Skip to main content
Log in

Malignant tumor of the retinal pigment epithelium with extraocular extension in a phthisical eye

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


• Background: Malignant tumors of the retinal pigment epithelium (RPE) are exceedingly rare. We describe the histopathologic and immunohistochemical features of a RPE neoplasm that was found accidentally in a blind and painful phthisical eye. • Methods: The enucleated eye was investigated by light microscopy, and tumor tissue was also studied by electron microscopy. Immunohistochemistry was performed using antibodies against HMB-45, S-100 protein, NSE, cytokeratins, vimentin, desmin, GFAP, the HNK-1 carbohydrate epitope and α-smooth muscle actin. • Results: The tumor was located mainly in the vitreous cavity with practically complete destruction of the retina, but foci of choroidal infiltration and extraocular extension along vascular channels were identified. The mitotic rate was high, and large areas of necrosis were present. No features of differentiation were seen, apart from occasional desmosome-like junctions and deposition of basal lamina at the ultrastructural level. Adjacent to the tumor, reactive hyperplasia and metaplasia of RPE cells was prominent. By immunohistochemistry, tumor cells revealed intense immunoreactivity with anti-vimentin and weak staining with anti-S-100 protein. The hyperplastic RPE cells also reacted for cytokeratins 8, 18 and 19 and for α-smooth muscle actin. At more than 1 year post enucleation the patient is well and shows no signs of recurrence or metastatic disease. • Conclusion: We present the features of a malignant tumor of the RPE with unequivocal extraocular extension. These findings raise the possibility that RPE hyperplasia may transform into a malignant tumor.

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

Access this article

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

Instant access to the full article PDF.

Similar content being viewed by others


  1. Bradl M, Klein-Szanto A, Porter S, Mintz B (1991) Malignant melanoma in transgenic mice. Proc Natl Acad Sci USA 88: 164–168

    CAS  PubMed  Google Scholar 

  2. 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

    CAS  PubMed  Google Scholar 

  3. Font RL, Zimmerman LE, Fine BS (1972) Adenoma of the retinal pigment epithelium. Histochemical and electron microscopic observations. Am J Ophthalmol 73: 544–554

    CAS  PubMed  Google Scholar 

  4. Fuchs U, Kiveld T, Tarkkanen A (1991) Cytoskeleton in normal and reactive human retinal pigment epithelial cells. Invest Ophthalmol Vis Sci 32:3178–3186

    CAS  PubMed  Google Scholar 

  5. Fuchs U, Kivelä T, Summanen P, Immonen I., Tarkkanen A (1992) An immunohistochemical and prognostic analysis of cytokeratin expression in malignant uveal melanoma. Am J Pathol 141: 169–181

    CAS  PubMed  Google Scholar 

  6. Garner A (1970) Tumours of the retinal pigment epithelium. Br J Ophthalmo1 54:715–723

    CAS  Google Scholar 

  7. Kapur RJ, Bigler SA, Skelly M, Gown AM (1992) Anti-melanoma monoclonal antibody HMB45 identifies an oncofetal glycoconjugate associated with immature melanosomes. J Histochem Cytochem 40: 207–212

    CAS  PubMed  Google Scholar 

  8. McKechnie NM, Boulton M, Robey HL, et al (1988) The cytoskeletal elements of human retinal pigment epithelium: in vitro and in vivo. J Cell Sci 91:303–312

    PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  11. Rodrigues M, Hidayat A, Karesh J (1988) Pleomorphic adenocarcinoma of ciliary epithelium simulating an epibulbar tumour. Am J Ophthalmol 106:595–600

    CAS  PubMed  Google Scholar 

  12. Shields JA, Augsburger JJ, Wallar PH, Shah HG (1983) Adenoma of the nonpigmented epithelium of the ciliary body. Ophthalmology 90: 1528–1530

    CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  14. Tso MOM, Albert DM (1972) Pathological condition of the retinal pigment epithelium. Neoplasms and nodular non-neoplastic lesions. Arch Ophthalmol 88: 27–38

    CAS  PubMed  Google Scholar 

  15. Vogel MH, Woltz U (1979) Malignes Epitheliom des retinalen Pigmentepithels. Klin Monatsbl Augenheilkd 175:592–601

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations


Rights and permissions

Reprints and permissions

About this article

Cite this article

Loeffler, K.U., Kivehi, T., Borgmann, H. et al. Malignant tumor of the retinal pigment epithelium with extraocular extension in a phthisical eye. Graefe's Arch Clin Exp Ophthalmol 234 (Suppl 1), S70–S75 (1996).

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: