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Junyang Zhao, Ningli Wang, Helen Dimaras, Christine Massey, Xiaolin Xu, Bin Li, Brenda L. Gallie; International Intraocular Retinoblastoma Classification Correlates with Pathological pTNM Staging. Invest. Ophthalmol. Vis. Sci. 2011;52(14):2469.
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Retinoblastoma is classified clinically at diagnosis by the International Intraocular Retinoblastoma Classification (IIRC) and after enucleation by pTNM stage. More severe pTNM stage indicates higher risk of metastasis. We sought to determine how IIRC predicted risk scored by pTNM and outcome of patients.
We performed a retrospective analysis of patients with advanced retinoblastoma, (IIRC Group D or E), diagnosed/enucleated between September 2006 and December 2009 in Beijing Tongren Retinoblastoma Center. Eligible patients received no treatment prior to enucleation. The extent of retinoblastoma invasion into optic nerve, choroid or anterior chamber on histopathology was summarized by pTNM stage. pTNM as determined by pathology reports was independently staged by authors. Stages pT3 and pT4 were considered high risk. Follow-up for all patients was within the last year except dead cases.
In total, 215 patients with advanced retinoblastoma were studied: 88 females, 127 males; 42 bilateral, 173 unilateral; 216 eyes analyzed (1 patient had both D/D eyes removed). Mean diagnosed age (MDA) was 26 months (mo) and mean follow-up time (MFUT) was 23 mo. The IIRC was Group D (68 eyes, MDA 20 mo, MFUT 22 mo) or Group E (148 eyes, MDA 28 mo, MFUT 24 mo). pTNM stage was pT1 (42 eyes: 16 D, 26 E), pT2 (140 eyes: 44 D, 96 E), pT3 (28 eyes: 8 D, 20 E), or pT4 (6 eyes: all E). High pathologic risk was scored in 12% (8/68) Group D and 18% (26/148) Group E eyes. Nine children died, all with Group E eyes; 2 died of chemotherapy complications, 1 died of the other unenucleated eye with advanced disease,6 died of metastasis resulted by enucleated eye. Overall survival was 95.8%.
Older children had worse IIRC classification at diagnosis. Group E eyes had higher pathologic risk (pT3 and pT4) than Group D (18% vs 12%) and higher mortality. IIRC correlates with pTNM and prognosis.
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