Clinicopathologic analysis of 32 ciliary body tumors

Abstract

Purpose

To describe the demographic profile, clinical and histopathologic features, and treatment of ciliary body tumors.

Study design

Retrospective, observational case series.

Methods

Thirty-two patients (32 eyes) with ciliary body tumors diagnosed histopathologically at Tokyo Medical University Hospital between 1994 and 2017 were retrospectively reviewed.

Results

The patients’ mean age at diagnosis was 45.4 ± 17.0 (range, 14–87) years. Ten of the patients were male, and 22, female. Twenty-four cases (75%) were benign tumors, comprising 9 melanocytomas, 7 adenomas, 4 mesectodermal leiomyomas, 2 leiomyomas, and 2 other tumors; and 8 cases (25%) were malignant tumors, comprising 6 melanomas and 2 low-grade adenocarcinomas. Local resection of the tumor was performed in 20 patients, including 3 cases of melanoma and 2 cases of adenocarcinoma. Enucleation was initially performed in 3 cases of melanoma, 1 case of melanocytoma with iris melanoma, and 2 cases of benign tumors difficult to differentiate clinically from melanoma. In the 17 patients who underwent local resection and were followed for at least 3 years, the outcome was best-corrected visual acuity better than 0.1 logMAR in 8 patients (47%), but hand motions in 2 patients (12%).

Conclusions

Melanocytoma and adenoma of the ciliary epithelium were the major ciliary body tumors found in this study. Management of ciliary body tumors with accurate clinical diagnosis remains challenging because of the anatomic characteristics and clinical similarities to melanoma in the majority of the cases.

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References

  1. 1.

    Shields JA, Augsburger JJ, Bernardino V Jr, Eller AW, Kulczycki E. Melanocytoma of the ciliary body and iris. Am J Ophthalmol. 1980;89:632–5.

    CAS  Article  Google Scholar 

  2. 2.

    Frangish GT, El Baba F, Traboulsi EI, Green WR. Melanocytoma of the ciliary body: presentation of four cases and review of nineteen reports. Surv Ophthalmol. 1985;29:328–34.

    Article  Google Scholar 

  3. 3.

    LoRusso FJ, Boniuk M, Font RL. Melanocytoma (magnocellular nevus) of the ciliary body: report of 10 cases and review of the literature. Ophthalmology. 2000;107:795–800.

    CAS  Article  Google Scholar 

  4. 4.

    Shields JA, Eagle RC Jr, Shields CL, Potter PD. Acquired neoplasms of the nonpigmented ciliary epithelium (adenoma and adenocarcinoma). Ophthalmology. 1996;103:2007–16.

    CAS  Article  Google Scholar 

  5. 5.

    Shields JA, Shields CL, Gündüz K, Eagle RC Jr. Adenoma of the ciliary body pigment epithelium: the 1998 Albert Ruedemann, Sr, memorial lecture, Part 1. Arch Ophthalmol. 1999;117:592–7.

    CAS  Article  Google Scholar 

  6. 6.

    Mansoor S, Qureshi A. Ciliary body adenoma of non-pigment epithelium. J Clinc Pathol. 2004;57:997–8.

    CAS  Article  Google Scholar 

  7. 7.

    Yan J, Liu X, Zhang P, Li Y. Acquired adenoma of the nonpigmented ciliary epithelium: analysis of five cases. Graefes Arch Clin Exp Ophthalmol. 2015;253:637–44.

    Article  Google Scholar 

  8. 8.

    Meyer SL, Fine BS, Zimmerman LE. Leiomyoma of the ciliary body electron microscopic verification. Am J Ophthalmol. 1968;66:1061–8.

    CAS  Article  Google Scholar 

  9. 9.

    Croxatto JO, Malbran ES. Unusual ciliary body tumor: mesectodermal leiomyoma. Ophthalmology. 1982;89:1208–12.

    CAS  Article  Google Scholar 

  10. 10.

    Shields JA, Shields CL, Eagle RC Jr. Mesectodermal leiomyoma of the ciliary body managed by partial lamellar iridocyclochoroidectomy. Ophthalmology. 1989;96:169–76.

    Google Scholar 

  11. 11.

    Lois N, Shields CL, Shields JA, Eagle RC Jr, Potter PD. Cavitary melanoma of the ciliary body: a study of eight cases. Ophthalmology. 1998;105:1091–8.

    CAS  Article  Google Scholar 

  12. 12.

    Goto H, Usui M, Ishii I. Efficacy of (123) N-isopropyl-p- [(123) I]- iodoamphetamine single photon emission computed tomography for the diagnosis of uveal malignant melanoma. Am J Ophthalmol. 2001;132:937–9.

    CAS  Article  Google Scholar 

  13. 13.

    Goto H. Clinical efficacy of 123I-IMP SPECT for the diagnosis of malignant uveal melanoma. Int J Clin Oncol. 2004;9:74–8.

    Article  Google Scholar 

  14. 14.

    Klauber S, Jensen PK, Prause JU, Kessing SV. Surgical treatment of iris and ciliary body melanoma: follow-up of a 25-year series of patients. Acta Ophthalmol. 2012;90:122–6.

    Article  Google Scholar 

  15. 15.

    Rospond-Kubiak I, Damato B. The surgical approach to the management of anterior uveal melanomas. Eye (Lond). 2014;28:741–7.

    CAS  Article  Google Scholar 

  16. 16.

    Suzuki J, Goto H, Usui M. Adenoma arising from nonpigmented ciliary epithelium concomitant with neovascularization of the optic disc and cystoid macular edema. Am J Ophthalmol. 2005;139:188–90.

    Article  Google Scholar 

  17. 17.

    Goto H, Usui Y, Nagao T. Perivascular epithelioid cell tumor arising from ciliary body treated by local resection. Ocul Oncol Pathol. 2015;1:88–92.

    Article  Google Scholar 

  18. 18.

    Jakobiec FA, Font RL, Tso MO, Zimmerman LE. Mesectodermal leiomyoma of the ciliary body: a tumor of presumed neural crest origin. Cancer. 1977;39:2102–13.

    CAS  Article  Google Scholar 

  19. 19.

    Koletsa T, Karayannopoulou G, Dereklis D, Vasileiadis I, Papadimitriou CS, Hytiroglou P. Mesectodermal leiomyoma of the ciliary body: report of a case and review of the literature. Pathol Res Pract. 2009;205:125–30.

    Article  Google Scholar 

  20. 20.

    Goto H, Mori H, Shirato S, Usui M. Ciliary body schwannoma successfully treated by local resection. Jpn J Ophthalmol. 2006;50:543–6.

    Article  Google Scholar 

  21. 21.

    Shields JA, Shields CL, Shah P, Sivalingam V. Partial lamellar sclerouvectomy for ciliary body and choroidal tumors. Ophthalmology. 1991;98:971–83.

    CAS  Article  Google Scholar 

  22. 22.

    Bae JH, Kwon JI, Yang WI, Li SC. Adenoma of the nonpigmented ciliary epithelium presenting with recurrent iridocyclitis: unique expression of glial fibrillary acidic protein. Graefes Arch Clin Exp Ophthalmol. 2011;249:1747–9.

    Article  Google Scholar 

  23. 23.

    Usui Y, Tsubota K, Agawa T, Ueda S, Umazuma K, Okunuki Y, et al. Aqueous immune mediators in malignant uveal melanomas in comparison to benign pigmented intraocular tumors. Graefes Arch Clin Exp Ophthalmol. 2017;255:393–9.

    CAS  Article  Google Scholar 

  24. 24.

    Wierenga APA, Cao J, Mouthaan H, van Weeghel C, Verdijk RM, van Duinen SG, et al. Aqueous humor biomarkers identify three prognostic groups in uveal melanoma. Invest Ophthalmol Vis Sci. 2019;60:4740–7.

    CAS  Article  Google Scholar 

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Correspondence to Hiroshi Goto.

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H. Goto, None; N. Yamakawa, None; K. Tsubota, None; K. Umazume, None; Y. Usui, None.

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Goto, H., Yamakawa, N., Tsubota, K. et al. Clinicopathologic analysis of 32 ciliary body tumors. Jpn J Ophthalmol (2021). https://doi.org/10.1007/s10384-021-00814-y

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Keywords

  • Ciliary body tumor
  • Histopathology
  • Local resection
  • Melanoma