rich variant of solitary fibrous tumor? Clinicopathologic and immunohistochemical analysis of a series in favor of a unifying concept. Am J Surg Pathol. 2000;24(7):971–9.

    CAS  PubMed  Google Scholar 

  34. Havlik DM, Farnath DA, Bocklage T. Solitary fibrous tumor of the orbit with a t(9;22)(q31;p13). Arch Pathol Lab Med. 2000;124(5):756–8.

    CAS  PubMed  Google Scholar 

  35. Carrera M, Prat J, Quintana M. Malignant solitary fibrous tumour of the orbit: report of a case with 8 years follow-up. Eye (Lond). 2001;15(Pt 1):102–4.

    CAS  Google Scholar 

  36. Fenton S, Moriarty P, Kennedy S. Solitary fibrous tumour of the orbit. Eye (Lond). 2001;15(Pt 1):124–6.

    CAS  Google Scholar 

  37. Gigantelli JW, Kincaid MC, Soparkar CN, et al. Orbital solitary fibrous tumor: radiographic and histopathologic correlations. Ophthalmic Plast Reconstr Surg. 2001;17(3):207–14.

    CAS  PubMed  Google Scholar 

  38. Giuffre I, Faiola A, Bonanno E, Liccardo G. Solitary fibrous tumor of the orbit. Case report and review of the literature. Surg Neurol. 2001;56(4):242–6.

    CAS  PubMed  Google Scholar 

  39. Goldsmith JD, van de Rijn M, Syed N. Orbital hemangiopericytoma and solitary fibrous tumor: a morphologic continuum. Int J Surg Pathol. 2001;9(4):295–302.

    CAS  PubMed  Google Scholar 

  40. Hayashi S, Kurihara H, Hirato J, Sasaki T. Solitary fibrous tumor of the orbit with extraorbital extension: case report. Neurosurgery. 2001;49(5):1241–5.

    CAS  PubMed  Google Scholar 

  41. Lucci LM, Anderson RL, Harrie RP, et al. Solitary fibrous tumor of the orbit in a child. Ophthalmic Plast Reconstr Surg. 2001;17(5):369–73.

    CAS  PubMed  Google Scholar 

  42. Takamura H, Kanno M, Yamashita H, Maeda K. A case of orbital solitary fibrous tumor. Jpn J Ophthalmol. 2001;45(4):412–9.

    CAS  PubMed  Google Scholar 

  43. Holbach LM, Colombo F, Schlotzer-Schrehardt U, Kirchner T. Solitary fibrous tumor of the orbit presenting 20 years after Hodgkin's disease. Orbit. 2002;21(1):49–544.

    PubMed  Google Scholar 

  44. O'Donovan DA, Bilbao JM, Fazl M, Antonyshyn OM. Solitary fibrous tumor of the orbit. J Craniofac Surg. 2002;13(5):641–4.

    PubMed  Google Scholar 

  45. Polito E, Tosi GM, Toti P, Schurfeld K, Caporossi A. Orbital solitary fibrous tumor with aggressive behavior. Three cases and review of the literature. Graefes Arch Clin Exp Ophthalmol. 2002;240(7):570–4.

    PubMed  Google Scholar 

  46. Bernardini FP, de Conciliis C, Schneider S, Kersten RC, Kulwin DR. Solitary fibrous tumor of the orbit: is it rare? Report of a case series and review of the literature. Ophthalmology. 2003;110(7):1442–8.

    PubMed  Google Scholar 

  47. Johnson TE, Onofrey CB, Ehlies FJ. Echography as a useful adjunct in the diagnosis of orbital solitary fibrous tumor. Ophthalmic Plast Reconstr Surg. 2003;19(1):68–74.

    PubMed  Google Scholar 

  48. Krishnakumar S, Subramanian N, Mohan ER, et al. Solitary fibrous tumor of the orbit: a clinicopathologic study of six cases with review of the literature. Surv Ophthalmol. 2003;48(5):544–54.

    PubMed  Google Scholar 

  49. Luo SH, Kao SC, Pan CS. Solitary fibrous tumor of the orbit. J Formos Med Assoc. 2003;102(10):726–8.

    PubMed  Google Scholar 

  50. Schellini SA, Hoyama E, Marques ME, Abreu ES, Yamashita S. Orbital solitary fibrous tumor: report of two cases and literature review. Jpn J Ophthalmol. 2003;47(4):415–8.

    PubMed  Google Scholar 

  51. Hsu SS, Lai PH, Wang JS, Yip CM. Solitary fibrous tumor of the orbit. J Chin Med Assoc. 2004;67(9):483–6.

    PubMed  Google Scholar 

  52. Galie M, Tieghi R, Cavazzini L, Clauser L. Solitary fibrous tumor of the orbit: a case report. Int J Oral Maxillofac Surg. 2005;34(3):331–3.

    CAS  PubMed  Google Scholar 

  53. Ness GO, Lybaek H, Arnes J, Rodahl E. Chromosomal imbalances in a recurrent solitary fibrous tumor of the orbit. Cancer Genet Cytogenet. 2005;162(1):38–44.

    CAS  PubMed  Google Scholar 

  54. Romer M, Bode B, Schuknecht B, Schmid S, Holzmann D. Solitary fibrous tumor of the orbit–two cases and a review of the literature. Eur Arch Otorhinolaryngol. 2005;262(2):81–8.

    CAS  PubMed  Google Scholar 

  55. Cerda-Nicolas M, Lopez-Gines C, Gil-Benso R, et al. Solitary fibrous tumor of the orbit: morphological, cytogenetic and molecular features. Neuropathology. 2006;26(6):557–63.

    PubMed  Google Scholar 

  56. Farah-Klibi F, Ferchichi L, Zairi I, et al. Lipomatous hemangiopericytoma (adipocytic variant of solitary fibrous tumor) of the orbit. A case report with review of the literature. Pathologica. 2006;98(6):645–8.

    CAS  PubMed  Google Scholar 

  57. Mascarenhas L, Lopes M, Duarte AM, et al. Histologically malignant solitary fibrous tumor of the orbit. Neurochirurgie. 2006;52(5):415–8.

    CAS  PubMed  Google Scholar 

  58. Meyer D, Riley F. Solitary fibrous tumor of the orbit: a clinicopathologic entity that warrants both a heightened awareness and an atraumatic surgical removal technique. Orbit. 2006;25(1):45–50.

    PubMed  Google Scholar 

  59. Warraich I, Dunn DM, Oliver JW. Solitary fibrous tumor of the orbit with epithelioid features. Arch Pathol Lab Med. 2006;130(7):1039–41.

    PubMed  Google Scholar 

  60. Kim HJ, Kim HJ, Kim YD, et al. Solitary fibrous tumor of the orbit: CT and MR imaging findings. AJNR Am J Neuroradiol. 2008;29(5):857–62.

    CAS  PubMed  Google Scholar 

  61. Leoncini G, Maio V, Puccioni M, et al. Orbital solitary fibrous tumor: a case report and review of the literature. Pathol Oncol Res. 2008;14(2):213–7.

    PubMed  Google Scholar 

  62. Miller NR, Agrawal N, Sciubba JJ, Lane AP. Image-guided transnasal endoscopic resection of an orbital solitary fibrous tumor. Ophthalmic Plast Reconstr Surg. 2008;24(1):65–7.

    PubMed  Google Scholar 

  63. Mukherjee B, Biswas J. Solitary fibrous tumor of the orbit. Indian J Pathol Microbiol. 2008;51(3):453–5.

    CAS  PubMed  Google Scholar 

  64. Tam ES, Chen EC, Nijhawan N, et al. Solitary fibrous tumor of the orbit: a case series. Orbit. 2008;27(6):426–31.

    PubMed  Google Scholar 

  65. Das JK, Sharma AS, Deka A, Das D. Solitary fibrous tumor of the orbit presenting in pregnancy. Indian J Ophthalmol. 2009;57(3):238–40.

    PubMed  PubMed Central  Google Scholar 

  66. Demirci H, Shields CL, Eagle RC Jr, Shields JA. Giant cell angiofibroma, a variant of solitary fibrous tumor, of the orbit in a 16-year-old girl. Ophthalmic Plast Reconstr Surg. 2009;25(5):402–4.

    PubMed  Google Scholar 

  67. Girnita L, Sahlin S, Orrego A, Seregard S. Malignant solitary fibrous tumour of the orbit. Acta Ophthalmol. 2009;87(4):464–7.

    PubMed  Google Scholar 

  68. Pitchamuthu H, Gonzalez P, Kyle P, Roberts F. Fat-forming variant of solitary fibrous tumour of the orbit: the entity previously known as lipomatous haemangiopericytoma. Eye (Lond). 2009;23(6):1479–81.

    CAS  Google Scholar 

  69. Savino G, Aliberti S, Colucci D, Perrotta V, Balestrazzi E. Atypical presentation of a case of solitary fibrous tumor of the orbit. Orbit. 2009;28(2–3):176–8.

    PubMed  Google Scholar 

  70. Adeleye AO, Ogun OA, Ogun GO. Orbital solitary fibrous tumor. Another rare case from Africa. Int Ophthalmol. 2010;30(3):315–8.

    CAS  PubMed  Google Scholar 

  71. Feuerman JM, Flint A, Elner VM. Cystic solitary fibrous tumor of the orbit. Arch Ophthalmol. 2010;128(3):385–7.

    PubMed  Google Scholar 

  72. Bandyopadhyay R, Ghosh AK, Roy R, et al. Solitary fibrous tumour of the orbit: an unusual presentation. J Indian Med Assoc. 2011;109(9):676–7.

    PubMed  Google Scholar 

  73. Furusato E, Valenzuela IA, Fanburg-Smith JC, et al. Orbital solitary fibrous tumor: encompassing terminology for hemangiopericytoma, giant cell angiofibroma, and fibrous histiocytoma of the orbit: reappraisal of 41 cases. Hum Pathol. 2011;42(1):120–8.

    PubMed  Google Scholar 

  74. Young TK, Hardy TG. Solitary fibrous tumor of the orbit with intracranial involvement. Ophthalmic Plast Reconstr Surg. 2011;27(3):e74–e7676.

    PubMed  Google Scholar 

  75. Chen H, Xiao CW, Wang T, et al. Orbital solitary fibrous tumor: a clinicopathologic study of ten cases with long-term follow-up. Acta Neurochir (Wien). 2012;154(2):249–55.

    Google Scholar 

  76. Kitamura Y, Akiyama T, Hirose S, Yoshida K. Optic nerve sheath solitary fibrous tumor. Acta Neurochir (Wien). 2012;154(4):633–5.

    Google Scholar 

  77. Ribeiro SF, Chahud F, Cruz AA. Orbital hemangiopericytoma/solitary fibrous tumor in childhood. Ophthalmic Plast Reconstr Surg. 2012;28(3):e58–60.

    PubMed  Google Scholar 

  78. Blandamura S, Alaggio R, Bettini G, et al. Four cases of solitary fibrous tumour of the eye and orbit: one with sarcomatous transformation after radiotherapy and one in a 5-year-old child's eyelid. J Clin Pathol. 2014;67(3):263–7.

    PubMed  Google Scholar 

  79. Griepentrog GJ, Harris GJ, Zambrano EV. Multiply recurrent solitary fibrous tumor of the orbit without malignant degeneration: a 45-year clinicopathologic case study. JAMA Ophthalmol. 2013;131(2):265–7.

    PubMed  Google Scholar 

  80. Meyer TN, Matos BH, Oliveira LR, Mendonca AT. Report of a case of solitary fibrous tumour of the orbit. Oral Maxillofac Surg. 2013;17(3):225–7.

    PubMed  Google Scholar 

  81. Parrozzani R, Fusetti S, Montesco C, Favero V, Midena E. Biphasic solitary fibrous tumor of the orbit with distant metastases. Int Ophthalmol. 2013;33(6):701–5.

    PubMed  Google Scholar 

  82. Polomsky M, Sines DT, Dutton JJ. Solitary fibrous tumor of the orbit with multiple cavities. Ophthalmic Plast Reconstr Surg. 2013;29(5):e117–e11919.

    PubMed  Google Scholar 

  83. Rose AM, Kabiru J, Rose GE. A rare case of orbital haemangiopericytoma arising in childhood. Orbit. 2013;32(6):384–6.

    PubMed  Google Scholar 

  84. Warner EJ, Burkat CN, Gentry LR. Orbital fibrous histiocytoma mimicking cavernous hemangioma on dynamic contrast-enhanced MRA imaging. Ophthalmic Plast Reconstr Surg. 2013;29(1):e3–5.

    PubMed  Google Scholar 

  85. Graue GF, Schubert HD, Kazim M. Correlation between clinical features, imaging and pathologic findings in recurrent solitary fibrous tumor of the orbit. Orbit. 2013;32(6):375–80.

    PubMed  Google Scholar 

  86. Kishimoto I, Shinohara S, Fujiwara K, et al. A case of intraorbital solitary fibrous tumor resected successfully with preoperative arterial embolization. Nihon Jibiinkoka Gakkai Kaiho. 2014;117(12):1477–82.

    PubMed  Google Scholar 

  87. Le CP, Jones S, Valenzuela AA. Orbital solitary fibrous tumor: a case series with review of the literature. Orbit. 2014;33(2):145–51.

    PubMed  Google Scholar 

  88. Liu Y, Li K, Shi H, Tao X. Solitary fibrous tumours in the extracranial head and neck region: correlation of CT and MR features with pathologic findings. Radiol Med. 2014;119(12):910–9.

    PubMed  Google Scholar 

  89. Wallace KM, Alaraj A, Aakalu VK, Aletich V, Setabutr P. Endovascular preoperative embolization of orbital hemangiopericytoma with n-butyl cyanoacrylate glue. Ophthalmic Plast Reconstr Surg. 2014;30(4):e97–100.

    PubMed  PubMed Central  Google Scholar 

  90. Genc A, Toktas Z, Azman C, Bozkurt SU, Kilic T. Solitary fibrous tumor of the orbit: a case report and review of the literature. Turk Neurosurg. 2015;25(6):984–7.

    PubMed  Google Scholar 

  91. Hashemi N, Ling JD, Soparkar C, et al. Transarterial onyx embolization of an orbital solitary fibrous tumor. Ocul Oncol Pathol. 2015;1(2):98–102.

    PubMed  PubMed Central  Google Scholar 

  92. Rahman T, Ahmed K, Sarmah J, Das A. Solitary fibrous tumor of orbit: a rare entity. Indian J Cancer. 2015;52(3):396–7.

    CAS  PubMed  Google Scholar 

  93. Tenekeci G, Sari A, Vayisoglu Y, Serin O. Giant solitary fibrous tumor of orbit. J Craniofac Surg. 2015;26(5):e390–e392392.

    PubMed  Google Scholar 

  94. Kunzel J, Hainz M, Ziebart T, et al. Head and neck solitary fibrous tumors: a rare and challenging entity. Eur Arch Otorhinolaryngol. 2016;273(6):1589–98.

    PubMed  Google Scholar 

  95. Tata A, Cohen-Inbar O, Sheehan JP. Treatment of orbital solitary fibrous tumour with gamma knife radiosurgery and systematic review of literature. BMJ Case Rep. 2016;2016.

  96. Feijo ED, Nery ACS, Caiado FR, Limongi RM. Solitary fibrous tumor of the lacrimal gland mimicking pleomorphic adenoma. Arq Bras Oftalmol. 2017;80(3):189–91.

    PubMed  Google Scholar 

  97. Jung SK, Paik JS, Park GS, Yang SW. CD34 + tumours of the orbit including solitary fibrous tumours: a six-case series. BMC Ophthalmol. 2017;17(1):59.

    PubMed  PubMed Central  Google Scholar 

  98. Moriyama M, Kodama S, Hirano T, Suzuki M. Endoscopic-modified medial maxillectomy and its limitation for a solitary fibrous tumor of the lacrimal sac and nasolacrimal duct. Auris Nasus Larynx. 2017;44(3):370–4.

    PubMed  Google Scholar 

  99. Smith SC, Gooding WE, Elkins M, et al. Solitary fibrous tumors of the head and neck: a multi-institutional clinicopathologic study. Am J Surg Pathol. 2017;41(12):1642–56.

    PubMed  PubMed Central  Google Scholar 

  100. Vu AF, Chundury RV, Blandford AD, Perry JD. Recurrent orbital solitary fibrous tumor in a 12-year-old. Ocul Oncol Pathol. 2017;3(2):83–6.

    PubMed  Google Scholar 

  101. Alam S, Backiavathy V, Mukherjee B, Subramanian K. A rare case of giant multicystic solitary fibrous tumor of the orbit. Orbit. 2018;37(1):69–72.

    PubMed  Google Scholar 

  102. Tanaka K, Yano H, Hayashi H, Hirano A. Total resection combined with osteotomy is more effective for orbital solitary fibrous tumor excision: a report of three cases. Int Ophthalmol. 2018;38(1):345–51.

    PubMed  Google Scholar 

  103. Zheng L, McCluskey P, Ghabrial R. Largest reported orbital solitary fibrous tumour. Clin Exp Ophthalmol. 2018;46(3):301–3.

    PubMed  Google Scholar 

  104. Demura M, Hayashi Y, Sasagawa Y, et al. Intraorbital solitary fibrous tumor requiring preoperative embolization of feeding artery. Asian J Neurosurg. 2019;14(2):593–7.

    PubMed  PubMed Central  Google Scholar 

  105. Hyde RA, Liu Y, Aakalu VK, Setabutr P. Solitary fibrous tumor of the orbit with growth during pregnancy: a case report. Orbit. 2019;38(3):256–8.

    PubMed  Google Scholar 

  106. Sagiv O, Bell D, Guo Y, et al. Pathological features and clinical course in patients with recurrent or malignant orbital solitary fibrous tumor/hemangiopericytoma. Ophthalmic Plast Reconstr Surg. 2019;35(2):148–54.

    PubMed  Google Scholar 

  107. Sayit AT, Elmali M, Gul A, Sullu Y. Solitary fibrous tumor of the orbit: Computed tomography and histopathological findings. J Cancer Res Ther. 2019;15(3):719–21.

    PubMed  Google Scholar 

  108. Patel MM, Jakobiec FA, Zakka FR, et al. Intraorbital metastasis from solitary fibrous tumor. Ophthalmic Plast Reconstr Surg. 2013;29(3):e76–e7979.

    PubMed  Google Scholar 

  109. Glazer-Hockstein C, Syed NA, Warhol M, Gausas RE. Malignant solitary fibrous tumor metastatic to the orbit. Ophthalmic Plast Reconstr Surg. 2004;20(6):471–3.

    PubMed  Google Scholar 

  110. Pasquali S, Gronchi A, Strauss D, et al. Resectable extra-pleural and extra-meningeal solitary fibrous tumours: a multi-centre prognostic study. Eur J Surg Oncol. 2016;42(7):1064–70.

    CAS  PubMed  Google Scholar 

  111. Salas S, Resseguier N, Blay JY, et al. Prediction of local and metastatic recurrence in solitary fibrous tumor: construction of a risk calculator in a multicenter cohort from the French Sarcoma Group (FSG) database. Ann Oncol. 2017;28(8):1979–87.

    CAS  PubMed  Google Scholar 

  112. Demicco EG, Wagner MJ, Maki RG, et al. Risk assessment in solitary fibrous tumors: validation and refinement of a risk stratification model. Mod Pathol. 2017;30(10):1433–42.

    PubMed  Google Scholar 

  113. Akaike K, Kurisaki-Arakawa A, Hara K, et al. Distinct clinicopathological features of NAB2-STAT6 fusion gene variants in solitary fibrous tumor with emphasis on the acquisition of highly malignant potential. Hum Pathol. 2015;46(3):347–56.

    CAS  PubMed  Google Scholar 

  114. Barthelmess S, Geddert H, Boltze C, et al. Solitary fibrous tumors/hemangiopericytomas with different variants of the NAB2-STAT6 gene fusion are characterized by specific histomorphology and distinct clinicopathological features. Am J Pathol. 2014;184(4):1209–18.

    CAS  PubMed  Google Scholar 

  115. Fritchie K, Jensch K, Moskalev EA, et al. The impact of histopathology and NAB2-STAT6 fusion subtype in classification and grading of meningeal solitary fibrous tumor/hemangiopericytoma. Acta Neuropathol. 2019;137(2):307–19.

    CAS  PubMed  Google Scholar 

  116. Dagrada GP, Spagnuolo RD, Mauro V, et al. Solitary fibrous tumors: loss of chimeric protein expression and genomic instability mark dedifferentiation. Mod Pathol. 2015;28(8):1074–83.

    CAS  PubMed  Google Scholar 

  117. Demicco EG, Wani K, Ingram D, et al. TERT promoter mutations in solitary fibrous tumour. Histopathology. 2018;73(5):843–51.

    PubMed  Google Scholar 

  118. Demicco EG, Griffin AM, Gladdy RA, et al. Comparison of published risk models for prediction of outcome in patients with extrameningeal solitary fibrous tumour. Histopathology. 2019;75(5):723–37.

    PubMed  Google Scholar 

  119. Chick JF, Chauhan NR, Madan R. Solitary fibrous tumors of the thorax: nomenclature, epidemiology, radiologic and pathologic findings, differential diagnoses, and management. AJR Am J Roentgenol. 2013;200(3):W238–W248248.

    PubMed  Google Scholar 

  120. Font RL, Hidayat AA. Fibrous histiocytoma of the orbit. A clinicopathologic study of 150 cases. Hum Pathol. 1982;13(3):199–209.

    CAS  PubMed  Google Scholar 

  121. Schweizer L, Koelsche C, Sahm F, et al. Meningeal hemangiopericytoma and solitary fibrous tumors carry the NAB2-STAT6 fusion and can be diagnosed by nuclear expression of STAT6 protein. Acta Neuropathol. 2013;125(5):651–8.

    CAS  PubMed  Google Scholar 

  122. Thompson LDR, Lau SK. Sinonasal tract solitary fibrous tumor: a clinicopathologic study of six cases with a comprehensive review of the literature. Head Neck Pathol. 2018;12(4):471–80.

    PubMed  Google Scholar 

  123. Meng W, Zhu HH, Li H, et al. Solitary fibrous tumors of the pleura with Doege-Potter syndrome: a case report and three-decade review of the literature. BMC Res Notes. 2014;7:515.

    PubMed  PubMed Central  Google Scholar 

  124. Fung EC, Crook MA. Doege-Potter syndrome and 'big-IGF2': a rare cause of hypoglycaemia. Ann Clin Biochem. 2011;48(Pt 2):95–6.

    CAS  PubMed  Google Scholar 

  125. Ganly I, Patel SG, Stambuk HE, et al. Solitary fibrous tumors of the head and neck: a clinicopathologic and radiologic review. Arch Otolaryngol Head Neck Surg. 2006;132(5):517–25.

    PubMed  Google Scholar 

  126. Thompson LD, Miettinen M, Wenig BM. Sinonasal-type hemangiopericytoma: a clinicopathologic and immunophenotypic analysis of 104 cases showing perivascular myoid differentiation. Am J Surg Pathol. 2003;27(6):737–49.

    PubMed  Google Scholar 

  127. Lasota J, Felisiak-Golabek A, Aly FZ, et al. Nuclear expression and gain-of-function beta-catenin mutation in glomangiopericytoma (sinonasal-type hemangiopericytoma): insight into pathogenesis and a diagnostic marker. Mod Pathol. 2015;28(5):715–20.

    CAS  PubMed  Google Scholar 

  128. Yammine K, Nasser HA, Hadi U, et al. Salvage preoperative embolization of an infratemporal solitary fibrous tumor: A case report with review of the literature. Medicine (Baltimore). 2018;97(13):e0251.

    Google Scholar 

Download references

Acknowledgement

Data presented as Abstract #2, Poster #97 at the 109th Annual Meeting of the United States and Canadian Academy of Pathology, Los Angeles, CA: March 3, 2020.

Funding

No external funding was obtained for this study.

Author information

Authors and Affiliations

  1. Department of Pathology, Southern California Permanente Medical Group, 5601 De Soto Avenue, Woodland Hills, CA, 91365, USA

    Lester D. R. Thompson

  2. Department of Pathology, University of California: Los Angeles, Los Angeles, CA, USA

    Sofia S. Liou

  3. Department of Ophthalmology, Southern California Permanente Medical Group, Harbor City, CA, USA

    Kenneth A. Feldman

Authors
  1. Lester D. R. Thompson
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Sofia S. Liou
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Kenneth A. Feldman
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Lester D. R. Thompson.

Ethics declarations

Conflict of interest

All authors declare that they have no conflict of interest as it relates to this research project

Ethical Approval

All procedures performed in this retrospective data analysis involving human participants were in accordance with the ethical standards of the institutional review board (IRB #5968), which did not require informed consent. The opinions or assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of Southern California Permanente Medical Group.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Thompson, L.D.R., Liou, S.S. & Feldman, K.A. Orbit Solitary Fibrous Tumor: A Proposed Risk Prediction Model Based on a Case Series and Comprehensive Literature Review. Head and Neck Pathol 15, 138–152 (2021). https://doi.org/10.1007/s12105-020-01184-6

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12105-020-01184-6

Keywords

Search

Navigation