An Operative Staging System and a Megavoltage Radiotherapeutic Technic for Cerebellar Medulloblastomas

Chang, Chu H.; Housepian, Edgar M.; Herbert, Charles

doi:10.1148/93.6.1351

Cited by 683 publications

(291 citation statements)

References 6 publications

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“…The level of staining of p53 in the present study, which employed microwaving for antigen retrieval in the immunohistochemistry was consistent with that of a previous study (Jaros et al, 1993), in which no antigen retrieval was employed. DNA sequencing results indicated that p53 gene mutations in this series of cPNETs were infrequent, which was consistent with the findings from other studies (Saylors et al, 1991;Adesina, 1994) and could explain the high sensitivity of medulloblastoma cells to radiotherapy (Chang et al, 1969). The low incidence of p53 gene mutations in these cPNETs suggested that the p53 protein in these tumours was likely to be wild-type and functional.…”

Section: Discussionsupporting

confidence: 91%

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The molecular pathology of p53 in primitive neuroectodermal tumours of the central nervous system

et al. 2002

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One hundred and one pre-treatment primary central primitive neuroectodermal tumours were analysed for the expression of p53 protein by immunohistochemistry using the monoclonal antibody DO-7. The staining intensity was classified into four groups: strong, medium, weak and negative and strong staining intensity was associated with the poorest survival. DNA sequencing of the p53 gene was performed in 28 cases representing all four staining groups. Mutations were found in only three of the strong staining tumours suggesting that DNA mutations were not common events and that in the majority of the tumours with over-expressed p53, the protein was likely to be wild-type. Results of immunohistochemistry showed a significantly positive relationship between the expression of p53 and Bax and Bcl-2 proteins, but not Waf-1. Multivariate analyses supported the prognostic value of p53 immunostaining in central primitive neuroectodermal tumours and also of age and gender of patients.

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Section: Discussionsupporting

confidence: 91%

“…These malignant tumours frequently occur between the ages of 3 and 12 and between 50 -70 (de Vita et al, 1982) and are sensitive to radiotherapy (Chang et al, 1969). With advances in radiotherapy, neurosurgery and better supportive care, overall survival rates have been greatly improved in the last two decades from 24 to 82%.…”

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confidence: 99%

The molecular pathology of p53 in primitive neuroectodermal tumours of the central nervous system

et al. 2002

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“…Both protocols included good and poor risk adult patients because in a previous pediatric trial poor-risk patients receiving the Packer regimen had a better outcome than did good-risk patients treated with radiation alone (Packer et al, 1991(Packer et al, , 1994. Patients were considered poor risk if there was evidence of brainstem involvement, tumor lling the fourth ventricle extending into the foramina of Luschka or Magendiae producing internal hydrocephalus, or metastatic disease (Chang et al, 1969). For the pediatric trial, median progression-free 5-year relapse was 85% (Packer et al, 1994).…”

Section: Discussionmentioning

confidence: 99%

“…They were treated at the University of Michigan and the University of California at San Diego on the Packer protocol (described below) and at Brown University on both the Packer and the POG protocols (described below) without randomization. Patients were staged according to Chang criteria (Chang et al, 1969) with spinal neuraxis MRI and lumbar puncture including cytology. According to Chang criteria, a rating >T2 and/or >M0 was considered poor risk.…”

Section: Methodsmentioning

confidence: 99%

Adult medulloblastoma: Multiagent chemotherapy

Greenberg¹

2001

Neuro-Oncology

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In this study, the records of 17 adult patients with medulloblastoma treated with craniospinal radiation and 1 of 2 multiagent chemotherapy protocols were reviewed for progression-free survival, overall survival, and toxicity, and the patients were compared with each other and with similarly treated children and adults. Records of patients treated at 3 institutions were reviewed. Seventeen medulloblastoma patients (11 female, 6 male) with a median age of 23 years (range, 18-47 years) were treated with surgery, craniospinal radiation (CSRT) plus local boost, and 1 of 2 adjuvant chemotherapy regimens. All tumors were infratentorial (10 in 4th ventricle and 7 in left or right hemisphere). Ten patients presented with hydrocephalus, and 7 of them were shunted. Eight patients had gross total resection, 7 had subtotal resection (>50% removed), and 2 had partial resection (<50% removed). Postoperatively, 3 patients had positive cytology and 3 had positive spinal MRI. Five patients were classi ed as good risk and 12 were classi ed as poor risk (Chang staging system). Ten patients were treated with the "Packer protocol," consisting of CSRT plus weekly vincristine followed by 8 cycles of cisplatin, lomustine, and vincristine. Seven patients were treated with the Pediatric Oncology Group (POG) protocol, consisting of alternating courses of cisplatin/etoposide and cyclophosphamide/vincristine, followed by CSRT. Eight of 17 patients relapsed, with all 8 relapsing at the primary site. Other relapse sites included the leptomeninges (5), bone (1), and brain (1). The estimated median relapse-free survival (Kaplan-Meier) for all patients was 48 months (95% con dence interval, >26 months to ¥). Median relapse-free survival for patients on the Packer protocol was 26 months, and for those on the POG regimen was 48 months (P = 0.410). Five of 10 on the Packer protocol were relapse-free, while 4 of 7 were relapse-free on the POG regimen. Two patients relapsed during chemotherapy and 6 relapsed after completing all therapy at 18, 18, 26, 30, 40, and 48 months. The estimated median survival of all patients was 56 months (95% con dence interval, 27 to ¥) with 11 patients alive; for the Packer protocol, median survival was 36 months, and for the POG protocol, it was 57 months (P = 0.058). The hazard ratio was 0 (95% con dence interval, 0 to ¥). Toxicity during the Packer protocol was moderately severe, with only 1 of 10 patients able to complete all therapy. Two patients had severe abdominal pain during CSRT + vincristine, and 5 had peripheral neuropathy during vincristine therapy. Hearing loss (>20 dB) occurred in 7, neutropenia (<500 µl) in 6, thrombocytopenia (<50,000 µl) in 6, nephrotoxicity (>25% ¯by creatinine clearance) in 2, and decreased pulmonary function (diffusing capacity for carbon monoxide ¯>40%) in 1. On the POG protocol, only 1 patient had persistent nausea and vomiting, 2 had peripheral neuropathy, and 3 had hearing de cit (>20 dB) or tinnitus. The POG and Packer protocols did not have a statistically signi cant differenc...

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“…Staging procedures such as evaluation of lumbar cerebrospinal fluid (CSF) was in accordance with the Chang classification [11]. Neuroradiologic response was evaluated according to criteria of the German National Reference Center for Neuroradiology in Würzburg, Germany [12].…”

Section: Diagnostic Measuresmentioning

confidence: 99%

Improved 6‐year overall survival in AT/RT – results of the registry study Rhabdoid 2007

et al. 2016

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Atypical teratoid rhabdoid tumors (AT/RT) are characterized by mutations and subsequent inactivation of SMARCB1 (INI1, hSNF5), a predilection for very young children and an unfavorable outcome. The European Registry for rhabdoid tumors (EU-RHAB) was established to generate a common European database and to establish a standardized treatment regimen as the basis for phase I/II trials. Thus, genetic analyses, neuropathologic and radiologic diagnoses, and a consensus treatment regimen were prospectively evaluated. From 2005 to 2009, 31 patients with AT/RT from four countries were recruited into the registry study Rhabdoid 2007 and treated with systemic and intraventricular chemotherapy. Eight patients received high-dose chemotherapy, 23 radiotherapy, and 17 maintenance therapy. Reference evaluations were performed in 64% (genetic analyses, FISH, MLPA, sequencing) up to 97% (neuropathology, INI1 stain). Germ-line mutations (GLM) were detected in 6/21 patients. Prolonged overall survival was associated with age above 3 years, radiotherapy and achievement of a complete remission. 6-year overall and event-free survival rates were 46% (±0.10) and 45% (±0.09), respectively. Serious adverse events and one treatmentrelated death due to insufficiency of a ventriculo peritoneal shunt (VP-shunt) and consecutive herniation were noted. Acquisition of standardized data including reference diagnosis and a standard treatment schedule improved data quality Cancer MedicineOpen Access 1766

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An Operative Staging System and a Megavoltage Radiotherapeutic Technic for Cerebellar Medulloblastomas

Cited by 683 publications

References 6 publications

The molecular pathology of p53 in primitive neuroectodermal tumours of the central nervous system

The molecular pathology of p53 in primitive neuroectodermal tumours of the central nervous system

Adult medulloblastoma: Multiagent chemotherapy

Improved 6‐year overall survival in AT/RT – results of the registry study Rhabdoid 2007

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