The change in patterns of relapse in medulloblastoma

Article Abstract:

Medulloblastoma is a primitive brain tumor, which occurs in the cerebellum of children. Like most brain tumors, it is treated with radiation and/or surgery. Most chemotherapeutic drugs do not enter the brain effectively and are therefore of little value in treating cancer in this location. However, medulloblastoma sometimes spreads (metastasizes) to the bone, and systemic chemotherapy may be valuable in preventing bone metastases, even if it does little to fight the primary tumor. A review was conducted of 89 cases of medulloblastoma. Fifty cases occurred between 1970 and 1983; these patients were treated with surgery followed by radiation. The remaining 39 patients were treated since 1983; these patients received chemotherapeutic treatment in addition to the surgery and radiation therapy. Relapses in the central nervous system (CNS) occurred in 19 of the 50 (38 percent) earlier patients, and in nine of the 39 (23 percent) later patients. Relapse occurring in the central nervous system was found to be strongly related to the dose of radiation. CNS relapses occurred in 50 percent of the patients who received less than 5,300 cGy radiation treatment, but in only 18 percent of the patients who received more than this amount. (A Gy, or Gray, is a dose of radiation equivalent to one joule of energy absorbed per kilogram of tissue. It is equal to 100 rads.) A total of six bone relapses were seen. Not a single bone relapse was observed in patients who had received systemic chemotherapy. The five-year survival was 55 percent for the patients treated during the earlier period. The five-year survival was 72 percent for the patients treated more recently, although the difference in survival between the two groups was not considered statistically significant. (Consumer Summary produced by Reliance Medical Information, Inc.)

Development and progression, Prognosis, Cancer invasiveness, Medulloblastoma, Brain cancer

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Definitive radiation therapy in the management of symptomatic patients with optic glioma: survival and long-term effects

Article Abstract:

Glioma of the optic nerve occurs primarily in children, with 90 percent of such malignancies developing within the first two decades of life. The location of the tumor greatly affects the prognosis, with gliomas within the optic chiasm yielding a poorer outlook than gliomas further anterior along the optic nerve. Since optic nerve gliomas in children are low grade, and sometimes remit spontaneously, some researchers recommend aggressive treatment only if there are signs of deterioration. A review of 24 children with optic glioma in the optic chiasm revealed that gamma irradiation resulted in a 100 percent survival rate after a mean follow-up of six years. The patients received a total of from 4,500 to 5,660 cGy (a Gy, or Gary, is 1 joule of absorbed energy per kilogram of tissue) over a period of 35 to 52 days. Of the 24 children, 21 were free of disease progression. Deterioration of vision was a determining factor in deciding to proceed with radiotherapy. In 14 cases, radiotherapy halted the deterioration, and in 7 patients, vision improved. The vision of two patients continued to deteriorate, and one was already blind from previous surgery. Some endocrine abnormalities were noted in 15 patients, particularly a reduction in growth hormone. Other investigators have reported a number of endocrine abnormalities in similar patients; it is not clear if the abnormalities are a result of the tumor or the treatment. The somewhat benign nature of optic glioma has prompted some authorities to recommend against radiotherapy, but these results suggest that in patients with deteriorating vision, the radiotherapy provides positive benefits. (Consumer Summary produced by Reliance Medical Information, Inc.)

Health aspects, Radiotherapy, Astrocytoma, Astrocytomas, Eye cancer

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Choroid plexus tumors in the breast cancer-sarcoma syndrome

Article Abstract:

Breast cancer-sarcoma syndrome, also called Li-Fraumeni or SBLA syndrome, is a familial disorder that predisposes affected individuals to the development of a variety of different cancers. It is inherited as an autosomal dominant trait. Patients inheriting this genetic trait often develop two or more different tumors. Recently, an 11-month-old girl was found to have cancer of the choroid plexus, the secretory tissue within the ventricles of the brain. A family history revealed numerous cases of cancer, and construction of a pedigree suggested that the family was affected by breast cancer-sarcoma syndrome. The diagnosis of this case prompted the reexamination of 24 previously reported families with the same genetic disorder; 2 additional cases of choroid plexus cancer were identified among 13 brain tumors. Choroid plexus cancer is quite uncommon, so the occurrence of three cases among the families examined suggests that this cancer is also among the tumors for which family members are at high risk. It is not yet known if choroid plexus tumors are also more common among other forms of familial cancer syndromes. (Consumer Summary produced by Reliance Medical Information, Inc.)

Case studies, Genetic aspects, Familial diseases, Brain tumors, Li-Fraumeni syndrome

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