Retinoblastoma in older children

Karcioglu, Zeynel A.; Abboud, Emad B.; Al-Mesfer, Saleh; Al-Rashed, Waleed; Pilapil, Delia H.

doi:10.1067/mpa.2002.120643

Cited by 28 publications

(30 citation statements)

References 15 publications

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“…The findings of our study from China on the frequency of a detection of retinoblastomas in older children are in agreement with results from large investigations in Western countries in which approximately 90% of all children with retinoblastoma are diagnosed before the age of 5 years [9,11,19]. As a corollary, our findings complement reports on the diagnosis of retinoblastoma in adults.…”

Section: Discussionsupporting

confidence: 82%

“…In previous large-scale studies, approximately 95% of all retinoblastomas had been diagnosed at an age of less than 5 years [1,2,3,4,5,6,7]. Retinoblastomas have only rarely been detected in children aged 5 years or older, and they may then present with atypical features, posing a diagnostic challenge and potentially leading to a masquerade syndrome [8,9,10,11,12,13,14]. It has also been discussed that retinoblastomas detected in children aged 5 years or older may have different biologic properties and a different prognosis from those of tumors when detected earlier in life [13].…”

Section: Introductionmentioning

confidence: 99%

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Retinoblastoma in Chinese Children Aged Five to Fourteen Years

Chang

Shi²,

Zhao³

et al. 2015

Ophthalmologica

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Objective: To assess the characteristics of retinoblastomas enucleated from Chinese children aged 5-14 years. Methods: This retrospective hospital-based study included all eyes with retinoblastomas consecutively enucleated in the Beijing Tongren Hospital between August 2003 and July 2013. Results: Out of 1,205 patients, 47 (3.9%) were 5 years or older. All tumors in this age group occurred unilaterally, the patients had a negative family history, and the tumors were detected at an age of 6.9 ± 1.8 years (range: 5-14). The main clinical features at the time of examining the as yet untreated children aged 5-7 years (n = 30) or >7-14 years (n = 10) were leukocoria, strabismus, pseudohypopyon, hypertension, vitreous seeds (‘snowballs'), and calcifications. In 12 patients (26%), the retinoblastoma had not initially been diagnosed as a tumor. Histopathology revealed tumor invasion into nonretinal tissue in 19 eyes (40%). Therapy included enucleation only (n = 22; 47%), adjuvant systemic chemotherapy (n = 24; 51%), and additional orbital exenteration (n = 1). After a mean follow-up of 3.0 ± 2.1 years (range: 0.2-9.8), which was done for 40 children, none of these children showed a tumor recurrence. Conclusions: Of the children undergoing enucleation for retinoblastoma in Beijing, 3.9% were aged 5 years or more. As in Western countries, the tumor occurrence was unilateral, their family history was negative, and the survival rate was relatively high in these children. In 1 out of 4 children, the tumor had initially been misdiagnosed due to a masquerade syndrome. Retinoblastoma should be considered in the differential diagnosis of any unclear intraocular situation in children.

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Section: Discussionsupporting

confidence: 82%

Section: Introductionmentioning

confidence: 99%

Retinoblastoma in Chinese Children Aged Five to Fourteen Years

Chang

Shi²,

Zhao³

et al. 2015

Ophthalmologica

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“…In our series, it was observed that 7.6% of children were older than 5 years at the time of initial diagnosis. Shields et al 12 and Karcioglu et al 13 have reported that 8.5% and 5.3% cases, respectively, were above 5 years of age at the time of diagnosis. Previous studies have observed that RB has no sex predilection 14 15.…”

Section: Discussionmentioning

confidence: 97%

Clinical presentation and survival of retinoblastoma in Indian children

et al. 2015

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“…Anteriorly located retinoblastoma can present without characteristic calcifications and is more common in older children compared with typical cases; it arises from the posterior retina in younger children. 2,3 Theoretically, positron-emission tomography might differentiate dysplasia and neoplasms, but this differentiation has not been confirmed for retinoblastoma. 4 However, findings of vitreous seeding or extension into the iris and anterior chamber may help to diagnose retinoblastoma.…”

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confidence: 99%

Retinal Dysplasia Mimicking Intraocular Tumor: MR Imaging Findings with Histopathologic Correlation

Graaf¹,

Valk²,

Moll

et al. 2007

American Journal of Neuroradiology

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SUMMARY:We report a 6-month-old boy who presented with unilateral leukocoria, retinal detachment, and a retrolental mass in a microphthalmic eye based on retinal dysplasia with concurrent optic nerve aplasia. Dysplastic retinal tissue, a rare congenital defect, may create a clinical and radiologic picture of an intraocular mass closely resembling tumor tissue. MR imaging findings with histopathologic correlation are presented to facilitate discrimination of the more common causes of leukocoria. Retinal dysplasia is a rare cause of childhood leukocoria, which can cause considerable diagnostic difficulty in the differentiation of benign and malignant intraocular pathology. When clinical diagnosis is uncertain, ocular MR imaging helps to characterize and differentiate between intraocular pathologies. However, in contrast to many lesions in the differential diagnosis of leukocoria, to our knowledge, detailed MR imaging findings in retinal dysplasia have not been previously reported. We report MR imaging findings of unilateral retinal dysplasia with concurrent optic nerve aplasia. Case ReportA 6-month-old boy was admitted with a history of a smaller left eye since birth and leukocoria for 1 month. He was born to nonconsanguineous parents at 41 weeks' gestation. Ophthalmic examination revealed no abnormalities in the right eye. The left eye was microphthalmic, with a corneal diameter of 9 mm (right, 10 mm). A yellowish-white retrolental mass, projecting from the inferomedial quadrant into the vitreous, was present (Fig 1). The mass in combination with retinal detachment obscured clear visualization of optic nerve disc and macula. Prominent irregular feeder vessels and small focal hemorrhages covered the surface of the mass. Differential diagnosis based on these findings included ciliary body medulloepithelioma, anteriorly located retinoblastoma, and persistent hyperplastic primary vitreous (PHPV).Ultrasonography showed a noncalcified mass with high reflectivity. MR imaging showed a mass arising from the nasal ciliary body region with high signal intensity (SI) on T1-weighted (T1WI) and low SI on T2-weighted (T2WI) images, combined with a normal-appearing vitreous and tent-shaped retinal detachment with subretinal exudate (Fig 2). The anterior chamber and lens showed no abnormalities. The right orbit, optic nerve, and portions of the optic chiasm related to the nerve were normal. The optic nerve and sheath complex were not visible within the left orbit or anywhere along the usual course of the nerve (Fig 2). Total aplasia of the left optic nerve was not combined with other congenital central nervous system (CNS) abnormalities. The mass showed intense homogeneous enhancement. Ultrasonography and MR imaging findings were more consistent with medulloepithelioma or PHPV, and a retinoblastoma could be ruled out. Five weeks later, enucleation was required due to enlargement of the mass with an increase of intraocular pressure, shallowness of the anterior chamber, and progression toward a phthisis bulbi.Histopathologic examin...

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Retinoblastoma in older children

Cited by 28 publications

References 15 publications

Retinoblastoma in Chinese Children Aged Five to Fourteen Years

Retinoblastoma in Chinese Children Aged Five to Fourteen Years

Clinical presentation and survival of retinoblastoma in Indian children

Retinal Dysplasia Mimicking Intraocular Tumor: MR Imaging Findings with Histopathologic Correlation

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