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Analysis of clinical features of RPE adenoma

  • Oncology
  • Published:
Graefe's Archive for Clinical and Experimental Ophthalmology Aims and scope Submit manuscript

A Correction to this article was published on 25 June 2020

This article has been updated

Abstract

Purpose

This study aims to evaluate the clinical features of adenoma of the retinal pigment epithelium (RPE) and eye-sparing treatment for it.

Methods

The patients underwent measurement of visual acuity and intraocular pressure, slit-lamp examination, ophthalmoscopy, fluorescein fundus angiography (FFA), indocyanine green angiography (ICGA), color Doppler imaging (CDI), and magnetic resonance imaging (MRI). The tumors were endoresected and studied histopathologically.

Results

Of the 16 Asian patients, 15 had RPE adenoma and 1 had RPE adenocarcinoma. Visual acuity decreased in three cases, mainly due to the macular detachment. All tumors were solitary and unilateral and measured from 1.7 × 3.2 × 2.4 to 9.3 × 8.0 × 6.6 mm3. The tumor was located in the macular area in 2 patients, in the juxtapapillary area in 1 patient, and in the peripheral fundus in 13 patients. The tumors were yellow-pink in 3 patients and brown in 13 patients. The tumors showed hypofluorescence in early stage and mottled hyperfluorescence with prominent leakage in late stage on fluorescein angiography. CDI demonstrated arterial blood signals within tumor, and MRI demonstrated hyperintensity and hypointensity in the T1- and T2-weighted images, respectively. The tumors were positive for S-100, neuron-specific enolase (NSE), CK, epithelial membrane antigen (EMA), and vimentin; occasionally positive for melanin-A; and usually negative for melanoma-specific antigen HMB45 and synaptophysin (Syn). The endoresection surgery was performed in all 16 patients by the microinvasive vitrectomy for the excision of intraocular tumors and reconstruction of the eyeball. The follow-up time was 1.5–13 years (mean, 5 years). No tumor recurrence occurred, and the retina remained attached in 16 eyes.

Conclusions

RPE-derived adenomas are rare and difficult to diagnose clinically. Local resection by the mircoinvasive vitrectomy is a feasible alternative treatment for RPE adenoma.

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Change history

  • 25 June 2020

    In the original version of our paper entitled Analysis of clinical features of RPE adenoma published in Graefe's Archive for Clinical and Experimental Ophthalmology.

References

  1. Karin U, Loeffler KU, Kivela T, Borgmann H et al (1996) Malignant tumor of the retinal pigment epithelium with extraocular extension in a phthisical eye. Graefes Arch Clin Exp Opthalmol 234:70–75

    Article  Google Scholar 

  2. Melis P, Shields CL, Marr BP et al (2009) Retinal pigment epithelial tumor in a young Asian female. Eur J Ophthalmol 3:487–489

    Google Scholar 

  3. Shields JA, Eagle RC Jr, Barr CC et al (1994) Adenocarcinoma of the retinal pigment epithelium arising from a juxtapapillary histoplasmosis scar. Arch Ophthalmol 112:650–653

    Article  CAS  Google Scholar 

  4. Shields JA, Shields CL, Gunduz K et al (1999) Neoplasms of the retinal pigment epithelium: the 1998 Albert Ruedemann, Sr, memorial lecture, part 2. Arch Ophthalmol 117(5):601–608

    Article  CAS  Google Scholar 

  5. Taomoto M, Nishimura T, Takahashi K et al (1999) A case of choroidal malignant melanoma treated by transcleral resection (in Japanese). Rinsho Ganka 53:1927–1932

    Google Scholar 

  6. Shields JA, Shields CL (2008) Tumors and related lesions of the pigment epithelium. In: Shields JA, Shields CL (eds) Intraocular tumors. An Atlas and Textbook, 2nd edn. Lippincott, Williams & Wilkins, Philadelphia, pp 432–483

    Google Scholar 

  7. Shields JA, Shields CL, Eagle RC Jr et al (2001) Adenocarcinoma arising from congenital hypertrophy of the retinal pigment epithelium. Arch Ophthalmol 119:597–602

    Article  CAS  Google Scholar 

  8. Trichopoulos N, Augsburger JJ, Schneider S (2006) Adenocarcinoma arising from congenital hypertrophy of the retinal pigment epithelium. Graefes Arch Clin Exp Ophthalmol 244:125–128

    Article  Google Scholar 

  9. Shields JA, Materin M, Shields CL et al (2001) Adenoma of the retinal pigment epithelium simulating a juxtapapillary choroidal neovascular membrane. Arch Ophthalmol 119:289–292

    CAS  PubMed  Google Scholar 

  10. Tso MOM, Albert DM (1972) Pathologic conditions of the retinal pigment epithelium: neoplasms and nodular non-neoplastic lesions. Arch Ophthalmol 88:27–38

    Article  CAS  Google Scholar 

  11. Ronne G (1945) Malignant tumor originated from the pigmentepithelium of the retina. Acta Ophthalmol 23:382–383

    Google Scholar 

  12. Stow MN (1949) Hyperplasia of the pigment epithelium simulating a neoplasm: report of two cases. Trans Am Acad Ophthalmol Otolaryngol 53:674–677

    CAS  PubMed  Google Scholar 

  13. Greer CH (1952) Epithelial tumours of the retinal pigment epithelium. Trans Ophthalmol Soc U K 72:265–277

    Google Scholar 

  14. Theobald GD, Floyd G, Kirk HQ (1958) Hyperplasia of the retinal pigment epithelium: simulating a neoplasm: report of two cases. Am J Ophthalmol 45:235–240

    Article  CAS  Google Scholar 

  15. Fair JR (1958) Tumors of the retinal pigment epithelium. Am J Ophthalmol 45:495–505

    Article  CAS  Google Scholar 

  16. Spiers F, Jensen OA (1963) Pseudo-epitheliomatous hyperplasia of the retinal pigment epithelium. Acta Ophthalmol 41:722–727

    Article  CAS  Google Scholar 

  17. Blodi FC, Reuling FH, Sornson ET (1965) Pseudomelanocytoma at the optic nerve head: an adenoma of the retinal pigment epithelium. Arch Ophthalmol 73:353–355

    Article  CAS  Google Scholar 

  18. Garner A (1970) Tumours of the retinal pigment epithelium. Br J Ophthalmol 54:715–723

    Article  CAS  Google Scholar 

  19. DeHaan AB (1971) Tumour of the pigmentepithelium. Ophthalmologica 163:26

    Article  CAS  Google Scholar 

  20. Shields JA, Eagle RC Jr, Shields CL et al (2009) Malignant transformation of congenital hypertrophy of the retinal pigment epithelium. Ophthalmology. 116(11):2213–2216. https://doi.org/10.1016/j.ophtha.2009.04.048

    Article  PubMed  Google Scholar 

  21. Font RL, Zimmerman LE, Fine BS (1972) Adenoma of the retinal pigment epithelium. Am J Ophthalmol 73:544–554

    Article  CAS  Google Scholar 

  22. Minckler D, Allen AW (1978) Adenocarcinoma of the retinal pigment epithelium. Arch Ophthalmol 96:2252–2254

    Article  CAS  Google Scholar 

  23. Laqua H (1981) Tumors and tumor-like lesions of the retinal pigment epithelium. Ophthalmologica 183:34–38

    Article  CAS  Google Scholar 

  24. Chamot L, Zografos L (1884) Tumeurs et pseudo-tumeurs de l’epithelium pigmentaire. J Fr Ophthalmol 7:825–836

    Google Scholar 

  25. Jampel HD, Schachat AP, Conway B et al (1986) Retinal pigment epithelial hyperplasia assuming tumor-like proportions: a report of two cases. Retina. 6:105–112

    Article  CAS  Google Scholar 

  26. Ramahefasolo S, Coscas G, Regenbogen L et al (1987) Adenocarcinoma of retinal pigment epithelium. Br J Ophthalmol 71:516–520

    Article  CAS  Google Scholar 

  27. Shields JA, Eagle RC Jr, Shields CL et al (1992) Pigmented adenoma of the optic nerve head simulating a melanocytoma. Ophthalmology. 99:1705–1708

    Article  CAS  Google Scholar 

  28. Fan JT, Robertson DM, Campbell RJ (1995) Clinicopathologic correlation of a case of adenocarcinoma of the retinal pigment epithelium. Am J Ophthalmol 119:243–245

    Article  CAS  Google Scholar 

  29. Finger PT, McCormick SA, Davidian M et al (1996) Adenocarcinoma of the retinal pigment epithelium: a diagnostic and therapeutic challenge. Graefes Arch Clin Exp Ophthalmol 234:22–27

    Article  Google Scholar 

  30. Wei W, Mo J, Jie Y et al (2010) Adenoma of the retinal pigment epithelium: a report of 3 cases. Can J Ophthalmol 45(2):166–170. https://doi.org/10.3129/i09-249

    Article  PubMed  Google Scholar 

  31. Edelstein C, Shields CL, Shields JA et al (1998) Presumed adenocarcinoma of the retinal pigment epithelium in a blind eye with a staphyloma. Arch Ophthalmol 116:525–528

    Article  CAS  Google Scholar 

  32. Shields JA, Shields CL, Ehya H et al (1993) Fine needle aspiration biopsy of suspected intraocular tumors: the 1992 Urwick Lecture. Ophthalmology. 100:1677–1684

    Article  CAS  Google Scholar 

  33. Shields JA, Shields CL, Shah P et al (1991) Partial lamellar sclerouvectomy for ciliary body and choroidal tumors. Ophthalmology. 98:971–983

    Article  CAS  Google Scholar 

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Funding

The National Natural Science Foundation of China (Nr. 81272981), the Beijing Natural Science Foundation (Nr. 7151003), and the priming scientific research foundation for the junior research in Beijing Tongren Hospital Capital Medical University (2017-YJJ-ZZL-009) provided financial support.

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Correspondence to Wenbin Wei.

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All authors declare that they have no conflict of interest.

Ethical approval

This study followed the tenets of the Declaration of Helsinki and its ethical standards of 1964. The study was approved by the Medical Ethics Committee of the Beijing Tongren Hospital, and written informed consent was obtained from all participants. All methods were performed in accordance with the relevant guidelines and regulations.

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The original version of this article was revised: The corresponding author's name of the article is Wenbin Wei, but it was misspelled as Weibin Wei.

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Zhou, N., Xu, X. & Wei, W. Analysis of clinical features of RPE adenoma. Graefes Arch Clin Exp Ophthalmol 258, 2831–2840 (2020). https://doi.org/10.1007/s00417-020-04784-8

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