Diencephalic syndrome and its relation to opticochiasmatic glioma: review of twelve cases.
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Biomedical subjects
Publications and source records attributed to J Mealey.
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This series of 228 patients with infantile non-neoplastic hydrocephalus who received either a ventriculoatrial (VA) or a ventriculoperitoneal (VP) shunt before 2 years of age was followed for an average of 7 years, and the results with the two types of shunt were compared. Mortality and infection rates were similar for both groups of patients, but children with VP shunts required significantly fewer revisions and had a much greater likelihood of not having any revisions during the follow-up period. Late complications occurred more frequently with VA shunts and were more serious. It is concluded that VP shunts offer significant advantages over VA systems in this population.
The authors have presented 81 children with intraspinal tumors, all less than 16 years of age. The clinical features indicated that gait disturbance, pain, and sphincter disturbance are the most prominent complaints. Reflex changes, paralysis, and sensory impairment are the most frequent physical findings. Cerebrospinal fluid protein was recorded in 47 patients and was abnormal in 34. Spine radiography was abnormal in 58% of the patients. Seventy of the patients had myelography, and it was diagnostic in 67. Surgery was performed on 79 patients, with a 2.5% operative mortality (30 days postsurgery). Of the 49 patients who are alive, 91.5% are able to walk. Mean follow-up period was 8.5 years. The authors document the benign course of the intramedullary astrocytomas and suggest an approach to their treatment. They encourage an aggressive approach to metastatic intraspinal tumors. The latrogenic origin of some epidermoid tumors is noted. The importance of early diagnosis and treatment of the intraspinal tumors in children is emphasized.
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A controlled, prospective, randomized study evaluated the use of 1,3-bis(2-chloroethyl)-1-nitrosourea (BCNU) and/or radiotherapy in the treatment of patients who were operated on and had histological confirmation of anaplastic glioma. A total of 303 patients were randomized into this study, of whom 222 (73%) were within the Valid Study Group (VSG), having met the protocol criteria of neuropathology, corticosteroid control, and therapeutic approach. Patients were divided into four random groups, and received BCNU (80 mg/sq m/day on 3 successive days every 6 to 8 weeks), and/or radiotherapy (5000 to 6000 rads to the whole brain through bilateral opposing ports), or best conventional care but no chemotherapy or radiotherapy. Analysis was performed on all patients who received any amount of therapy (VSG) and on the Adequately Treated Group (ATG), who had received 5000 or more rads radiotherapy, two or more courses of chemotherapy, and had a minimum survival of 8 or more weeks (the interval that would have been required to have received either the radiotherapy or chemotherapy). Median survival of patients in the VSG was, best conventional care: 14 weeks (ATG: 17.0 weeks); BCNU: 18.5 weeks (ATG: 25.0 weeks); radiotherapy: 35 weeks (ATG: 37.5 weeks); and BCNU plus radiotherapy: 34.5 weeks (ATG: 40.5 weeks). All therapeutic modalities showed some statistical superiority compared to best conventional care. There was no significant difference between the four groups in relation to age distribution, sex, location of tumor, diagnosis, tumor characteristics, signs or symptoms, or the amount of corticosteroid used. An analysis of prognostic factors indicates that the initial performance status (Karnofsky rating), age, the use of only a surgical biopsy, parietal location, the presence of seizures, or the involvement of cranial nerves II, III, IV, and VI are all of significance. Toxicity included acceptable, reversible thrombocytopenia and leukopenia.
Intraspinal epidermoid tumors may be congenital or acquired in origin. Lumbar punctures performed with needles without stylets or with ill-fitting stylets are responsible for the implantation of epidermal fragments into the spinal canal and the resultant iatrogenic intraspinal epidermoid tumors. Pain in the back or lower extremities is a striking clinical feature in these cases. Myelography should be considered for every patient who has pain in the lower extremities or back and who has had previous lumbar punctures.
The authors review treatment and results in 45 cases of medulloblastoma arising in childhood. The surgical mortality rate observed was 11%. Of those completing postoperative cerebrospinal irradiation at this institution, 53% have survived for 3 years, 41% for 5 years, and 22% for 10 years. The extent of surgical resection of the cerebellar tumor had no significant bearing on the prognosis. Those cases remaining free of recurrent disease had received significantly higher doses of postoperative irradiation, approaching 5000 rads to the whole brain or posterior fossa and 4000 rads to the spinal axis. Repeat irradiation and chemotherapy (vincristine, the nitrosoureas, and methotrexate) provided good palliation in most cases and significantly extended the survival time. However, 28 of 29 patients who developed locally recurrent or metastatic disease have died. Vincristine was considered the chemotherapeutic drug of choice and in 14 cases its use was associated with remissions lasting 2 to 18 months. The combination of chemotherapy and repeat irradiation was followed by remissions of longer duration compared to retreatment by irradiation alone when the disease recurred within 2 years. The inherent value of ventricular shunting procedures and steroid therapy for recurrent intracranial disease could not be ascertained. The findings in this study suggest that the primary treatment of medulloblastoma should be extended to include chemotherapy and optimum radiation therapy, since once recurrent disease develops retreatment is essentially palliative and a fatal outcome is virtuallly certain.
A controlled, prospective, randomized study evaluated the use of mithramycin in the treatment of anaplastic glioma compared to a similar group of patients receiving best conventional care. From a total of 116 patients in the study, 96 were within the valid study group. All patients were operated on, had histological confirmation of anaplastic glioma, and received radiotherapy at the discretion of the principal investigator. Fifty-two patients received mithramycin at a dose of 25 mug/kg/day for 21 days, while 44 patients were in the control group. There was no significant difference in the median survival from time of randomization in those receiving mithramycin (21 weeks) as compared to those not receiving mithramycin (26 weeks). There was no significant difference between the two groups in relation to age distribution, sex, location, diagnosis, tumor characteristics, signs or symptoms, or radiotherapy received. Duration of symptoms correlates positively with survival and was also significantly longer in the control group than in the treated group. This, however, did not account for the failure of mithramycin to be found an effective agent. Although the study was not designed to evaluate the efficacy of radiotherapy, patients who were so treated had a significant improvement in survival. The toxic complications of mithramycin included gastrointestinal symptoms, dermatological involvement, anemia, and liver dysfunction, indicating the need for close supervision.
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The use of conditioned medium (CM) obtained from monolayer cultures of human glioma cells induced colony formation from single glioma cells in culture. In contrast, no colonies were observed in cultures incubated with nonconditioned standard Eagle's basal medium. The number of colonies formed closely depended on the concentration of CM. The glioma CM not only stimulated colony formation but also induced the formation of fibrillary cell extensions. Culture conditions influencing the production of colony-stimulating factors included cell density and duration of culture medium contact with glioma cells. The colony-stimulating activity (CSA) was stable after freezing and thawing, but decreased 30-40% when CM was exposed to temperatures over 66 degrees C for 30 minutes. In addition, the CSA was filtratable (0.45 mu), dialyzable, and passable through an Amicon PM10 filter, which indicated a molecular weight less than 10,000. The use of CM provided an improved method for quantitative assays of neural tumor cells, based on their colony formation in culture.
In vitro cultivation of medulloblastoma cells was successfully established from hamster brain tumors induced by simian adenovirus SA7. These tumor cells possess morphological features in cultures that are very similar to those of the original tumor. Both tubular and rosette growth patterns were evident. At the ultrastructural level only small numbers of cytoplasmic organelles could be detected in the tumor cells, typical of generally immature and/or undifferentiated cells. The proliferation of these tumor cells depended upon properly dense initial platings. In addition, it was found that single medulloblastoma cells could be stimulated to produce colonies if treated for 10 days with conditioned medium. Inoculation of these cells into synergenic animals resulted in 100% uptake. Survival of the hamsters was directly correlated with the number of cells injected. This new medulloblastoma cell line may prove to be a useful model for experimental brain tumor studies.
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