1/20. retinoblastoma with acute lymphoblastic leukemia, polyposis coli, and multiple hamartomas.It has long been recognized that compared with their age- and sex-matched controls, survivors of hereditary retinoblastoma have a considerably higher risk of the development of second malignancies (10% at 20 years and 15% at 30 years of follow-up), including osteosarcoma, leiomyosarcoma, melanoma, fibrosarcoma, and other rare spindle cell sarcomas. patients with the nongenetic variety of retinoblastoma do not particularly seem to have an increased incidence of other malignancies than the general population. However, it should be noted that a child with unilateral disease carries a 15% chance of having germline mutation. The cumulative mortality rate from second malignancies was 1.5% at 40 years after unilateral retinoblastoma diagnosis and 26% for bilateral cases in a large survey of 1458 patients. A child with unilateral retinoblastoma, cafe au lait spots, hairy nevus, and grouped pigmentation of retina in the fellow eye is described who furthermore developed acute leukemia and polyposis coli.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
2/20. Fine structure of a radiation-induced osteogenic sarcoma.An osteogenic sarcoma arose in the right orbit of a 7-year-old boy some 5 years after the right orbit had been treated by four courses of radiotherapy (total dose approximately 13,000 rads) for a multicentric retinoblastoma. death occurred 6 months after the orbital tumor was first detected. Study of the orbital tumor by electron microscopy revealed a cell population of varied morphology in which two main types were identified. In one group, the cells were large with radiolucent cytoplasm, which contained long branching segments of rough endoplasmic reticulum. In the second group, the cells were smaller with irregular nuclei and an electron-dense cytoplasm, which contained short segments of dilated rough endoplasmic reticulum and numerous mitochondria. The first group of cells closely resembled osteoblasts, while the second group had some features of osteoclasts or their percursors. The branching processes of the tumor cells were separated by an amorphous ground substance, which contained collagen-like fibrils and hydroxyapatite crystals. Crystal deposition was in some instances in close relation to extracellular membrane-bound vesicles.- - - - - - - - - - ranking = 123.99110597345keywords = radiation-induced (Clic here for more details about this article) |
3/20. Dup(12)(q13-q22) and 13q14 deletion in a case of B-cell chronic lymphocytic leukemia.Cases with partial trisomy 12 have rarely been found in B-cell chronic lymphocytic leukemia (CLL). We report our clinical, cytogenetic and fluorescence in situ hybridization (FISH) findings in a CLL patient with a duplication of part of the long arm of chromosome 12 between bands q13-q22. This patient was the only case with this duplication among the 112 cases (0.9%) of CLL cytogenetically analyzed in our laboratory. FISH studies using unique-sequence specific probes for the RB-1 (retinoblastoma) gene and the D13S319 locus at the 13q14 band showed a monoallelic loss for the D13S319 locus (20% of cells) with a diploid RB-1 gene. Our case showed an atypical morphology (35% prolymphocytes), a high proliferation rate and progression of the disease, indicating that the duplication of this region may be equivalent to complete trisomy 12 in CLL patients.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
4/20. 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.- - - - - - - - - - ranking = 61.995552986727keywords = radiation-induced (Clic here for more details about this article) |
5/20. genome instability in secondary solid tumors developing after radiotherapy of bilateral retinoblastoma.genome alterations of seven secondary tumors (five osteosarcomas, one malignant peripheral sheath nerve tumor, one leiomyosarcoma) occurring in the field of irradiation of patients treated for bilateral retinoblastoma have been studied. These patients were predisposed to develop radiation-induced tumors because of the presence of a germ line mutation in the retinoblastoma gene (RB1). Tumor cells were characterized by a high chromosome instability whereas microsatellites and minisatellites were found to be stable. In all tumors, the normal RB1 allele was lost with the corresponding chromosome 13, whereas the germ line mutated allele was retained. The two alleles of TP53 were inactivated, one by deletion of the short arm of chromosome 17, the other by mutation. As compared with non-radiation-induced tumors, the observed panel of TP53 mutations was uncommon with sites not recurrently found otherwise and a high rate of deletions (3/7). In these predisposed patients, the loss of the single normal allele of RB1 is rather due to the radiation-induced chromosome instability than a direct effect of ionizing radiation.- - - - - - - - - - ranking = 92.99332948009keywords = radiation-induced (Clic here for more details about this article) |
6/20. New findings in the chromosome 13 long-arm deletion syndrome and retinoblastoma.New clinical and pathologic findings in patients with deletion of the long arm of chromosome 13 (13q-) include optic nerve hypoplasia and retinal dysplasia. fibroblasts derived from patients with a 13q- syndrome with and without retinoblastoma, as well as from familial and sporadic retinoblastoma, are a useful model for the study of genetic susceptibility to the development of spontaneous and radiation-induced cancers. fibroblasts from patients with hereditary retinoblastoma appear more radiosensitive than fibroblasts from patients with sporadic retinoblastoma or normal control patients.- - - - - - - - - - ranking = 30.997776493363keywords = radiation-induced (Clic here for more details about this article) |
7/20. Reconstruction of a complex hemifacial deformity with multiple simultaneous free-flap transfers: case report.Severe disfigurement, facial paralysis, abnormal continuity of oral and nasal passages, and velopharyngeal incompetence resulted, following maxillectomy and cranial-base resection for a radiation-induced sarcoma. Oral lining, bone support, facial muscle, and outer skin were provided with a single-staged transfer of radial forearm, scapular bone, scapular skin, and serratus anterior muscle with anastamoses to the contralateral neck. This combination of flaps may be considered in complex facial deformities, and may obviate the need for staged prefabrication.- - - - - - - - - - ranking = 30.997776493363keywords = radiation-induced (Clic here for more details about this article) |
8/20. Cataract surgery and intraocular lens implantation in a retinoblastoma case treated by external-beam radiation therapy.We report a case of extracapsular cataract extraction with posterior chamber intraocular lens implantation in a pediatric patient with a radiation-induced cataract that developed after combined external-beam radiation therapy and chemotherapy for retinoblastoma. A 12-year follow-up revealed effective tumor regression and sustained visual recovery.- - - - - - - - - - ranking = 30.997776493363keywords = radiation-induced (Clic here for more details about this article) |
9/20. bone marrow transplantation for therapy-related acute myeloid leukemia in congenital retinoblastoma associated with 13q deletion syndrome.Children with constitutional deletion of the long arm of chromosome 13 are at risk for retinoblastoma (RB) due to loss of the RB tumor suppressor gene. The prognosis is poor since the tumors are often bilateral, aggressive, and recurrent and the patients often harbor other congenital abnormalities. One further complication is that of therapy-related malignancies later in life. We report a case of allogeneic stem cell transplantation for therapy-related acute myeloid leukemia in an 8-year-old girl after multimodality treatment for refractory bilateral relapsing RB, with excellent outcome in both the ophthalmic and marrow disease.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
10/20. phacoemulsification surgery and foldable intraocular lens implantation in a child with regressed retinoblastoma.PURPOSE: The management of retinoblastoma has been shifting toward conservative treatment. Visual preservation has become a concern as tumor regression is achieved. To report the surgical approach and outcome of a radiation-induced cataract in an only eye with regressed retinoblastoma. methods: Single case report and review of literature. RESULTS: Small incision sutureless phacoemulsification and implant of an acrylic foldable intraocular lens was performed. Best-corrected visual acuity 1 year after surgery was 20/20. No tumor recurrence has been noted after 27 months of follow-up. CONCLUSIONS: phacoemulsification and foldable intraocular lens implant seemed to be an appropriate therapeutic option for this case.- - - - - - - - - - ranking = 30.997776493363keywords = radiation-induced (Clic here for more details about this article) |
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