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Query: UMLS:C0027651 (tumor)
 685,946 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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.
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PMID:Osteogenic sarcoma after retinoblastoma radiotherapy. 11 16

Case reports of neuroblastoma revealed that some individuals are genetically predisposed and that this genetic predisposition may have other consequences. According to a mutation model, two classes of individuals could acquire neuroblastoma. One (prezygotic) was a rare class that carried a dominant gene imparting high risk of the tumor. The other (postzygotic) comprised all other individuals, each at low risk. The model related tumor incidence to germinal and somatic mutation rates and thereby carried implications for environmental modification of tumorigenesis and demographic variation in incidence. Case reports also revealed associations of neuroblastoma with congenital defects and a susceptibility to second tumors. Analogy with retinoblastoma and Wilms' tumor of the kidney suggested that these associations could result from action of a neuroblastoma gene or from chromosomal aberration. One known dominantly inherited condition, von Recklinghausen's disease, could dispose to neuroblastoma and create some associations. According to the two-mutation model, neuroblastoma may have been a single recessive gene disorder at the level of the cell. The phenomena of aganglionosis, neuroblastoma in situ, maturation of neuroblastoma to ganglioneuroma, and spontaneous regression suggested that such a neuroblastoma gene interfered with normal developmental processes. The specificities of this gene and of those for von Recklinghausen's disease and pheochromocytoma suggested that the functiof a membrane macromolecule.
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PMID:Developmental genetics of neuroblastoma. 18 2

All known tumor types have been reported in the neonate. A numerical listing and discussion are beyond the scope of this review. Wells and Fraumeni give some insight into common congenital malignant neoplasms. Table 2 lists the percentage of neonatal deaths caused by type-specific cancers. Retinoblastoma is probably the most common malignant tumor in the neonate. About seven per cent of these tumors have been apparent at birth. This tumor is not discussed in either article because it is not lethal until muypes in neonatal and pediatric patients. Some congenital malformations in the in the neonate are recognized as being frankly benign (cysts), potentially malignant (teratomas), and frankly malignant (neuroblastoma). A high percentage of teratomas are benign in the newborn period. Leukemia in the newborn appears to be more aggressive yet neuroblastoma has a better prognosis. More studies are needed to help us define why the neonate does better with some tumors and worse with others. Surface cell markers on neonatal leukemia, B and T cell function studies, and other immunologic surveillance studies are needed. Study of neonatal oncology may add to our knowledge of carcinogenesis and oncogenesis in the future.
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PMID:Neonatal oncology. 19 75

A search of the records of 10 pediatric oncology centers revealed 102 children with more than one malignant neoplasm. In this group of 102 patients, all pediatric cancers were seen as initial lesions, but Wilms' tumor and retinoblastoma were over-represented and leukemia and brain tumors underrepresented. Survival variation as well as tumor susceptibility may be responsible for this disproportion. Osteosarcomas and chondrosarcomas were the most frequent second malignant neoplasms (SMN). Embryonal tumors were rare as SMN and adult-type tumors (carcinomas) appeared at earlier than expected ages, whether arising after irradiation or not related to that form of therapy. Radiation was associated with 69 SMN, genetic disease accounted for 27 SMN and both conditions were noted in 15 SMN. In the group of 21 patients for whom neither radiation nor a known genetic disorder could be implicated, there were three with colon carcinoma and glioma and five with leukemia or lymphoma and glioma. These combinations may reflect new tissue-specific hereditary cancer syndromes.
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PMID:Patterns of second malignant neoplasms in children. 19 10

3':5'-Cyclic-AMP phosphodiesterase (EC 3.1.4.17) and the activating factor of cyclic nucleotide phosphodiesterase were detected in cultured human cell lines from patients with lymphoblastic leukemia and retinoblastoma and in the Brown-Pearce (rabbit) carcinoma. The homogenate of lymphoblasts contained levels of the activating factor in excess of that required to produce maximal activation of the endogenous phosphodiesterase. The activating factor found in these malignant cells appears to be similar to the calcium-binding protein activator of bovine brain phosphodiesterase on the basis of the molecular weight obtained from gel filtration, electrophoretic patterns, calcium requirement for the activity, and the effect of calcium on the proteolysis. In addition, the tumor-derived activator was able to restore the activity of activator-deficient phosphodiesterase from the bovine brain.
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PMID:Cyclic nucleotide phosphodiesterase and protein activator in human cancer cell lines and Brown-Pearce carcinoma. 20 Jul 56

The embryonal tumors of children occur in dominantly heritable and nonhereditary forms, which indicates that a dominant mutation can be on the carcinogenic pathway. A model which fits age-specific incidence hypothesizes that both forms arise as a consequence of two mutations. The background incidences of these tumors then reflect spontaneous mutation rates in germinal and somatic cells and may be increased by mutagens. The gene for one tumor (retinoblastoma) seems to be located on chromosome 13. Clues to the pathophysiology of these tumor genes come from consideration of their tissue specificity, origin from embryonal cells, and developmental effects. Childhood cancers may be manifestations of the homozygous states of a series of genes concerned with differentiation in specific embryonal tissues.
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PMID:Mutagenesis and embryonal carcinogenesis. 22 68

An 11-year-old Caucasian girl who had been cured of bilateral retinoblastoma developed non-radiation-induced osteosarcoma in multiple sites of the extremities. Investigation of the medical histories of 36 of her family members through six generations revealed that 8 relatives on the maternal side (22%) had malignant tumors, predominately genitourinary carcinomas, 2(6%) had benign tumors only, and 2(6%) had both benign and malignant neoplasms. The histologic variety of these tumors, the predominance of genitourinary carcinoma, the higher than expected frequency of tumor appearance over six generations, and the occurrence of malignant tumors in direct lineage suggest that the case of retinoblastoma followed by osteosarcoma is part of a familial cancer syndrome.
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PMID:A new familial cancer syndrome? A spectrum of malignant and benign tumors including retinoblastoma, carcinoma of the bladder and other genitourinary tumors, thyroid adenoma, and a probable case of multifocal osteosarcoma. 26 92

Genetic effects of cancer in childhood were examined among offspring of patients enrolled in the tumor registries of the Sidney Farber Cancer Institute and the Kansas University Medical Center. For 146 patients, 84 women and 62 men, 293 pregnancies were reported after cessation of treatment of diverse neoplasms. The outcomes of 286 completed pregnancies were as follows: 242 live births (1 set of twins), 1 stillbirth, 25 spontaneous abortions, and 19 therapeutic abortions. Seven live-born infants died during the first 2 years of life, a frequency in accord with expectation. Two offspring have developed cancer. One girl and her father had bilateral hereditary retinoblastoma. A second girl developed acute myelocytic leukemia; her mother had received radiotherapy during childhood for a brain tumor. Compared with their cousins and with published figures for the general population, the study progeny had no excess of congenital anomalies or other diseases. Chromosome and immunoglobulin studies of a few offspring did not reveal damage from preconception exposure to cancer chemotherapy and radiotherapy. Findings indicated that large collaborative studies are needed to monitor the offspring of childhood cancer survivors for inherited traits associated with the parental tumors and for mutagenic effects of therapy, particularly intense multimodality treatments.
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PMID:Offspring of patients treated for cancer in childhood. 28 96

In 47 patients (46 children and 1 adult) with unilateral and bilateral retinoblastoma, the titer of the carcinoembryonic antigen (CEA) was determined. The CEA titer of children with active retinoblastomas was 1.44 +/- 0.26 ng/ml (x +/- SEM). Those patients whose retinoblastoma was inactivated by therapy did not show a significantly lower CEA titer. In the group of children with one eye removed because of a unilateral retinoblastoma, the CEA titer was significantly (P less than 0.1) lower. The globes of 25 children were examined histologically. In those cases with invasion of the optic nerve or choroid, the CEA titer was significantly higher (P less than 0.1) as compared with those where the tumor was limited to the retina alone.
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PMID:[Carcinoembryonic antigen in retinoblastomas (author's transl)]. 30 12

Vitreous seeds in retinoblastoma were studied clinically and pathologically. Vitreous seeds were found in 36.6% of retinoblastoma eyes. When tumors involved more than three quadrants of the retina and vitreous seeds were present, the prognosis was poor. When the tumor involved less than one-quarter of the retina, vitreous seeds were rare. Vitreous seeds were found most frequently in cases of undifferentiated tumor cells and endophytum type of proliferation. Although most vitreous seeds were necrotic tumor cells, some were almost intact tumor cells which were apt to be situated along blood vessels. The blood vessels in vitreous seeds had no pericytes and were derived from the tumor itself. Thus it is possible that tumor cells in the vitreous body can migrate to the anterior segment of the eye. Some tumor cells in vitreous seeds had much cytoplasm which contained mitochondria, ribosomes, fibrils, centrioles, and cilia with a presumed photoreceptor outer segment and intercellular junctions. These cytoplasmic features are very similar to those of neuroepithelial-type retinoblastoma cells. Undifferentiated cells were necrotic. Calcium deposition was found mainly in the necrotic cytoplasm of the tumor cells and occasionally on the chromatin granules of the nucleus. This may provide evidence that calcium can be bound to DNA to form radiopaque masses. No calcium-producing cells were found. Vitreous seeds contain a small number of almost intact tumor cells which are neuroepithelial in type, but most cells are necrotic. Although tumor cells may migrate to the anterior segment of the eye along or through blood vessels, the presence of vitreous seeds in itself is not always a bad prognostic sign. The prognosis is probably more closely related to the extent of the invasion of a tumor associated with vitreous seeds.
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PMID:Vitreous seeds in retinoblastoma, clinical significance and ultrastructure. 31 88


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