Rare. Considered either as a highly differentiated form of retinoblastoma (see this term) or as a benign (or spontaneously regressive) form of retinoblastoma (see retinoma). The age of onset is later than classic cases of retinoblastoma. The overall risk of transformation into retinoblastoma is estimated at 3-4 %.  Fundoscopic examination: grayish and translucent retinal mass, presence of calcifications, alterations of the pigmentary epithelium of the retina, chorioretinal atrophy with or without staphyloma (deformation of the sclera), few or no retinal exudate.

The main histological features of this tumor are:

-        the presence of photoreceptor cells (Homer-Wright or Flexner-Wintersteiner rosettes)

-        cytoplasm-rich cells and a better distributed nuclear chromatin

-        the absence of mitosis, which explains the resistance to chemotherapy

-        the presence of calcifications without signs of necrosis

Anesthetic implications: 

frequent ophthalmologic examinations

References : 

-        Shields CL, Srinivasan A, Lucio-Alvarez A, Shiels JA.
Retinocytoma/retinoma: comparative analysis of clinical features in 78 tumors and rate of transformation into retinoblastoma over 20 years.
J Am Ass Pediatr Ophthalmol Strabismus 2021 ; 25 :147.e1-147.e8

Updated: July 2022