Global Retinoblastoma Study Group IMPORTANCE Early diagnosis of retinoblastoma, the most common intraocular cancer, can save both a child's life and vision. However, anecdotal evidence suggests that many children across the world are diagnosed late. To our knowledge, the clinical presentation of retinoblastoma has never been assessed on a global scale.OBJECTIVES To report the retinoblastoma stage at diagnosis in patients across the world during a single year, to investigate associations between clinical variables and national income level, and to investigate risk factors for advanced disease at diagnosis. DESIGN, SETTING, AND PARTICIPANTSA total of 278 retinoblastoma treatment centers were recruited from June 2017 through December 2018 to participate in a cross-sectional analysis of treatment-naive patients with retinoblastoma who were diagnosed in 2017. MAIN OUTCOMES AND MEASURESAge at presentation, proportion of familial history of retinoblastoma, and tumor stage and metastasis. RESULTSThe cohort included 4351 new patients from 153 countries; the median age at diagnosis was 30.5 (interquartile range, 18.3-45.9) months, and 1976 patients (45.4%) were female. Most patients (n = 3685 [84.7%]) were from low-and middle-income countries (LMICs). Globally, the most common indication for referral was leukocoria (n = 2638 [62.8%]), followed by strabismus (n = 429 [10.2%]) and proptosis (n = 309 [7.4%]). Patients from high-income countries (HICs) were diagnosed at a median age of 14.1 months, with 656 of 666 (98.5%) patients having intraocular retinoblastoma and 2 (0.3%) having metastasis. Patients from low-income countries were diagnosed at a median age of 30.5 months, with 256 of 521 (49.1%) having extraocular retinoblastoma and 94 of 498 (18.9%) having metastasis. Lower national income level was associated with older presentation age, higher proportion of locally advanced disease and distant metastasis, and smaller proportion of familial history of retinoblastoma. Advanced disease at diagnosis was more common in LMICs even after adjusting for age (odds ratio for low-income countries vs upper-middle-income countries and HICs, 17.92 [95% CI,, and for lower-middle-income countries vs upper-middle-income countries and HICs, 5.74 [95% CI,). CONCLUSIONS AND RELEVANCEThis study is estimated to have included more than half of all new retinoblastoma cases worldwide in 2017. Children from LMICs, where the main global retinoblastoma burden lies, presented at an older age with more advanced disease and demonstrated a smaller proportion of familial history of retinoblastoma, likely because many do not reach a childbearing age. Given that retinoblastoma is curable, these data are concerning and mandate intervention at national and international levels. Further studies are needed to investigate factors, other than age at presentation, that may be associated with advanced disease in LMICs.

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Purpose: Spontaneous regression is a rare but well-known feature of retinoblastoma, which is predicted in approximately 2 of every 100 cases. Unilateral, unifocal spontaneous regression of retinoblastoma is infrequent; bilateral multifocal spontaneous regression of retinoblastoma is even more extraordinary. In many of the previously reported cases, the eye (or eyes) containing the spontaneously regressed tumor has been phthisical and blind. Herein, we describe the case of a young woman with unilateral, spontaneously regressed retinoblastoma (RB), with inflamed, blind, and phthisis in the left eye as an uncommon presentation of spontaneously regressed retinoblastoma Methods: This is a case report: An 18-year-old woman came to the outpatient clinic with a chief complaint of pain in the left eye since 1 month prior and a history of dysfunctional “cat’s” eye since childhood.  Results: Exotropia and atrophy bulbi was noticed in the left eye. Anterior segment examination revealed signs of inflammation including conjunctival hyperemia, shallow anterior chamber, rubeosis iridis, and posterior synechia with white “chalky” mass suggestive calcification. B-scan ultrasonography showed a point like lesion and solitary solid intraocular mass with calcification in vitreus. Orbital computed tomography scan (CT-Scan) showed atrophy of the left eye with intraocular calcification without optic nerve and extraocular muscle involvement. Enucleation and histopathology examination were done and revealed undifferentiated retinoblastoma beyond the sign of metastasis in other parts of the eye. Conclusion: Blind painful eye may present as the main feature of spontaneously regressed retinoblastoma that possibly caused by an inflammatory reaction.