Skip to main content

Clinico-epidemiological analysis of 1000 cases of orbital tumors

Abstract

Purpose

To clarify the incidence, demography and clinical features of orbital tumors diagnosed in a single institute in Japan.

Study design

Retrospective, observational case series.

Methods

Patients with primary orbital tumors including tumor-like lesions diagnosed clinically or histopathologically at Tokyo Medical University Hospital between 1995 and 2019 were analyzed. Incidence of all orbital tumors, demographic profile and clinical features of major benign and malignant tumors were reviewed retrospectively.

Results

Totally 1000 cases of primary orbital tumor were diagnosed clinically or histopathologically during the study period. Benign tumors accounted for 72% and malignant tumors 28%. 55% of benign tumors and 99% of malignant tumors were proven histopathologically. The most common benign orbital tumor was idiopathic orbital inflammation (27%), followed by IgG4-related ophthalmic disease (17%), cavernous venous malformation (13%) and pleomorphic adenoma (9%). The most common malignant tumor was lymphoma (70%), followed by adenoid cystic carcinoma (7%) and solitary fibrous tumor (5%).

Conclusions

Epidemiology of orbital tumors has changed by the improvement of imaging techniques, establishment of novel clinical and histopathological criteria, and changes in population age structure associated with the aging society. Currently, lymphoproliferative diseases including lymphoma and IgG4-related ophthalmic diseases form the major orbital tumors in Japan.

This is a preview of subscription content, access via your institution.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7
Fig. 8
Fig. 9
Fig. 10
Fig. 11
Fig. 12
Fig. 13
Fig. 14
Fig. 15
Fig. 16
Fig. 17
Fig. 18
Fig. 19
Fig. 20
Fig. 21
Fig. 22

References

  1. 1.

    Shields JA, Shields CL, Scartozzi R. Survey of 1264 patients with orbital tumors and simulating lesions: the 2002 Montgomery Lecture, part 1. Ophthalmology. 2004;111:997–1008.

    PubMed  Article  Google Scholar 

  2. 2.

    Bonavolontà G, Strianese D, Grassi P, Comune C, Tranfa F, Uccello G, et al. An analysis of 2,480 space-occupying lesions of the orbit from 1976 to 2011. Ophthalmic Plast Reconstr Surg. 2013;29:79–86.

    PubMed  Article  Google Scholar 

  3. 3.

    Hassan WM, Alfaar AS, Bakry MS, Ezzat S. Orbital tumors in USA: difference in survival patterns. Cancer Epidemiol. 2014;38:515–22.

    PubMed  Article  Google Scholar 

  4. 4.

    Honavar SG, Manjandavida FP. Recent advances in orbital tumors—a review of publications from 2014–2016. Asia Pac J Ophthalmol (Phila). 2017;6:153–8.

    Google Scholar 

  5. 5.

    Mombaerts I, Ramberg I, Coupland SE, Heegaard S. Diagnosis of orbital mass lesions: clinical, radiological, and pathological recommendations. Surv Ophthalmol. 2019;64:741–56.

    PubMed  Article  Google Scholar 

  6. 6.

    Tailor TD, Gupta D, Dalley RW, Keene CD, Anzai Y. Orbital neoplasms in adults: clinical, radiologic, and pathologic review. Radiographics. 2013;33:1739–58.

    PubMed  Article  Google Scholar 

  7. 7.

    Demirci H, Shields CL, Karatza EC, Shields JA. Orbital lymphoproliferative tumors: analysis of clinical features and systemic involvement in 160 cases. Ophthalmology. 2008;115:1626–31.

    PubMed  Article  Google Scholar 

  8. 8.

    Ohtsuka K, Hashimoto M, Suzuki Y. High incidence of orbital malignant lymphoma in Japanese patients. Am J Ophthalmol. 2004;138:881–2.

    PubMed  Article  Google Scholar 

  9. 9.

    Shikishima K, Kawai K, Kitahara K. Pathological Evaluation of orbital tumours in Japan: analysis of a large case Series and 1379 cases reported in the Japanese literature. Clin Exp Ophthalmol. 2006;34:239–44.

    PubMed  Article  Google Scholar 

  10. 10.

    Yamamoto M, Ohara M, Suzuki C, Naishiro Y, Yamamoto H, Takahashi H, et al. Elevated IgG4 concentrations in serum of patients with Mikulicz’s disease. Scand J Rhematol. 2004;33:432–3.

    CAS  Article  Google Scholar 

  11. 11.

    Umehara H, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4RD), 2011. Mod Rheumatol. 2012;22:21–30.

    CAS  PubMed  Article  Google Scholar 

  12. 12.

    Goto H, Takahira M, Azumi A. Japanese Study Group for IgG4-related ophthalmic disease. Diagnostic criteria for IgG4-related ophthalmic disease. Jpn J Ophthalmol. 2015;59:1–7.

    CAS  PubMed  Article  Google Scholar 

  13. 13.

    Japanese study group of IgG4-related ophthalmic disease. A prevalence study of IgG4-related ophthalmic disease in Japan. Jpn J Ophthalmol 2013;57:573–9.

  14. 14.

    Calandriello L, Grimaldi G, Petrone G, Rigante M, Petroni S, Riso M, et al. Cavernous venous malformation (cavernous hemangioma) of the orbit: current concepts and a review of the literature. Surv Ophthalmol. 2017;6:393–403.

    Article  Google Scholar 

  15. 15.

    Ohtsuka K, Hashimoto M, Akiba H. Serial dynamic magnetic resonance imaging of orbital cavernous hemangioma. Am J Ophthalmol. 1997;123:396–8.

    CAS  PubMed  Article  Google Scholar 

  16. 16.

    Rootman DB, Heran MK, Rootman J, White VA, Luemsamran P, Yucel YH. Cavernous venous malformations of the orbit (so-called cavernous haemangioma): a comprehensive evaluation of their clinical, imaging and histologic nature. Br J Ophthalmol. 2014;98:880–8.

    PubMed  Article  Google Scholar 

  17. 17.

    Clarós P, Choffor-Nchinda E, Lopez-Fortuny M, Claros A, Quintana S. Orbital cavernous haemangioma; profile and outcome of 76 patients managed surgically. Acta Otolaryngol. 2019;139:720–5.

    PubMed  Article  Google Scholar 

  18. 18.

    McNab AA, Tan JS, Xie J, Selva D, Hardy TG, Starte J, et al. The natural history of orbital cavernous hemangiomas. Ophthalmic Plast Reconstr Surg. 2015;31:89–93.

    PubMed  Article  Google Scholar 

  19. 19.

    Gupta A, Prabhakaran VC, Dodd T, Davis G, Selva D. Orbital cavernous haemangiomas: immunohistochemical study of proliferative capacity, vascular differentiation and hormonal receptor status. Orbit. 2012;31:386–9.

    PubMed  Article  Google Scholar 

  20. 20.

    Bond JB, Haik BG, Taveras JL, Francis BA, Numaguchi Y, Mihara F, et al. Magnetic resonance imaging of orbital lymphangioma with and without gadolinium contrast enhancement. Ophthalmology. 1992;99:1318–24.

    CAS  PubMed  Article  Google Scholar 

  21. 21.

    Iliff WJ, Green WR. Orbital lymphangiomas. Ophthalmology. 1979;86:914–29.

    CAS  PubMed  Article  Google Scholar 

  22. 22.

    Goto H, Usui M, Okada S. Histopathological study of orbital lymphangioma in an infant. Jpn J Ophthalmol. 2004;48:594–7.

    PubMed  Article  Google Scholar 

  23. 23.

    Pointdujour-Lim R, Lally SE, Shields JA, Eagle RC Jr, Shields CL. Orbital schwannoma: radiographic and histopathologic correlation in 15 cases. Ophthalmic Plast Reconstr Surg. 2018;34:162–7.

    PubMed  Article  Google Scholar 

  24. 24.

    Wang Y, Xiao LH. Orbital schwannoma: findings from magnetic resonance imaging in 62 cases. Eye (Lond). 2008;22:1034–9.

    CAS  Article  Google Scholar 

  25. 25.

    Dutton JJ. Optic nerve sheath meningiomas. Surv Ophthalmol. 1992;37:167–83.

    CAS  PubMed  Article  Google Scholar 

  26. 26.

    Hénaux PL, Bretonnier M, Restee P-JL, Morandi X. Modern management of meningiomas compressing the optic nerve: a systematic review. World Neurosurg. 2018. https://doi.org/10.1016/j.wneu.2018.07.020.

    Article  PubMed  Google Scholar 

  27. 27.

    Sasano S, Shikishima K, Aoki M, Sakai T, Tsutsumi Y, Nakano T. Efficacy of intensity-modulated radiation therapy for optic nerve sheath meningioma. Graefes Arch Clin Exp Ophthalmol. 2019;257:2297–306.

    PubMed  Article  Google Scholar 

  28. 28.

    Pushker N, Meel R, Kumar A, Kashyap S, Sen S, Bajaj MS. Orbital and periorbital dermoid/epidermoid cyst: a series of 280 cases and a brief review. Can J Ophthalmol. 2020;55:167–71.

    PubMed  Article  Google Scholar 

  29. 29.

    Goh RLZ, Hardy TG, Williams RA, McNab AA. Respiratory epithelial cysts of the orbit. Orbit. 2016;35:245–9.

    PubMed  Article  Google Scholar 

  30. 30.

    Rose GE, Wright JE. Pleomorphic adenoma of the lacrimal gland. Br J Ophthalmol. 1992;76:395–400.

    CAS  PubMed  PubMed Central  Article  Google Scholar 

  31. 31.

    Gündüz AK, Yeşiltaş YS, Shields CL. Overview of benign and malignant lacrimal gland tumors. Curr Opin Ophthalmol. 2018;29:458–68.

    PubMed  Article  Google Scholar 

  32. 32.

    Lai T, Prabhakaran VC, Malhotra R, Selva D. Pleomorphic adenoma of the lacrimal gland: is there a role for biopsy? Eye (Lond). 2009;23:2–6.

    CAS  Article  Google Scholar 

  33. 33.

    Garrity JA, Henderson JW, Cameron JD. Henderson’s orbital tumors. 4th ed. Philadelphia: Lippincott Williams & Wilkins; 2007. p. 343–60.

    Google Scholar 

  34. 34.

    Sogabe Y, Ohshima K, Azumi A, Takahira M, Kase S, Tsuji H, et al. Location and frequency of lesions in patients with IgG4-related ophthalmic diseases. Graefes Arch Clin Exp Ophthalmol. 2014;252:531–8.

    CAS  PubMed  Article  Google Scholar 

  35. 35.

    Dmirci H, Shields CL, Shields JA, Honavar SG, Mercado GJ, Tovilla JC. Orbital tumors in the older adult population. Ophthalmology. 2002;109:243–8.

    Article  Google Scholar 

  36. 36.

    Sjö LD, Ralfkiaer E, Prause JU, Petersen JH, Madsen J, Pedersen NT, et al. Increasing incidence of ophthalmic lymphoma in Denmark from 1980 to 2005. Invest Ophthalmol Vis Sci. 2008;49:3283–8.

    PubMed  Article  Google Scholar 

  37. 37.

    Hassan WM, Bakry MS, Hassan HM, Alfaar AS. Incidence of orbital, conjunctival and lacrimal gland malignant tumors in USA from Surveillance, Epidemiology and End Results, 1973–2009. Int J Ophthalmol. 2016;9:1808–13.

    PubMed  PubMed Central  Google Scholar 

  38. 38.

    Olsen TG, Holm F, Mikkelsen LH, Rasmussen PK, Coupland SE, Esmaeli B, et al. Orbital lymphoma-an international multicenter retrospective study. Am J Ophthalmol. 2019;199:44–57.

    PubMed  Article  Google Scholar 

  39. 39.

    Vest SD, Mikkelsen LH, Holm F, Rasmussen PK, Hindso TG, Knudsen MKH, et al. Lymphoma of the lacrimal gland—an international multicenter retrospective study. Am J Ophthalmol. 2020;219:107–20.

    CAS  PubMed  Article  Google Scholar 

  40. 40.

    Lacrimal Gland Tumor Study Group. Epidemiological survey of lacrimal fossa lesions in Japan: number of patients and their sex ratio by pathological diagnosis. Jpn J Ophthalmol. 2005;49:343–8.

    Article  Google Scholar 

  41. 41.

    Alkatan H, Roberts F, White VA. Adenocarcinoma of the lacrimal gland. In: Grossniklaus HE, Eberhart CG, Kivelä TT, editors. WHO Classification of Tumors of the Eye. WHO Press, Geneva; 2018.

  42. 42.

    See TRO, Stålhammar G, Tang T, Manusow JS, Jordan DR, Nerad JA, et al. Primary ductal adenocarcinoma of the lacrimal gland: a review and report of five cases. Surv Ophthalmol. 2020;65:371–80.

    PubMed  Article  Google Scholar 

  43. 43.

    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;110:1442–8.

    PubMed  Article  Google Scholar 

  44. 44.

    Furusato E, Valenzuela IA, Fanburg-Smith JC, Auerbach A, Furusato B, Cameron JD, 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;42:120–8.

    PubMed  Article  Google Scholar 

  45. 45.

    Shinder R, Al-Zubidi N, Esmaeli B. Survey of orbital tumors at a comprehensive cancer center in the United States. Head Neck. 2011;33:610–4.

    PubMed  Article  Google Scholar 

Download references

Acknowledgements

This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Author information

Affiliations

Authors

Corresponding author

Correspondence to Hiroshi Goto.

Ethics declarations

Conflicts of interest

H. Goto, None; N. Yamakawa, None; H. Komatsu, None; M. Asakage, None; K. Tsubota, None; S. Ueda, None; R. Nemoto, None; K. Umazume, None; Y. Usui, None; H. Mori, None.

Additional information

Publisher's Note

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

Corresponding Author: Hiroshi Goto

About this article

Verify currency and authenticity via CrossMark

Cite this article

Goto, H., Yamakawa, N., Komatsu, H. et al. Clinico-epidemiological analysis of 1000 cases of orbital tumors. Jpn J Ophthalmol 65, 704–723 (2021). https://doi.org/10.1007/s10384-021-00857-1

Download citation

Keywords

  • Orbital tumor
  • Epidemiology
  • Histopathology
  • Imaging
  • Lymphoproliferative disease