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      Atypical Presentation of Bilateral Retinoblastoma with Floaters and Sub-Internal Limiting Membrane Seeds in an 11-Year-Old Asian Indian Male

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          Purpose: To report bilateral retinoblastoma in an 11-year-old child presenting with floaters and sub-internal limiting membrane (sub-ILM) seeds. Method: An 11-year-old child presented with floaters of 4 months’ duration. Examination revealed circumscribed, whitish, subhyaloid, floccular nodules in the juxtapapillary region of both eyes. No solid tumor was seen. Swept-source optical coherence tomography revealed moderately reflective echoes in the sub-ILM space. Vitreous aspiration of sub-ILM deposits and cytopathological examination revealed retinoblastoma. Intravitreal melphalan (20 μg/0.02 mL) was followed by 6 monthly cycles of high-dose systemic chemotherapy (vincristine, etoposide, carboplatin). Metastatic workup and genetic testing for RB1 mutational screening were negative. Ocular lesions resolved after 13 weeks of treatment. Brain metastases developed after 20 weeks and were treated with radiation. Bilateral vitreous seeds recurred at 44 weeks and were treated with intravitreal chemotherapy (melphalan 20 μg/0.02 mL and topotecan 20 μg/0.02 mL). There was no extra-ocular spread from sclerotomy sites. Results: Retinoblastoma and brain metastases resolved with multimodal treatment without recurrence at 59 weeks of follow-up. Conclusion: Atypical manifestation of retinoblastoma can be seen in older children presenting with “floaters” and bilateral deposits of sub-ILM seeds, without solid tumor.

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          Author and article information

          Ocular Oncology and Pathology
          S. Karger AG
          September 2018
          14 February 2018
          : 4
          : 5
          : 309-312
          aShree Bhagwan Mahavir Vitreoretinal Services, Sankara Nethralaya, Chennai, India
          bLarson and Toubro Department of Ocular Pathology, Vision Research Foundation, Sankara Nethralaya, Chennai, India
          Author notes
          *Dr. Pukhraj Rishi, MD, FRCS, Medical Research Foundation, Sankara Nethralaya, No. 18, College Road, Nungambakkam, Chennai, Tamil Nadu 600016 (India), E-Mail docrishi@yahoo.co.in
          486341 PMC6167686 Ocul Oncol Pathol 2018;4:309–312
          © 2018 S. Karger AG, Basel

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          Figures: 3, Pages: 4
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