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.

<b><i>Objective:</i></b> The aim of the study was to describe the clinical presentation, management strategies, and outcomes in a case series of primary lacrimal sac tumors. <b><i>Methods:</i></b> This retrospective study was conducted in Sheikh Fajilatunnessa Mujib Eye Hospital and Training Institute, Bangladesh, from July 1 to December 31, 2020, and included all patients who were evaluated, treated, and followed up for at least 6 months from January 2013 to October 2020. One patient developed a recurrence of the adenocarcinoma of the lacrimal sac after 1 year of primary treatment. Patients’ demographic data were analyzed and reviewed from published articles on lacrimal sac tumors. We assessed patients clinically, followed by radiological evaluation. We also analyzed the biopsy technique, treatment modality, and recurrence. An oncologist reviewed all patients to prepare a plan for adjuvant treatment. <b><i>Results:</i></b> Ten patients with lacrimal sac tumors were included in this study. Swelling in the medial canthal region was the most common presenting feature (100%), followed by epiphora (60%) and pain (30%). Open biopsy was preferred over fine-needle aspiration biopsy. Incisional biopsy or complete excisional biopsy was performed for all suspected malignancies. Malignant tumors were found in 7 (70%) cases, and benign tumors in 3 (30%) cases. Non-Hodgkin’s lymphoma (NHL) (40%) was the most common malignant lacrimal sac tumor. Mucosa-associated lymphoid tissue lymphoma was 75%, and diffuse large B-cell lymphoma was 25% among the cases of NHL. Patients with epithelial malignancy were treated with external beam radiation therapy, while NHL patients were treated with chemotherapy (CHOP regimen). Recurrence was noted in 1 case (10%) of epithelial malignancy after 1 year of treatment. <b><i>Conclusion:</i></b> Successful management of lacrimal sac tumors requires a high index of suspicion, as these are fatal tumors, often misdiagnosed as dacryocystitis. Nonepithelial malignancies are more predominant than epithelial malignancies, and hematolymphoid tumors are most frequent.

Patients with lacrimal sac rhinosporidiosis usually present with a soft, doughy swelling in the lacrimal sac area. Bathing in stagnant water is a common risk factor. A modified DCR yields excellent outcomes.

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Basal cell carcinoma (BCC) is a common eyelid skin tumour that can arise very rarely from the conjunctiva.• The pigmented variant of BCC accounts for 6% of skin BCCs but has never been reported in the conjunctiva. Novel Insights• This is the first report of a pigmented BCC of the conjunctiva, mimicking a conjunctival melanoma.• The pigmented BCC showed prominent melanocyte hyperplasia, melanophages and tumour cell cytoplasmic melanin. • Pigmented BCC should be considered in the differential diagnosis of pigmented, non-melanocytic tumours of the conjunctiva. AbstractA 60-year-old, South-Asian male patient presented with a dark brown lesion on his left conjunctiva surface near the limbus for 2 years. Examination showed a globular, dark brown-coloured mass near the temporal limbus at 4 to 5 o'clock position, measuring 3 × 3 × 2 mm. There were a few scattered flat pigmented lesions near the mass and also a few prominent vessels. The mass underwent wide local excision, with a clear margin, followed by double freeze cryotherapy of the conjunctival margins along with the application of Mitomycin C. The bare sclera was covered by amniotic membrane. Histology surprisingly revealed a nodular basal cell carcinoma (BCC) containing melanin. The melanin was present in the tumour cell cytoplasm, within melanophages and within dendritic, Melan A-positive melanocytes. The BCC was immunohistochemically positive for BerEP4 and negative for EMA. The clinically observed smaller nodules flanking the main pigmented BCC corresponded histologically to pigmented superficial growth pattern BCC connected to the under surface of the conjunctival epithelium. This is the first report of a pigmented BCC of the conjunctiva, mimicking a conjunctival melanoma. Pigmented BCC should be considered in the differential diagnosis of pigmented tumours of the conjunctiva.