Retinoma Underlying Retinoblastoma Revealed After Tumor Response to 1 Cycle of Chemotherapy

Management and outcome of unilateral retinoblastoma

“…Patients 11 and 14 each had retinoma underlying the active retinoblastoma after failed eye conservation therapy. [12][13][14] Of the 106 children primarily enucleated, 6 were IIRC group C, 56 group D, and 44 group E (Table 1). Histopathologic high-risk features were present in 17 of the enucleated eyes: 4 had massive choroidal involvement alone (TNM pT3a), 6 showed retrolaminar nerve involvement of which 1 was beyond the resection margin (TNM pT4), 3 had massive choroidal and optic nerve involvement (TNM pT3b), and 4 eyes had anterior segment involvement.…”

Management and outcome of unilateral retinoblastoma

“…Patients 11 and 14 each had retinoma underlying the active retinoblastoma after failed eye conservation therapy. [12][13][14] Of the 106 children primarily enucleated, 6 were IIRC group C, 56 group D, and 44 group E (Table 1). Histopathologic high-risk features were present in 17 of the enucleated eyes: 4 had massive choroidal involvement alone (TNM pT3a), 6 showed retrolaminar nerve involvement of which 1 was beyond the resection margin (TNM pT4), 3 had massive choroidal and optic nerve involvement (TNM pT3b), and 4 eyes had anterior segment involvement.…”

A White Retinal Lesion With Calcification in an 11-Year-Old Boy

“…1,2 Key features distinguishing retinoma from retinoblastoma include underlying chorioretinal changes, indicative of a chronic and quiescent state, and absence of vascularization or subretinal fluid. [1][2][3][4] Vitreous seeds may seldomly accompany retinomas as inactive spheres floating in the vitreous in front of the retina, carrying anuncertainclinicalsignificancethatcontraststoretinoblastomaseeds, known to represent worse disease staging and prognosis. 3,5 While retinoblastoma is mainly diagnosed in the first months or years of life by the presence of leukocoria and strabismus, retinomas can be detected incidentally during fundoscopy at any age, being more commonly discovered in relatives of patients with retinoblastoma.…”

“…Retinoma is a white-gray gelatinous retinal tumor with chalky calcification that resembles retinoblastoma but lacks aggressive intraocular or systemic behavior . Key features distinguishing retinoma from retinoblastoma include underlying chorioretinal changes, indicative of a chronic and quiescent state, and absence of vascularization or subretinal fluid . Vitreous seeds may seldomly accompany retinomas as inactive spheres floating in the vitreous in front of the retina, carrying an uncertain clinical significance that contrasts to retinoblastoma seeds, known to represent worse disease staging and prognosis …”

Characterization and pharmacologic targeting of EZH2, a fetal retinal protein and epigenetic regulator, in human retinoblastoma

“…In a prior study, Eagle et al evaluated photoreceptor differentiation in a cohort of enucleated eyes with retinoblastoma and found that photoreceptor differentiation was identified in tumors of all histologic grades and that the mean age of patients was 23 months 44 . The absence of EZH2 staining in the foci of photoreceptor differentiation is not surprising given photoreceptor differentiation is hypothesized to be a remnant of the precursor lesion (retinocytoma) rather than re-differentiation of the retinoblastoma 45 , 46 . In our clinicopathologic analysis, EZH2 protein was not correlated with histologic grade.…”

Management and outcome of unilateral retinoblastoma

“…Patients 11 and 14 each had retinoma underlying the active retinoblastoma after failed eye conservation therapy. [12][13][14] Of the 106 children primarily enucleated, 6 were IIRC group C, 56 group D, and 44 group E (Table 1). Histopathologic high-risk features were present in 17 of the enucleated eyes: 4 had massive choroidal involvement alone (TNM pT3a), 6 showed retrolaminar nerve involvement of which 1 was beyond the resection margin (TNM pT4), 3 had massive choroidal and optic nerve involvement (TNM pT3b), and 4 eyes had anterior segment involvement.…”

A White Retinal Lesion With Calcification in an 11-Year-Old Boy

“…1,2 Key features distinguishing retinoma from retinoblastoma include underlying chorioretinal changes, indicative of a chronic and quiescent state, and absence of vascularization or subretinal fluid. [1][2][3][4] Vitreous seeds may seldomly accompany retinomas as inactive spheres floating in the vitreous in front of the retina, carrying anuncertainclinicalsignificancethatcontraststoretinoblastomaseeds, known to represent worse disease staging and prognosis. 3,5 While retinoblastoma is mainly diagnosed in the first months or years of life by the presence of leukocoria and strabismus, retinomas can be detected incidentally during fundoscopy at any age, being more commonly discovered in relatives of patients with retinoblastoma.…”

“…Retinoma is a white-gray gelatinous retinal tumor with chalky calcification that resembles retinoblastoma but lacks aggressive intraocular or systemic behavior . Key features distinguishing retinoma from retinoblastoma include underlying chorioretinal changes, indicative of a chronic and quiescent state, and absence of vascularization or subretinal fluid . Vitreous seeds may seldomly accompany retinomas as inactive spheres floating in the vitreous in front of the retina, carrying an uncertain clinical significance that contrasts to retinoblastoma seeds, known to represent worse disease staging and prognosis …”

Characterization and pharmacologic targeting of EZH2, a fetal retinal protein and epigenetic regulator, in human retinoblastoma

“…In a prior study, Eagle et al evaluated photoreceptor differentiation in a cohort of enucleated eyes with retinoblastoma and found that photoreceptor differentiation was identified in tumors of all histologic grades and that the mean age of patients was 23 months 44 . The absence of EZH2 staining in the foci of photoreceptor differentiation is not surprising given photoreceptor differentiation is hypothesized to be a remnant of the precursor lesion (retinocytoma) rather than re-differentiation of the retinoblastoma 45 , 46 . In our clinicopathologic analysis, EZH2 protein was not correlated with histologic grade.…”

Management and outcome of unilateral retinoblastoma

“…Patients 11 and 14 each had retinoma underlying the active retinoblastoma after failed eye conservation therapy. [12][13][14] Of the 106 children primarily enucleated, 6 were IIRC group C, 56 group D, and 44 group E (Table 1). Histopathologic high-risk features were present in 17 of the enucleated eyes: 4 had massive choroidal involvement alone (TNM pT3a), 6 showed retrolaminar nerve involvement of which 1 was beyond the resection margin (TNM pT4), 3 had massive choroidal and optic nerve involvement (TNM pT3b), and 4 eyes had anterior segment involvement.…”

A White Retinal Lesion With Calcification in an 11-Year-Old Boy

“…1,2 Key features distinguishing retinoma from retinoblastoma include underlying chorioretinal changes, indicative of a chronic and quiescent state, and absence of vascularization or subretinal fluid. [1][2][3][4] Vitreous seeds may seldomly accompany retinomas as inactive spheres floating in the vitreous in front of the retina, carrying anuncertainclinicalsignificancethatcontraststoretinoblastomaseeds, known to represent worse disease staging and prognosis. 3,5 While retinoblastoma is mainly diagnosed in the first months or years of life by the presence of leukocoria and strabismus, retinomas can be detected incidentally during fundoscopy at any age, being more commonly discovered in relatives of patients with retinoblastoma.…”

“…Retinoma is a white-gray gelatinous retinal tumor with chalky calcification that resembles retinoblastoma but lacks aggressive intraocular or systemic behavior . Key features distinguishing retinoma from retinoblastoma include underlying chorioretinal changes, indicative of a chronic and quiescent state, and absence of vascularization or subretinal fluid . Vitreous seeds may seldomly accompany retinomas as inactive spheres floating in the vitreous in front of the retina, carrying an uncertain clinical significance that contrasts to retinoblastoma seeds, known to represent worse disease staging and prognosis …”

Characterization and pharmacologic targeting of EZH2, a fetal retinal protein and epigenetic regulator, in human retinoblastoma

“…In a prior study, Eagle et al evaluated photoreceptor differentiation in a cohort of enucleated eyes with retinoblastoma and found that photoreceptor differentiation was identified in tumors of all histologic grades and that the mean age of patients was 23 months 44 . The absence of EZH2 staining in the foci of photoreceptor differentiation is not surprising given photoreceptor differentiation is hypothesized to be a remnant of the precursor lesion (retinocytoma) rather than re-differentiation of the retinoblastoma 45 , 46 . In our clinicopathologic analysis, EZH2 protein was not correlated with histologic grade.…”