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Sameh E. Gaballah, Brenda L Gallie; Spatiotemporal evolution of germline retinoblastoma. Invest. Ophthalmol. Vis. Sci. 2018;59(9):1639. doi: https://doi.org/.
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© ARVO (1962-2015); The Authors (2016-present)
To correlate spatial (distribution) and temporal (timing) emergence of heritable retinoblastoma in the first 6 months of age and evaluate screening and systemic chemotherapy impact. Screening is scheduled examination of at risk eyes for retinoblastoma (H1 by TNMH staging; family history, detected RB1 mutation or fellow eye of germline unilateral presentation) prior to parental complaint.
Retrospective single institute review of H1 retinoblastoma children before 6 months of age from 1/1/2000 to 1/11/2017. Age, family history, screening or not, RB1 status, each tumor/eye (timing, site and size), and chemotherapy details were collected. Eyes with cT2 stage or higher were excluded due to imprecise tumor evaluation. A tumor was large if > 2 disc diameter and vision threatening if < 3 mm from the fovea.
The study included 76 eyes of 51 H1 children (23 familial, 20 screened) having 225 tumors (median 3/eye, range 1-9). Tumors were 55 central, 129 mid-peripheral and 41 peripheral in 47 (62%), 60 (79%) and 26 (34%) eyes and medians 102, 112 and 118 days of age, respectively with 33 visually threatening tumors at median 87 days of age. During the first month, 78% (18/23) of tumors were central. Screened eyes (34/76) had 104 tumors (3 large, 9 vision threatening); 12/34 eyes (35%) had all tumors concurrently (20/104, 19%) at diagnosis. Non-screened eyes (42/76) had 121 tumors (37 large, 24 vision threatening); 28/42 eyes (65%) had all tumors concurrently (72/124, 58%) at diagnosis. Tumors in non-screened eyes were significantly larger, vision threatening and concurrent (p= 0.001, 0.024, 0.001 respectively, Fisher exact test). Systemic chemotherapy was used to treat 39 eyes in 28 children; 28% (11/39) subsequently developed 17 tumors, median 171 days from diagnosis. Of non-chemotherapy treated eyes, 3/37 (8%) were early enucleated and 16 (44%) subsequently developed 37 tumors, median 100 days from diagnosis, significantly less than chemotherapy treated eyes (p=0.05, Mood's median test).
Earlier tumors were observed to be more central where they threatened vision, screening facilitated smaller tumor detection, and systemic chemotherapy was associated with decreased and/or delayed second tumors.
This is an abstract that was submitted for the 2018 ARVO Annual Meeting, held in Honolulu, Hawaii, April 29 - May 3, 2018.
Proportional scheme of central screened (green) and non-screened (yellow) tumors (birth-6 months).
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