Prognostic factors for medulloblastoma

Jenkin, Derek; Shabanah, Mohamed Al; Shail, Essam Al; Gray, Alan; Hassounah, Maher; Khafaga, Yasser; Kofide, A.; Mustafa, Mahmoud M.; Schultz, Henrik B.

doi:10.1016/s0360-3016(00)00431-4

Cited by 59 publications

(44 citation statements)

References 32 publications

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“…Also, in a recent study, localized, non-metastatic disease (M0 Chang stage) was a positive predictor (n = 30) [1] while in other studies [5,6] including the present one, M0 stage was not a prognostic factor. The problems of defining prognostic factors in adult medulloblastoma is paralleled by inconclusive findings in pediatric series where higher age and low M-stage were frequently [15,17,29], but not consistently [19,20] prognostic factors. The value of factors such as lateral localization or desmoplastic histology is also unclear [12].…”

Section: Discussionmentioning

confidence: 94%

Adult medulloblastoma

et al. 2005

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Adult medulloblastoma is a rare tumor with few retrospective studies published so far. The role of adjuvant chemotherapy or chemotherapy at relapse is unclear. This study reports therapy and outcome in all adult (>or=16 years old) medulloblastoma (n=34) and supratentorial primitive neuroectodermal tumor (PNET) patients (n=2) treated in 2 neuro-oncological centers between 1976 and 2002. The median age was 24.5 years (range 16-76). After resection, 16 patients were treated with craniospinal radiotherapy alone, 20 patients also received adjuvant chemotherapy (8 vincristine, CCNU, cisplatin; 7 methotrexate alone or methotrexate/vincristine-based polychemotherapy; 5 other protocols). Median survival in the whole cohort was 126 months (2+ - 200+months). Five-year and 10-year survival rates were 79 % and 56%. Adjuvant chemotherapy was associated with a non-significant trend to prolonged survival (relative risk (RR) 1.89; p=0.068). The median progression-free survival (PFS) after primary therapy was 83 months. At relapse, 10 of 12 evaluable patients achieved a complete response upon second-line therapy. The median survival times from first (n=17) and second relapse (n=9) were 21 months (0-67+ months; 5/17 without second relapse) and 20 months (1-29 months). Cox regression analysis revealed the infiltration of the floor of the 4(th) ventricle at diagnosis as the only therapy-independent prognostic factor (RR 0.48; p=0.03). In conclusion, adjuvant chemotherapy may prolong survival in adult medulloblastoma patients. Moreover, second-line therapy may be beneficial for these patients. As in pediatric medulloblastoma patients, primary infiltration of the floor of the 4(th) ventricle indicates a poor prognosis.

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Section: Discussionmentioning

confidence: 94%

Adult medulloblastoma

et al. 2005

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“…Modern advances in treatment regimens have improved 5-year survival rates to ϳ70% for standard-risk patients (1-4), but survival rates for high-risk patients are as low as 25% (5,6). This clinical dichotomy, in which metastatic disease at presentation, age Ͻ3 years, or residual tumor Ͼ1.5 cm 2 on magnetic resonance imaging categorize patients as high-risk, is the only stratification of medulloblastoma patients currently undertaken for therapeutic purposes.…”

Section: Introductionmentioning

confidence: 99%

Combined Histopathological and Molecular Cytogenetic Stratification of Medulloblastoma Patients

Lamont¹,

McManamy²,

Pearson³

et al. 2004

Clinical Cancer Research

193

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This study examined the utility of stratifying children with medulloblastomas by a combination of refined histopathological classification and molecular cytogenetic evaluation.Detailed histopathological classification of tumors from a cohort of patients (n ‫؍‬ 87) composed mainly of children entered into the International Society of Pediatric Oncology (SIOP)/United Kingdom Children's Cancer Study Group PNET3 trial (n ‫؍‬ 65), included identification of the large cell/anaplastic phenotype. Fluorescence in situ hybridization was used to detect chromosome 17 abnormalities, losses of 9q22 and 10q24, and amplification of the MYCC and MYCN oncogenes.The large cell/anaplastic phenotype, which was present in 20% of medulloblastomas, emerged as an independent prognostic indicator. Loss of 17p13.3 (38% of medulloblastomas) was found across all of the histopathological variants, whereas MYCC/MYCN amplification (6%/8% of medulloblastomas) was significantly associated with the large cell/ anaplastic phenotype. Both of these genetic abnormalities emerged as prognostic indicators. Loss of 9q22 was associated with the nodular/desmoplastic medulloblastoma variant, whereas loss of 10q24 was found in all of the variants. Together with metastatic tumor at presentation, the large cell/anaplastic phenotype, 17p13.3 loss, or high-frequency MYC amplification defined a high-risk group of children whose outcome was significantly (P ‫؍‬ 0.0002) poorer than a low-risk group without these tumor characteristics.Combined evaluation of novel histopathological features and molecular cytogenetic abnormalities promises to allow stratification of patients with medulloblastoma, such that those likely to be cured will be spared the side effects of maximal therapy, which can be targeted at those with aggressive disease.

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“…Currently, patients are divided into average and high-risk groups, with the high-risk group being characterized by three clinical parameters: age group (<3 years), residual tumor after resection >1.5 cm (3), and evidence of metastasis at presentation (3,4). Recent reports, however, indicate that these clinical variables are an inadequate method of defining disease risk (5). To further develop parameters for clinical outcome prediction, molecular genetic markers such as expression of trkC (6), c-myc (7), erbB (8), PDGFRB (9, 10) chromosomal abnormality iso-17q (11), a gain of 8q (12), or anaplastic histology (13) have also been suggested to be potential aids in predicting prognosis.…”

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confidence: 99%