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Chemoreduction for retinoblastoma. Analysis of tumor control and risks for recurrence in 457 tumors.
PURPOSE: To evaluate retinoblastoma control following chemoreduction. DESIGN: Interventional case series. METHODS: Prospective. SETTING: Single center trial. PATIENT POPULATION: 457 retinoblastomas in 193 eyes of 125 patients. Nonrandomized, noncomparative study. INTERVENTION: All patients received intravenous vincristine, etoposide, and carboplatin,. The tumors were managed with chemoreduction alone (group W) or chemoreduction combined with thermotherapy (group X), cryotherapy (group Y), or both thermotherapy and cryotherapy (group Z). MAIN OUTCOME MEASURE: Tumor recurrence in each treatment group. RESULTS: Of 457 retinoblastomas, 63 (14%) were in group W, 256 (56%) in group X, 127 (28%) in group Y, and 11 (2%) in group Z. The tumor was located in the macula in 33 (52%) of group W, 109 (43%) of group X, 3 (2%) of group Y, and 9 (1%) of group Z. The mean tumor thickness at initial examination was 7 mm for group W, 4 mm for group X, 2 mm for group Y, and 3 mm for group Z. Using Kaplan-Meier estimates, recurrence of the individual retinoblastoma at 7 years was found in 45% of group W and 18% for combined groups X, Y, and Z. Risk factors predictive of tumor recurrence by multivariate analysis included macular tumor location for all groups and additionally female gender for group W and increasing tumor thickness for groups X, Y, and Z. CONCLUSIONS: Chemoreduction alone or combined with cryotherapy or thermotherapy is effective for treatment of retinoblastoma, but tumor recurrence rate is highest for those located in the macula and those with greater thickness.
Beta-ray brachytherapy with 106Ru plaques for retinoblastoma.
PURPOSE: A retrospective analysis of 134 patients who received (106)Ru brachytherapy for retinoblastomas (175 tumors in 140 eyes). Treatment and follow-up were analyzed with special emphasis on tumor control organ, preservation, and late complications. RESULTS: Treated tumors had a mean height and diameter of 3.7+/-1.4 mm and 5.0+/-2.8 disk diameters, respectively. The radiation dose values were recalculated according to the calibration standard recently introduced by the National Institute of Standards and Technology. The recalculation revealed a mean applied dose of 419 Gy at the sclera (SD, 207 Gy) and 138 Gy (SD, 67 Gy) at the tumor apex. The 5-year tumor control rate was 94.4%. Tumor recurrence was more frequent in eyes with vitreous tumor cell seeding or fish-flesh regression. The estimated 5-year eye preservation rate was 86.5%. Previous treatment by brachytherapy or external beam radiotherapy, as well as a large tumor diameter, were significant factors for enucleation. The radiotherapy-induced complications after 5 years of follow-up were retinopathy (22%), optic neuropathy (21%), and cataract (17%). These complications were significantly more frequent after prior brachytherapy or external beam radiotherapy. CONCLUSION: Brachytherapy using (106)Ru plaques is a highly efficient therapy with excellent local tumor control and an acceptable incidence of side effects.
Macular retinoblastoma: evaluation of tumor control, local complications, and visual outcomes for eyes treated with chemotherapy and repetitive foveal laser ablation.
OBJECTIVE: To determine tumor control rates, complication rates, and visual acuity (VA) for patients with macular retinoblastoma undergoing systemic chemotherapy and foveal diode laser ablation. DESIGN: Noncomparative retrospective case series. PARTICIPANTS: All patients with retinoblastoma in the macula at the Bascom Palmer Eye Institute between 1991 and 2004 were evaluated. Patients included in the study were managed by the same clinician with a planned therapeutic strategy. METHODS: Patients with Reese-Ellsworth groups I to IV disease underwent 4 to 9 cycles of systemic chemotherapy with vincristine, etoposide, and carboplatin, and patients with group V disease underwent 6 to 10 cycles with or without cyclosporine. All tumors underwent repetitive diode laser ablation (2-24 sessions) applied to the foveal and extrafoveal portions of tumors at each visit until tumors were deemed inactive for at least 6 months. MAIN OUTCOME MEASURES: Recurrence requiring external beam radiation therapy or enucleation; VA; and complications including iris atrophy, peripheral focal lens opacity, peripheral anterior synechiae, and foveal neovascular membrane. RESULTS: Forty-four eyes of 33 patients were treated. Eyes were classified as Reese-Ellsworth group I (1 [2%]), II (6 [12%]), III (3 [7%]), IV (5 [9%]), or V (29 [67%]). Thirty-eight eyes (86%) had successful tumor control and avoided enucleation at a median follow-up of 36 months. At 3 years, tumor control rates were 100% for Reese-Ellsworth groups I to IV and 83% for group V. All eyes requiring enucleation were in Reese-Ellsworth group V. No eyes required external beam radiation. The most common complications were iris atrophy (61%) and focal lens opacity (14%). Strabismus was noted in 16% of eyes. Snellen VA measured 20/40 or better in 36% of eyes, 20/80 or better in 57%, and 20/400 or better in 86%. An increasing number of laser applications and chemotherapy cycles was not associated with decreased VA, strabismus, or development of an afferent pupillary defect but was associated with development of local complications. CONCLUSIONS: A unique approach to the application of diode laser therapy characterized by repetitive foveal treatments and aggressive chemotherapy resulted in tumor control rates that exceed those previously published. Furthermore, despite laser application directly to the fovea, 57% of patients retained 20/80 or better vision.
Second nonocular tumors in survivors of heritable retinoblastoma and prior radiation therapy.
PURPOSE: The principal objectives of this study were to estimate the incidence of second tumors among children treated for heritable retinoblastoma during a 50-year period and to investigate the relationship between these tumors and previous radiation therapy. METHODS: The records of all retinoblastoma patients examined at the Mayo Clinic from 1941 through 1990 were retrospectively reviewed. The therapeutic modality used to manage the tumor, the occurrence of any second malignancy, and current follow-up on all patients were evaluated. RESULTS: Eighty-two (46%) of 180 children with retinoblastoma had bilateral tumors (76 patients) or unilateral disease and a positive family history (six patients) and were followed for an average of 21.8 years (range, 1 month to 53 years). The Kaplan-Meier estimates of second nonocular tumors among the 82 patients with heritable retinoblastoma were 12% at 10 years, 16% at 25 years, and 30% at 40 years. Although 14 of the 15 patients who developed second malignancies had received radiation therapy, only four of the malignancies occurred within the field of irradiation. CONCLUSIONS: The relatively low incidence of second tumors among long-term survivors of heritable retinoblastoma in this series of patients occurred predominantly outside the field of irradiation. The variable incidence of second nonocular malignancies in previous reports may reflect variations in radiation technique and dosage.
Lymphoma mimicking scleritis.
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Plaque radiotherapy for retinoblastoma: long-term tumor control and treatment complications in 208 tumors.
OBJECTIVE: To evaluate the clinical factors predictive for tumor recurrence and treatment complications in a large series of children who underwent plaque radiotherapy for retinoblastoma. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: The participants included 141 children with retinoblastoma who were managed on the Oncology Service at Wills Eye Hospital with plaque radiotherapy between July 1976 and June 1999. MAIN OUTCOME MEASURES: Tumor recurrence and treatment complications. RESULTS: There were 208 tumors managed with plaque radiotherapy. The mean patient age at plaque treatment was 19 months. Prior treatment to the retinoblastoma of concern was delivered to 148 tumors (71%) and included various combinations of treatments such as intravenous chemoreduction, external beam radiotherapy, laser photocoagulation, thermotherapy, and cryotherapy. For 72 retinoblastomas (35%), more than one therapeutic method had failed to achieve tumor control before the use of plaque radiotherapy. Of the 208 retinoblastomas managed with plaque radiotherapy, Kaplan-Meier estimates of tumor control were 83% at 1 year and 79% at 5 years. Of the 60 tumors treated only with plaque radiotherapy (primary treatment), recurrence at 1 year was 12%. Of the 148 tumors treated after failure of other methods (secondary treatment), specific Kaplan-Meier estimates of tumor recurrence at 1 year was detected in 8% of tumors previously treated with chemoreduction, 25% of tumors previously treated with external beam radiotherapy, 34% tumors previously treated with both chemoreduction and external beam radiotherapy, and 8% of tumors previously treated with laser photocoagulation, thermotherapy, or cryotherapy (methods other than chemoreduction and external beam radiotherapy). Using multivariable analysis, the risks for tumor recurrence included the presence of tumor seeds in the vitreous, presence of subretinal tumor seeds, and increasing patient age. Using Kaplan-Meier estimates, radiation complications at 5 years of follow-up included nonproliferative retinopathy in 27%, proliferative retinopathy in 15%, maculopathy in 25%, papillopathy in 26%, cataract in 31%, glaucoma in 11%, and scleral necrosis in 0%. CONCLUSIONS: Plaque radiotherapy for retinoblastoma provides tumor control in 79% of cases at 5 years of follow-up. It is particularly useful for those tumors that fail treatment with chemoreduction, laser photocoagulation, thermotherapy, and cryotherapy. Tumors in young patients without vitreous or subretinal seeding show the best long-term control.
Alternative treatment in retinoblastoma.
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Disseminated retinoblastoma successfully treated with myeloablative chemotherapy--implication for molecular detection of minimal residual disease.
A useful marker for detecting minimal residual disease (MRD) has not been established yet in retinoblastoma. We assessed neuroendocrine protein gene product 9.5 (PGP9.5) expression, one of the markers for detecting MRD in neuroblastoma, in a patient with disseminated retinoblastoma. A 3-year-old boy with disseminated retinoblastoma in multiple bones and marrow was referred to our hospital. He received intensive treatment and has maintained CR for 48 months following myeloablative chemotherapy with hematopoietic stem cell transplantation (SCT). PGP9.5 expression was serially assessed by RT-PCR in peripheral blood mononuclear cells (PBMC), bone marrow cells (BMC) and mobilized peripheral blood stem cells (PBSC). Initially, his BMC consisted of 96% tumor cells which were proved to express PGP9.5 by RT-PCR. Moreover, PBMC were found to be positive for PGP9.5 indicating the presence of tumor cells in the peripheral blood. After intensive chemotherapy, PGP9.5 expression became negative in both PBMC and BMC. Prior to SCT, PBSC and BMC transplants were confirmed negative for PGP9.5 expression. It is suggested that PGP9.5 expression is a useful marker for evaluating therapeutic effects as well as detecting MRD in retinoblastoma.
Multifocal osteosarcoma following retinoblastoma.
Three survivors of retinoblastoma, one with hereditary bilateral and two with nonhereditary (spontaneous) unilateral disease, developed multifocal osteosarcoma. For one patient, unilateral retinoblastoma was followed by primitive neuroepithelioma at age 13 years. Multifocal chondroblastic osteosarcoma represented the patient's third malignant neoplasm. The course of multifocal osteosarcoma in these three patients compares to that of multifocal osteosarcoma which presents de novo in other patients without prior retinoblastoma.
Osteosarcoma following retinoblastoma: age at onset and latency period.
In order to assess the role of genetic predisposition in the induction of radiation-induced tumors, we performed statistical analysis on data from the literature and from our own Institute with regard to the age at onset and the latency period of osteosarcoma as the second primary tumor for retinoblastoma with or without subsequent radiotherapy. In retinoblastoma survivors who subsequently developed osteosarcoma, the age at onset of retinoblastoma was young (average of 12 months) in both unilateral and bilateral forms. This suggests that all or almost all of the patients were genetically predisposed by a mutation of one allele of the RB1 gene. For retinoblastoma patients, osteosarcomas occurred 1.2 years earlier inside than outside the radiation field. The latency period between radiotherapy and osteosarcoma onset was 1.3 years shorter inside than outside the radiation field. Interestingly, a bimodal distribution of latency periods was observed for osteosarcomas arising inside, but not outside the radiation field: 40% occurred after a short latency, while the latency of the remaining 60% was comparable to that of osteosarcoma occurring outside the radiation field. This suggests that different mechanisms may be involved in radiocarcinogenesis. A radiation-induced mutation of the second RB1 allele may be the cause of osteosarcomas occurring after a short delay, while other genes may be affected in those occurring after a longer delay.
Lack of activity of oral etoposide for relapsed intraocular retinoblastoma.
BACKGROUND: Intravenous etoposide is widely used in multiagent chemotherapy regimens for intraocular retinoblastoma despite the lack of phase II data documenting its efficacy. Because oral etoposide has been found to be highly effective in patients with relapsed medulloblastoma and neuroblastoma who had previously received intravenous etoposide, we investigated its use for intraocular retinoblastoma. PROCEDURE: A pilot trial of oral etoposide (50 mg/m2/ day for 21 days) in five children (6 eyes) with relapsed refractory intraocular retinoblastoma was performed. All had previously received chemotherapy, including intravenous etoposide in four patients, and all had received radiation therapy. Three patients (3 eyes) had vitreous seeds. Response was evaluated after one cycle. RESULTS: No serious acute toxicity was encountered, and no responses were noted. Four patients (5 eyes) had progressive disease. Stable disease was noted in one eye without vitreous disease. One patient developed secondary acute myeloid leukemia 30 months after exposure to oral etoposide. CONCLUSIONS: Oral etoposide was not an effective agent in this population. The role of etoposide in the treatment of higher risk intraocular retinoblastoma deserves further study.
Imprinting diseases and IVF: Danish National IVF cohort study.
BACKGROUND: The aim of this study was to compare the frequency of imprinting diseases in children born after IVF with the incidence in naturally conceived children. METHODS: All singleton children born in Denmark from January 1, 1995 through December 31, 2001 were stratified into children born without and after IVF, and were followed from birth until the end of 2002 in the National Register of Patients and the Central Register of Psychiatric Diseases, which include all discharge diagnoses from somatic and psychiatric hospitals/clinics, respectively. Included in the study were malignancies, mental, behavioural and neurological diseases, congenital syndromes, and developmental disturbances. Only diagnosis codes potentially relevant for imprinting diseases were included. RESULTS: During the 7-year study period, 442,349 singleton non-IVF and 6052 IVF children were born. Mean follow-up time was 4.5 and 4.1 years for the two groups, respectively, corresponding to 2 million and 25 000 follow-up years. In the IVF/non-IVF cohort, we detected 0/72 children with cancer, 47/3766 with mental diseases, 72/3654 neurological diseases, 4/287 congenital syndromes and 96/6727 developmental disturbances, in a total of 219/14,506 clinical outcomes. The number of children with specific imprinting diseases in the non-IVF group was 54: 44 kidney cancers, five retinoblastoma, three Prader-Willi syndrome and two Russel-Silver syndrome. Anticipating the same occurrence in IVF children, the total expected number was calculated to be 0.74. The observed number in the IVF group was 0. We found a significantly increased risk of cerebral palsy in the IVF group, with a rate ratio (RR) (IVF:non-IVF) of 1.8 [95% confidence interval (CI) 1.2-2.8; P < 0.01], and of sleeping disturbances, with an RR 2.0 (95% CI 1.2-3.3). The incidence rate of childhood cancers, mental diseases, congenital syndromes and developmental disturbances was equal in the two groups. CONCLUSIONS: We found no indication of an increased risk of imprinting diseases after IVF, but an 80% increased risk of cerebral palsy. We observed equal frequencies of childhood cancers, mental diseases, congenital syndromes and developmental disturbances in the two groups. Danish register data do not support reports of an increased risk of imprinting diseases after IVF.
Parental age and risk of childhood cancers: a population-based cohort study from Sweden.
BACKGROUND: Frequent germ line cells mutations were previously demonstrated to be associated with aging. This suggests a higher incidence of childhood cancer among children of older parents. A population-based cohort study of parental ages and other prenatal risk factors for five main childhood cancers was performed with the use of a linkage between several national-based registries. METHODS: In total, about 4.3 million children with their parents, born between 1961 and 2000, were included in the study. Multivariate Poisson regression was used to obtain the incidence rate ratios (IRR) and 95% confidence interval (CI). Children <5 years of age and children 5-14 years of age were analysed independently. RESULTS: There was no significant result for children 5-14 years of age. For children <5 years of age, maternal age were associated with elevated risk of retinoblastoma (oldest age group's IRR = 2.39, 95%CI = 1.17-4.85) and leukaemia (oldest age group's IRR = 1.44, 95%CI = 1.01-2.05). Paternal age was significantly associated with leukaemia (oldest age group's IRR = 1.31, 95%CI = 1.04-1.66). For central nervous system cancer, the effect of paternal age was found to be significant (oldest age group's IRR = 1.69, 95%CI = 1.21-2.35) when maternal age was included in the analysis. CONCLUSION: Our findings indicate that advanced parental age might be associated with an increased risk of early childhood cancers.
Lifetime risks of common cancers among retinoblastoma survivors.
BACKGROUND: Compared with the general population, carriers of germline mutations in RB1 who survive retinoblastoma (i.e., hereditary retinoblastoma survivors) are at increased risk of early-onset second cancers, particularly sarcomas, brain tumors, and melanoma. However, their risks for the epithelial cancers that commonly occur after age 50 years are not known. METHODS: We used hospital records to identify British retinoblastoma survivors born between 1873 and 1950, a period when few British retinoblastoma patients received high-dose radiotherapy. Cancers and deaths were identified by linkage with national registration records. All statistical tests were two-sided. RESULTS: We could trace the cancer histories of 144 survivors of hereditary retinoblastoma. From age 25 to age 84, there were 58 subsequent cancers, for a cumulative cancer incidence of 68.8% (95% confidence interval [CI] = 48.0% to 87.4%) and a cumulative cancer mortality of 56.3% (95% CI = 40.5% to 73.3%). Only eight of the 58 cancers were of bone or soft tissue, in marked contrast to findings from contemporary studies of American patients treated with external beam radiotherapy, among whom most second tumors are sarcomas. Compared with the general population, hereditary retinoblastoma survivors had higher mortality from lung cancer (standardized mortality ratio [SMR] = 7.01, 95% CI = 3.83 to 11.76), bladder cancer (SMR = 26.31, 95% CI = 8.54 to 61.41), and all other epithelial cancers combined (SMR = 3.29, 95% CI = 1.64 to 5.89). The overall standardized mortality ratio for epithelial cancer was inversely proportional to the approximate square of age (exponent of age = -2.1, 95% CI = -3.6 to -0.7), declining from 11.32 (95% CI = 4.15 to 24.64) at age 25-44 to 2.83 (95% CI = 1.04 to 6.16) at age 65-84. CONCLUSIONS: Survivors of hereditary retinoblastoma who are not exposed to high-dose radiotherapy have a high lifetime risk of developing a late-onset epithelial cancer. Most of the excess cancer risks in hereditary retinoblastoma survivors might be preventable by limiting exposures to DNA damaging agents (radiotherapy, tobacco, and UV light).
Bilateral increased Tl-201 uptake of lacrimal glands caused by crying of a child during injection.
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Diffuse infiltrating retinoblastoma: an inherited case.
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De novo intraocular retinoblastoma development after chemotherapy in patients with hereditary retinoblastoma.
OBJECTIVE: Identification of incidence and risk factors for recurrence of de novo retinoblastomas after chemotherapy treatment in patients with hereditary retinoblastoma. METHODS: A retrospective, case-control study of 32 patients (50 eyes) with sporadic or familial bilateral retinoblastomas was conducted. Patients received a systemic chemotherapy regimen applying three courses of a combination of three drugs (including vincristine, etoposide, carboplatin, or cyclophosphamide) followed by additional local therapy. The primary outcome analyzed was the development of retinoblastomas, probably arising as the cause of a new mutational event (de novo) after completion of chemotherapy treatment. RESULTS: Patients were treated with an average of 5.8 +/- 1.8 chemotherapy courses (4.6 +/- 2.4-year follow-up time). Development of de novo tumors occurred in 48% of the treated eyes. These tumors occurred during chemotherapy treatment or within 7 months of chemotherapy completion. No de novo tumors developed in patients older than 3.2 years. Children who developed de novo tumors were significantly younger at the time of diagnosis (6.7 +/- 6.3 months vs 14.4 +/- 11.4 months, P < 0.001), and had a significantly lower number of tumors per eye at treatment begin (2.6 +/- 2.3 tumors vs 4.3 +/- 6.4 tumors, P < 0.001). The difference of the total numbers of retinoblastomas that developed per eye between the patients that developed de novo retinoblastomas during or after chemotherapy and patients who did not was not statistically significant (4.9 +/- 2.7 and 4.3 +/- 6.4, respectively, P = 0.8). No eye was lost because of de novo retinoblastoma development, and 92% of the eyes were preserved. CONCLUSIONS: De novo retinoblastomas developed both during and after completion of chemotherapy treatment. Younger children were at a significantly higher risk for developing de novo intraocular retinoblastomas. Good tumor control and eye preservation rates were achieved with regular and frequent control examinations in addition to the immediate treatment of de novo retinoblastomas.