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

The clinical and therapeutic features of 20 patients with medulloblastoma treated at Children's Hospital and Medical Center, Seattle, from 1980 to 1987, were retrospectively analyzed with regard to prognosis. The overall actuarial 5-year survival rate was 63%, with 57% of patients free from recurrence at 5 years. Residual contrast enhancement on CT scans taken immediately postoperatively was associated with a significantly decreased 5-year recurrence-free survival rate; the 5-year recurrence-free survival rate was 100% for those patients without post-operative residual enhancement compared to 41% for those patients with residual enhancement. A high risk group of patients with residual contrast enhancement persisting one year following diagnosis was identified. No patient in this group survived without disease progression. Other factors, including sex, age at diagnosis, evidence of tumor dissemination, or extent of surgical resection as reported by the neurosurgeon, did not significantly influence prognosis.
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PMID:The prognostic significance of postoperative residual contrast enhancement on CT scan in pediatric patients with medulloblastoma. 146 Apr 89

Children whose brain tumor involves two or more compartments at presentation differ clinically and pathologically from children whose brain tumor is confined to one compartment. In this study of 3,291 children with a brain tumor, at least 10% had a tumor that occupied two or three compartments at first hospitalization. Infratentorial tumors occupying multiple compartments were 1.7 times more likely to involve the cervicomedullary junction than the mesodiencephalic junction. Younger children (1-3 years) were more likely to have had multiple compartment tumors than older children. Children whose tumor was limited to the infratentorial compartment had a longer survival than children whose tumor also occupied other compartments. Ependymoma, anaplastic ependymoma, and astrocytoma (nos) were over represented among infratentorial multiple compartment tumors. Pilocytic astrocytoma, primitive neuroectodermal tumor (medulloblastoma), and desmoplastic medulloblastoma were less likely to have occupied multiple compartments at the time of the first surgical exploration. The distributions of histologic features in tumors at the cervicomedullary junction differed from those in tumors limited to the posterior fossa or to the spinal canal. Seizures were more likely if the tumor was confined to the supratentorial compartment, whereas nausea or vomiting and headache were more likely if the tumor was confined to the infratentorial compartment. Children whose tumor was confined to the spinal canal were significantly more likely to have bladder symptoms and back and/or abdominal pain than those whose tumor also involved compartments above the foramen magnum. We conclude that brain tumors apparently confined to one compartment at presentation are biologically and structurally different from tumors evident in two or more compartments.
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PMID:Childhood brain tumors that occupy more than one compartment at presentation. Multiple compartment tumors. 146 64

The goal of this study was to determine if certain imaging features suggest the diagnosis of cerebellar medulloblastoma in adults and to determine how often the classic CT appearance seen in children is present in adults. The study included 28 adult patients with proved cerebellar medulloblastoma. The tumor was located in the cerebellar vermis in 14 patients and in a cerebellar hemisphere in 14 patients. Thirteen patients had unenhanced CT of the brain, all patients had contrast-enhanced CT, and eight patients had unenhanced MR imaging. The imaging features in adults were compared with those in children, as described in the literature. In our adult patients, all tumors were hyperdense compared with gray matter on unenhanced CT and showed a slight to moderate increase in density after injection of contrast medium. Thirteen lesions had well-defined margins, and 15 had poorly defined margins. Low-density areas consistent with cystic and necrotic degeneration were detected in 23 (82%) of the 28 tumors. By comparison, in children, medulloblastoma usually originates in the vermis. As in adults, the mass is hyperdense on unenhanced CT, but enhances markedly and homogeneously after injection of contrast medium. Usually no evidence of cyst formation or necrosis is seen, and the tumor margins are well defined. This classic CT appearance of medulloblastoma in children was identified in only three (11%) of the 28 adult patients. Medulloblastoma has a variable MR appearance in both children and adults. On T2-weighted images, lesions are hypo-, iso-, or hyperintense compared with normal gray matter. The CT findings of medulloblastoma in adults usually differ from those of medulloblastoma in children. The tumor has a variable and nonspecific appearance in adults and should always be considered in the differential diagnosis of a mass in the posterior fossa.
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PMID:CT and MR imaging findings in adults with cerebellar medulloblastoma: comparison with findings in children. 150 35

We present four cases of infantile cerebellar neoplasms composed of cells with large vesicular nuclei with prominent nucleoli. All four cases were strongly immunoreactive for synaptophysin, and one case showed immunoreactivity for neurofilaments. Filter hybridization for N-myc and c-myc oncogenes showed a 27-fold c-myc amplification in one case. The cytogenetic analysis in this case showed Double-Minutes and isochromosome 17q. An intracerebral xenograft in nude mice obtained from one such tumor showed a similar morphology to that of the original tumor as well as strong immunoreactivity for synaptophysin and neurofilaments. All the neoplasms were characterized by highly aggressive behavior leading to early cerebrospinal fluid dissemination despite radiotherapy and chemotherapy. We conclude that large-cell medulloblastoma represents a distinct and more aggressive variant of medulloblastoma that requires more aggressive therapy.
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PMID:Large-cell medulloblastomas. A distinct variant with highly aggressive behavior. 153 Jan 8

A case of a 13-month-old boy with craniosynostosis associated with medulloblastoma was presented. Sagittal suture was prematurely closed, and coronal and lambdoid sutures were widely separated. A large infiltrative tumor in the midcerebellar region was partially removed and treated by irradiation and chemotherapy. A review of the current literature failed to reveal a similar report.
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PMID:Craniosynostosis in association with medulloblastoma. 153 Mar 88

In order to evaluate the effect of intensive combined chemotherapy in pediatric brain tumors, we designed a phase II study of high-dose busulfan and thiotepa followed by bone marrow transplantation (BMT) in children with measurable recurrent brain tumors. As alkylating agents, busulfan and thiotepa were expected to exhibit a steep dose effect and no overlapping extramedullary toxicity. Moreover, both drugs have an excellent distribution into the central nervous system in humans. Since May 1988, 20 children (median age 6 years) have been treated. Busulfan (150 mg/m2/day x 4) given orally was followed by thiotepa (350 mg/m2/day x 3), given as a 1 h i.v. infusion. Cryopreserved bone marrow was reinfused 48 h after completion of chemotherapy. Tumor response was assessed by computed tomography and magnetic resonance imaging 4 to 6 weeks after BMT. Five partial responses were observed (three of six medulloblastomas, one of five ependymomas, one of two primitive neuroectodermal tumors); two patients with medulloblastoma and one with brain stem tumor achieved an objective response. Ten patients had stable disease and one progressive disease. One patient is not evaluable because of early toxic death. Toxicity was high in terms of aplasia and cutaneous, hepatic and neurological complications. The overall response rate of 26% is encouraging since all patients had disease refractory to all conventional therapies.
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PMID:High-dose busulfan and thiotepa with autologous bone marrow transplantation in childhood malignant brain tumors: a phase II study. 843 18

The preoperative MR studies of 25 patients with surgically proved medulloblastomas were retrospectively reviewed in order to characterize these neoplasms with regard to their MR signal intensity, size, location, and appearance after contrast enhancement. Gadopentetate dimeglumine--enhanced MR images were available in 11 patients. On short TR/short TE images, medulloblastomas generally had low to intermediate signal, and were predominantly slightly hyperintense relative to muscle and hypointense relative to white matter. On long TR/long TE images, medulloblastomas generally had intermediate to moderately high signal, predominantly hyperintense relative to muscle and white matter. Tumor signal relative to gray matter varied considerably on both short TR and long TR images. Signal heterogeneity on long TR/long TE images was observed in 91% of the lesions and resulted from intratumoral cystic zones, small blood vessels, and/or calcifications. In the patients who received gadopentetate dimeglumine, the fraction of tumor volume showing enhancement was found to be less than one third in two cases, between one third and two thirds in four cases, and greater than two thirds in five cases. The mean tumor size was 3.6 x 4.0 x 3.5 cm. The most frequent location of medulloblastoma was the mid and inferior vermis. We conclude that the unenhanced and enhanced MR characteristics of medulloblastomas are somewhat variable. Medulloblastomas should be included in the differential diagnosis when the MR findings described are present in the appropriate patient population.
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PMID:MR imaging features of medulloblastomas. 154 6

A patient who had been treated for cerebellar medulloblastoma presented progressive abdominal distension and tachypnea. Metastases of the tumor in the abdominal and thoracic cavity were confirmed by cytology. The authors treated the metastasis with a combination of intravenous administration of 20 mg/m2/day cisplatin and 60 mg/m2/day etoposide for two 5-day cycles. After chemotherapy, the patient recovered fully with time and follow-up radiological studies demonstrated no evidence of tumor recurrence. Combination chemotherapy with cisplatin and etoposide is to be considered effective for extraneural metastasis of medulloblastoma.
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PMID:[A case of extraneural metastasis of medulloblastoma successfully treated with cisplatin and etoposide]. 155 5

The expression of epidermal growth factor receptor (EGF-R) was examined in 27 primary human brain tumors (7 glioblastomas, 10 astrocytomas, 5 oligodendrogliomas, 1 schwannoma, 1 ganglioneuroma, 1 medulloblastoma, 1 ependymoma, 1 histiocytic lymphoma), in 6 brain metastases from lung carcinomas and in 20 meningiomas. Peritumoral tissues histologically normal excised surgically along with a large tumor were used as control. All plasma membranes from brain tissues tested showed specific EGF binding. The EGF receptor is expressed at low levels in the control human brain and at very high levels in 60% of the total intracranial tumors studied. When the various histological types of tumors were analyzed, the higher percentage of positive tumors was found with the meningiomas (85%) and the glioblastomas (71%), while the lower percentage of positivity was found with the oligodendrogliomas (40%) and the astrocytomas (30%). A good correlation between binding and total amount of EGF-R protein detected by Western Blot was also observed.
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PMID:Epidermal growth factor receptor in human brain tumors. 156 Jan 88

Between 1966 and 1986, 14 patients with histologically verified cerebellar medulloblastomas were seen at J. Bordet Institute after total (4 cases) or partial (10 cases) tumor removal. Twelve received postoperative irradiation after some courses of chemotherapy (Vincristine, Procarbazine, Methotrexate) for 5. In 10 cases the X ray treatment was given to the whole cerebrospinal axis with approximately 50 Gy delivered to the primary tumor site, 25-35 Gy to the remaining brain and spinal cord. As in other series of the literature the long term prognosis is not too good, with approximately 50% of the patients surviving more than 5 years. Failure at the primary site happened in 4 cases. As a consequence of the small number of patients treated, no conclusion can be drawn from risk factor analysis. Yet it appears that younger patients fared better than older ones. A detailed description of the side effects of the treatment is therefore given for the five children treated at prepubertal age: some growth impairment seems to be the rule, a consequence of GH deficiency--if not compensated--and of the direct effect of ionizing radiation on the vertebral bodies. While discussing the progress of neurosurgery and radiotherapy for the treatment of medulloblastoma, the authors emphasize the need of more efficient adjuvant chemotherapy regimens and the interest of multicenter trial for evaluating them.
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PMID:[Medulloblastoma: analysis of 14 cases treated at the J. Bordet Institute and literature review]. 156 1


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