RPE Adenocarcinoma Arising from a Congenital Hypertrophy of the RPE (CHRPE) Treated with Proton Therapy

Introduction

Congenital hypertrophy of the retinal pigment epithelium (CHRPE) can present as a solitary or multiple flat pigmented fundus lesions which may slightly enlarge over years [1], [2]. In a series of 330 solitary CHRPE, a flat, horizontal enlargement could be noticed in 83 %, after more than three years. In this study [2], the median growth rate was estimated at 2 µm/mm base lesion/month. In some rare cases, a nodular, vertical growth in a CHRPE has been described [3], [4], [5], [6]. Only three of these underwent a histopathological analysis diagnosing an RPE adenocarcinoma arising from CHRPE [4], [5], [6].

We report the clinico-pathological findings of an RPE adenocarcinoma arising from CHRPE that – to the best of our knowledge – was the first to be, successfully, treated with proton beam radiotherapy.

• References

• 1 Chamot L, Zografos L. [Tumors and pseudotumors of the pigment epithelium]. J Fr Ophtalmol 1984; 7: 825-836
• 2 Shields CL, Mashayekhi A, Ho T et al. Solitary congenital hypertrophy of the retinal pigment epithelium: clinical features and frequency of enlargement in 330 patients. Ophthalmology 2003; 110: 1968-1976
• 3 Shields JA, Shields CL, Singh AD. Acquired tumors arising from congenital hypertrophy of the retinal pigment epithelium. Arch Ophthalmol 2000; 118: 637-641
• 4 Shields JA, Shields CL, Eagle jr. RC et al. Adenocarcinoma arising from congenital hypertrophy of retinal pigment epithelium. Arch Ophthalmol 2001; 119: 597-602
• 5 Trichopoulos N, Augsburger JJ, Schneider S. Adenocarcinoma arising from congenital hypertrophy of the retinal pigment epithelium. Graefes Arch Clin Exp Ophthalmol 2006; 244: 125-128
• 6 Shields JA, Eagle jr. RC, Shields CL et al. Malignant transformation of congenital hypertrophy of the retinal pigment epithelium. Ophthalmology 2009; 116: 2213-2216
• 7 Shields JA, Shields CL, Gunduz K et al. Neoplasms of the retinal pigment epithelium: the 1998 Albert Ruedemann, Sr, memorial lecture, Part 2. Arch Ophthalmol 1999; 117: 601-608
• 8 Shields JA, Eagle jr. RC, Barr CC et al. Adenocarcinoma of retinal pigment epithelium arising from a juxtapapillary histoplasmosis scar. Arch Ophthalmol 1994; 112: 650-653
• 9 Loeffler KU, Kivela T, Borgmann H et al. Malignant tumor of the retinal pigment epithelium with extraocular extension in a phthisical eye. Graefes Arch Clin Exp Ophthalmol 1996; 234 (Suppl. 01) S70-S75
• 10 Heindl LM, Naumann GO, Kruse FE et al. Aggressive metastasising adenocarcinoma of the retinal pigment epithelium with trisomy 21. Br J Ophthalmol 2008; 92: 389-391
• 11 Guerin EP, Wong D, Silvestri G et al. Cytokeratin subtyping to distinguish reactive and neoplastic RPE cells. Br J Ophthalmol 2006; 90: 801-802