Retinoblastoma in Children Older than 6 Years of Age.
Misdiagnosis
Older age
Retinoblastoma
Journal
Ocular oncology and pathology
ISSN: 2296-4681
Titre abrégé: Ocul Oncol Pathol
Pays: Switzerland
ID NLM: 101656139
Informations de publication
Date de publication:
Dec 2020
Dec 2020
Historique:
received:
19
12
2019
accepted:
21
05
2020
entrez:
15
1
2021
pubmed:
16
1
2021
medline:
16
1
2021
Statut:
ppublish
Résumé
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). 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. 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. 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. 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.
Identifiants
pubmed: 33447589
doi: 10.1159/000509040
pii: oop-0006-0395
pmc: PMC7772882
doi:
Types de publication
Journal Article
Langues
eng
Pagination
395-404Informations de copyright
Copyright © 2020 by S. Karger AG, Basel.
Déclaration de conflit d'intérêts
The authors have no conflicts of interest.
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