Rachna Meel1,2, Seema Kashyap3,2, Sameer Bakhshi4,5, Mandeep Singh Bajaj1,2, Meenakshi Wadhwani2. 1. Oculoplastics and Ocular Oncology Service, New Delhi, India. 2. Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India. 3. Department of Ocular Pathology, All India Institute of Medical Sciences, New Delhi, India. 4. Department of Medical Oncology, All India Institute of Medical Sciences, New Delhi, India. 5. Dr. B.R.A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India.
Abstract
OBJECTIVE: The aim of this work was to study the clinical and histopathology features and treatment outcome in retinoblastoma cases presenting at an older age (>6 years). DESIGN: This was a retrospective study. We recruited 48 retinoblastoma patients who were treated at our institute over 7 consecutive years and were older than 6 years at presentation. METHODS: Medical records were reviewed for data, including age at diagnosis, gender, laterality, family history, lag time, first symptom, misdiagnosis, clinical findings, grade and stage of disease at diagnosis, treatment, outcome, and follow-up status. Histopathology slides were reviewed and assessed for the presence of histopathological high-risk features (HRF) for metastasis. The main outcome measures were the frequency of atypical clinical features like hyphema, pseudohypopyon, glaucoma, cataract, vitreous hemorrhage, and phthisis, and misdiagnosis, prior intervention, stage of disease at presentation, and treatment outcome. RESULTS: In total, 48/610 (7.8%) patients were older than 6 years, with a median age of 7 years (range 6-31). Retinoblastoma was bilateral in 7 cases. The most common initial symptom was white reflex followed by a decrease in vision. The median lag time was 9 months. Fourteen cases (29.2%) were misdiagnosed, with endophthalmitis the most common misdiagnosis. Twenty-six (54%) patients had intraocular disease, 12 (25%) had locally advanced disease, and 10 (21%) had metastatic disease at presentation. Overall, 67% (14/21) of the eyes that were enucleated upfront for presumed intraocular disease had histopathological HRF. At last follow-up, 31/36 (86%) who were treated were alive and healthy, while 5 (14%) patients had disease progression. CONCLUSIONS: This is the largest study of older age retinoblastoma and shows that it forms a significant percentage of retinoblastoma in developing countries, is misdiagnosed in one-third of cases, and may present at an advanced stage in 46% of cases.
OBJECTIVE: The aim of this work was to study the clinical and histopathology features and treatment outcome in retinoblastoma cases presenting at an older age (>6 years). DESIGN: This was a retrospective study. We recruited 48 retinoblastoma patients who were treated at our institute over 7 consecutive years and were older than 6 years at presentation. METHODS: Medical records were reviewed for data, including age at diagnosis, gender, laterality, family history, lag time, first symptom, misdiagnosis, clinical findings, grade and stage of disease at diagnosis, treatment, outcome, and follow-up status. Histopathology slides were reviewed and assessed for the presence of histopathological high-risk features (HRF) for metastasis. The main outcome measures were the frequency of atypical clinical features like hyphema, pseudohypopyon, glaucoma, cataract, vitreous hemorrhage, and phthisis, and misdiagnosis, prior intervention, stage of disease at presentation, and treatment outcome. RESULTS: In total, 48/610 (7.8%) patients were older than 6 years, with a median age of 7 years (range 6-31). Retinoblastoma was bilateral in 7 cases. The most common initial symptom was white reflex followed by a decrease in vision. The median lag time was 9 months. Fourteen cases (29.2%) were misdiagnosed, with endophthalmitis the most common misdiagnosis. Twenty-six (54%) patients had intraocular disease, 12 (25%) had locally advanced disease, and 10 (21%) had metastatic disease at presentation. Overall, 67% (14/21) of the eyes that were enucleated upfront for presumed intraocular disease had histopathological HRF. At last follow-up, 31/36 (86%) who were treated were alive and healthy, while 5 (14%) patients had disease progression. CONCLUSIONS: This is the largest study of older age retinoblastoma and shows that it forms a significant percentage of retinoblastoma in developing countries, is misdiagnosed in one-third of cases, and may present at an advanced stage in 46% of cases.
Authors: Guillermo Chantada; François Doz; Celia B G Antoneli; Richard Grundy; F F Clare Stannard; Ira J Dunkel; Eric Grabowski; Carlos Leal-Leal; Carlos Rodríguez-Galindo; Enrique Schvartzman; Maja Beck Popovic; Bernhard Kremens; Anna T Meadows; Jean-Michel Zucker Journal: Pediatr Blood Cancer Date: 2006-11 Impact factor: 3.167
Authors: Joaquim Caetano de Aguirre Neto; Célia Beatriz Gianotti Antoneli; Karina Braga Ribeiro; Marcus Simões Castilho; Paulo Eduardo R S Novaes; Martha M M Chojniak; Victor Arias Journal: Pediatr Blood Cancer Date: 2007-03 Impact factor: 3.167