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.
Introduction:The close relationship between diseases of the paranasal sinuses system and the orbit is based on their anatomical relationship. About 60% to 80% of the osseous eye socket is made up by sinus walls.
Abstrak:Retinoblastoma merupakan keganasan intraokular tersering pada anak-anak. Deteksi dini memegang peranan penting dalam manajemen retinoblastoma karena sangat mempengaruhi prognosa. Namun, hal ini sulit dilakukan di negara berkembang dengan tingkat sosioekonomi penduduk yang rendah. Penelitian ini bertujuan untuk menggambarkan profil retinoblastoma di RS Dr. M. Djamil Padang tahun 2003-2012, dan menunjukkan hubungan antara faktor sosioekonomi dengan manajemen retinoblastoma. Data diperoleh dari rekam medis pasien. Terdapat 99 pasien retinoblastoma dalam periode tersebut, 56 orang (56,56%) laki-laki dan 43 orang (43,44%) perempuan.Rentang umur terbanyak yaitu 3-4 tahun (40,40%). Retinoblastoma unilateral terdapat pada 76 pasien (76,76 %). Protusio merupakan keluhan tersering sebanyak 65,66% (65 orang). Kombinasi eksenterasi dan kemoterapi merupakan tindakan yang umum dilakukan (32,32%). Alasan terbanyak datang terlambat untuk berobat disebabkan oleh karena tidak punya biaya (42,42%) dan tidak mengerti mengenai bahaya penyakitnya (35,35%). Kedua faktor tersebut menjadi dilemma, oleh karena di satu sisi kita berusaha mendeteksi dini penyakit retinoblastoma ini, sementara dari segi finansial dan faktor sosioekonomi keluarga penderita tidak mendukung. Kata kunci: retinoblastoma, manajemen penyakit, faktor sosioekonomi, keterlambatan diagnosis. Abstract: Retinoblastoma is the most common intraocular malignancy in children. Early detection plays an important role in management of
Background. Proptosis is a condition that describes a protrusion or forward shift of the eyeball and is commonly used to describe the condition of protrusion of the eyeball. Proptosis generally occurs due to a mismatch between the orbital capacity and the volume of the tissue structure in the orbital cavity. It is a common manifestation of a wide variety of diseases of the structures in and around the orbit and in some systemic diseases. This study aimed to describe the clinical profile of proptosis patients in Dr. M. Djamil General Hospital Padang. Methods. We reviewed the medical record of the oncology subdivision of ophthalmology patients associated with proptosis retrospectively, such as inflammatory, infectious, tumors, and vascular, admitted to the M.Djamil General Hospital in Padang from January 2019 to August 2020. The ocular and systemic history and examination details were recorded. Results. We found 48 proptosis patients from January 2019 to August 2020, consisting of 30(62.5%) males and 18(37,5%) females. The majority of subjects were in the Adult age group (79,17%). Unilateral and bilateral proptosis were found in 37(77%) and 11(23%), with mostly axial proptosis (83,33%). Inflammatory tumors were found in 17(35,41) and 29(60%) patients. Conclusion. Proptosis is a common clinical feature of various local and systemic diseases. Even though proptosis have diverse etiology and pathogenesis, they usually present with similar clinical manifestation. Proptosis may be one of the most important keys to disease guidance from a close structure that requires a multidisciplinary approach.
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