1/67. Establishment and characterization of a second primary osteosarcoma cell line (OSrb/N-M) from a patient cured of bilateral retinoblastoma.A cell line, designated OSrb/N-M, was established from the second primary osteosarcoma that developed in a 17-year-old Japanese female patient who had suffered from bilateral retinoblastoma at infancy. The OSrb/N-M cells grew as an adherent monolayer and retained some osteogenic biochemical phenotypes. In cytogenetic analyses, this cell line revealed many structural and numerical abnormalities, however, the bands q14 of both chromosomes 13 appeared to be normal, whereas the constitutional cells displayed normal female karyotypes. Immunoblot studies using monoclonal antibodies specific to RB protein demonstrated that the tumor cells did not express RB protein, suggesting that the OSrb/N-M cells might suffer from a loss-of-function mutation at this gene locus. Thus, this cell line is useful to study the molecular mechanism for the tumorigenesis of osteosarcoma with regard to an association with retinoblastoma.- - - - - - - - - - ranking = 1keywords = osteosarcoma, osteogenic, sarcoma (Clic here for more details about this article) |
2/67. 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.- - - - - - - - - - ranking = 1.2222966499445keywords = osteosarcoma, sarcoma (Clic here for more details about this article) |
3/67. Multifocal osteosarcoma as second tumor after childhood retinoblastoma.We present a case of multifocal osteosarcoma (MFOS) arising 11.5 years after successful treatment of bilateral retinoblastoma. The clinical, imaging and pathological findings at onset, after therapy, and during follow-up are described. Fluorescent in situ hybridization did not reveal a deletion of the RB-1 retinoblastoma gene, although the presence of an inactivating mutation invisible to this method cannot be ruled out. The MFOS may have been a second multifocal tumor associated with the original retinoblastoma or a post-irradiation sarcoma with extensive metastases.- - - - - - - - - - ranking = 0.76938530170391keywords = osteosarcoma, sarcoma (Clic here for more details about this article) |
4/67. 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 = 0.1691367677089keywords = osteosarcoma, sarcoma (Clic here for more details about this article) |
5/67. 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 = 0.44363704015127keywords = osteogenic, sarcoma (Clic here for more details about this article) |
6/67. Nonocular cancer in retinoblastoma survivors.From a review of the records of 2,302 patients with retinoblastoma collated from CPMC and AFIP, it was found that retinoblastoma patients who survived their original eye cancer have a high incidence of second nonocular malignancies. The second neoplasms occur almost exclusively (97.5%) in patients who have had bilateral retinoblastoma, although retinoblastoma is much more commonly unilateral. The second neoplasms have appeared between 1 and 42 years after the successful treatment of retinoblastoma and have been fatal in approximately 85% of cases. Seventy-one percent of patients develop tumors in the field of the radiation beam; many of these were following treatment with low doses of radiation and after short latent periods. Nineteen percent of patients develop tumors clearly out of the field of radiation (eg, osteogenic sarcoma of the femur). retinoblastoma patients appear to be unusually radio-sensitive to low doses of radiation and develop tumors in the field of radiation following treatment of 3,500 rads. They have a high incidence of nonocular tumors clearly distant from the radiation beam develop nonocular malignancies even if no radiation has been given. The incidence of multiple malignancies in patients successfully treated for retinoblastoma appears to be higher than for any other primary malignancy whether they receive radiation or not.- - - - - - - - - - ranking = 0.088727408030254keywords = osteogenic, sarcoma (Clic here for more details about this article) |
7/67. Orbital leiomyosarcoma after retinoblastoma.patients with the inherited, bilateral form of retinoblastoma have an increased incidence of osteogenic sarcoma such that the mortality from the secondary tumor exceeds that of the initial bilateral retinoblastoma. We report a 29-year-old male survivor of bilateral retinoblastomas originally diagnosed at 8 months of age, whose treatment eventually included bilateral enucleation, bilateral orbital radiation, and systemic chemotherapy. At age 26, a tumor removed from his right maxillary sinus was diagnosed as fibroma. At age 29, he developed an inferior orbital mass that extended into the right maxillary sinus. A biopsy and comparison with the previous maxillary sinus mass revealed both lesions to be leiomyosarcoma. Both light and electron microscopy supported the diagnosis. The patient has survived treatment with orbital exenteration and maxillectomy combined with postoperative radiation to the right orbital-maxillary area. This appears to be the fourth case of leiomyosarcoma in the third decade of life in a male patient with a previously irradiated orbit after enucleation for bilateral retinoblastoma. leiomyosarcoma appears to be another orbital tumor associated with bilateral retinoblastoma.- - - - - - - - - - ranking = 0.12687667645055keywords = osteogenic, sarcoma (Clic here for more details about this article) |
8/67. Osteogenic sarcoma after retinoblastoma radiotherapy.Development of osteogenic sarcoma after retinoblastoma radiotherapy in three patients, two of whom were siblings, is reported. Pluridirectional tomography and plain skull radiography demonstrated soft tissue masses, sinus opacification, and bone destruction and sclerosis in all three patients. Computed tomography reliably indicated the presence or absence of intracranial tumor extension in the two patients in whom it was performed. Radionuclide bone scanning was a useful adjunct for osteosarcomatous detection.- - - - - - - - - - ranking = 0.26331407122777keywords = osteosarcoma, osteogenic, sarcoma (Clic here for more details about this article) |
9/67. 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 = 1.0913091506559keywords = osteosarcoma, sarcoma (Clic here for more details about this article) |
10/67. leiomyosarcoma of the bladder fourteen years after cyclophosphamide therapy for retinoblastoma.We report a case of bladder leiomyosarcoma which occurred in a 22-year-old female who had been treated with cyclophosphamide for a period of 68 months for retinoblastoma diagnosed at 18 months postpartum. Partial cystectomy was performed. Forty-two months after the operation she is tumor-free.- - - - - - - - - - ranking = 0.027249477443068keywords = sarcoma (Clic here for more details about this article) |
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