Retinoblastoma associated with orbital pseudocellulitis and high-risk retinoblastoma: a study of 32 eyes.

Abstract

To study the correlation between retinoblastoma (RB) associated with orbital pseudocellulitis and high-risk histopathology features. Retrospective study of 32 patients who underwent primary enucleation for RB presenting with orbital pseudocellulitis. All RB patients presented with orbital pseudocellulitis. The mean age at presentation of RB was 30months (median, 24months; range, 3-70months). There were 14 (44%) males and 18 (56%) females. All patients were referred with a diagnosis of RB with orbital pseudocellulitis. Tumor was bilateral in 12 (38%) patients but orbital pseudocellulitis was unilateral in all cases. The pseudocellulitis features included proptosis (n = 9; 28%), eyelid edema (n = 22; 69%), conjunctival congestion (n = 23; 72%), and conjunctival chemosis (n = 15; 47%). Based on clinical features and orbital imaging, all patients were diagnosed to have group E intraocular RB. All patients received intravenous steroids prior to enucleation. On histopathology, tumor necrosis was present in all cases with a mean % necrosis of 60% (median, 60%; range, 10% to 90%). Most tumors (72%) were poorly differentiated. High-risk histopathology features were noted in 23 (72%) cases and adjuvant chemotherapy was advised for all these patients. The most common high-risk histopathology features included post-laminar optic nerve infiltration (34%) and scleral infiltration (22%). Over a mean follow-up period of 34months (median, 9months; range, < 1-188months), there was no event of metastasis or death in any patient. RB presenting with orbital pseudocellulitis is associated with high incidence of high-risk histopathology features.