Medulloblastoma: is the 5-year survival rate improving?

David, Karoly M.; Casey, Adrian; Hayward, Richard; Harkness, William; Phipps, Kim; Wade, Angie

doi:10.3171/jns.1997.86.1.0013

Cited by 108 publications

(68 citation statements)

References 55 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…1,7,30,31 Among these patients, 2 had early relapse in the spinal axis despite more aggressive therapy. In agreement with other reports on adults 1,4,19,32,36,40 and children, 17,37,48 we found that the presence of metastatic disease at presentation was an important prognostic factor for overall and event-free survival times. In a larger multicenter series, 7 metastatic disease did not appear to be correlated with a worse prognosis, but data were available for only 61% of the patients, and half of the patients with Stage M0 medulloblastomas received chemotherapy either before or after, or both before and after, radiotherapy.…”

Section: Prognostic Factorssupporting

confidence: 82%

Survival and prognostic factors in a series of adults with medulloblastomas

Riffaud

Saïkali

Leray

et al. 2009

JNS

View full text Add to dashboard Cite

Object In this article, the authors report their experience in the management of adult patients with medulloblastoma at their institution to identify prognostic factors important for survival and disease control. Methods Between 1977 and 2005, 27 patients who were ≥ 16 years old and had medulloblastoma were treated consecutively. There were 16 women and 11 men with a median age of 21 years (range 16–54 years). Gross-total resection was performed in 21 patients, subtotal (≥ 90%) in 2, incomplete in 1, and biopsy in 3 patients. Six patients had the desmoplastic variant, and 21 patients presented with classic medulloblastoma. Staging according to the Chang classification showed 4 patients with tumors invading the brainstem (2 with Stage T3b and 2 with Stage T4), 3 patients with metastases (2 with Stage M2 and 1 with Stage M3), and 1 patient in whom the stage was unknown (Stage MX) who died 10 days postoperatively. Twenty patients were assigned to the standard-risk group and 7 to the high-risk group. All patients except the one whose status was classified as Stage MX underwent craniospinal radiotherapy at our institution. Seven patients received chemotherapy before radiotherapy. Results The 5- and 10-year overall survival rates for the present study were 81 and 62%, respectively. The median overall survival time was 17.7 years. The 5- and 10-year event-free survival rates were 72 and 57%, respectively. The median event-free survival time was 17.9 years. Univariate analysis showed that survival was significantly correlated with sex (women had a better prognosis than men) and M stage (patients without metastases had a better outcome). Patient age, duration of symptoms, Karnofsky Performance Scale score at presentation, hydrocephalus, tumor location, brainstem invasion, extent of resection, histological subtype, preradiotherapy chemotherapy, risk group, and period of presentation were not significant variables. Multivariate analysis identified sex and M stage as well as the period of presentation as independent prognostic factors for overall and event-free survival times. Eleven patients suffered tumor recurrence within a median time of 4.2 years. The posterior fossa was not the most common site of recurrence, and delayed recurrence was not rare. All patients in whom the tumor recurred have died despite aggressive treatments. The median survival time after diagnosis of recurrence was 2.5 years. Questionnaires on quality of life and cognition showed high scores in favor of limited negative effects in the perception of mental and physical health after treatment. The authors observed 1 supposed second malignancy (thyroid carcinoma) and no evidence of pituitary dysfunction. Conclusions Long-term survival is possible in adults treated for medulloblastoma. Although rare, metastasis seeding at presentation is a poor prognostic factor. The possibility of delayed recurrence necessitates close follow-up of all patients. Tumor recurrences should be treated with aggressive therapies as some patients may have sustained response. Adjuvant chemotherapy should be given to high-risk patients, but its role in reducing recurrences, particularly distant ones, remains unclear in the standard-risk group.

show abstract

Section: Prognostic Factorssupporting

confidence: 82%

Survival and prognostic factors in a series of adults with medulloblastomas

Riffaud

Saïkali

Leray

et al. 2009

JNS

View full text Add to dashboard Cite

show abstract

“…They are known to exhibit predominantly neuronal differentiation and a tendency to metastasize via cerebrospinal fluid pathways. Although significant advances have been made in the treatment of MBs, the 5-year survival rate of 50% still represents an unfavorable course (1). Because the initial step of intraspinal spread is characterized by both seeding of tumor cells on the arachnoid or pia mater as well as the lysis of collagenous barriers to enhance angiogenesis and local invasion, attention has been focused on the ECM degradation.…”

Section: Discussionmentioning

confidence: 99%

“…Despite recent improvements in the survival of patients with MB, secondary tumor growth is observed in up to 40% of patients, resulting in a poor prognosis. Dissemination to the leptomeninges of the brain or seeding of tumor cells in the neuroaxis are the most common forms of metastasis (1)(2)(3)(4)(5). The underlying molecular mechanisms of brain tumor invasiveness are complex and have been studied mainly in malignant glial tumors and are closely related to proteolytic degradation of the extracellular matrix (ECM; Ref.…”

Section: Introductionmentioning

confidence: 99%

Expression of Matrix Metalloproteinases and Their Inhibitors in Medulloblastomas and Their Prognostic Relevance

Özen

Krebs

Hemmerlein³

et al. 2004

Clinical Cancer Research

View full text Add to dashboard Cite

Purpose and Experimental Design:The cellular mechanisms leading to metastatic disease in medulloblastoma (MB), the most common malignant brain tumor in childhood, are mainly unknown. Recently, however, the involvement of matrix metalloproteinases (MMPs) has been suggested. We examined the expression and localization of four MMPs-MMP-2 and -9, membrane-type 1 and 2 MMP (MT1-and MT2-MMP)-and correlated the data with those for their main inhibitors, tissue inhibitors of metalloproteinases (TIMP-1, -2, and -3), in 83 classical and 18 desmoplastic MBs.Results: Independent of the histological subtype, MMP-2 expression was found in a small percentage of tumors, whereas MMP-9 and MT1-or MT2-MMP were expressed in >75% of tumor samples. The expression of TIMP-1, -2, and -3, on the other hand, was found to depend on the histological subtype: TIMP-3 was often found in classical MB, whereas TIMP-2 was often expressed in desmoplastic MB (P ‫؍‬ 0.007-0.001). In addition, both TIMP-3 and -2 correlated significantly with the expression of all studied metalloproteinases except MMP-2. TIMP-1, detected only in classical MB in a low percentage, was the only TIMP that correlated with the expression of MMP-2. Kaplan-Meier estimation revealed significantly reduced long-term survival of patients with strong MMP expression in tumor samples. In multivariate logistic regression analysis, however, the prognosis was significantly determined only by clinical parameters.Conclusions: TIMP-3 and -2 expression is highly correlated with histological subtypes of MBs and strongly associated with the expression of certain MMPs. The expression of TIMPs and MMPs, however, does not determine prognosis independently of clinical parameters.

show abstract

“…1 Although advances in multimodality therapy with aggressive surgery, radiation and chemotherapy have led to dramatic improvement in the prognosis over the past decades, the 5-year survival rate still ranges from 50 to 70%. [2][3][4] In particular, leptomeningeal dissemination that results from metastases through the CSF space is thought to be one of the most hideous features of medulloblastomas and characterizes their malignant behavior. The CSF metastases appear as foci of nodular or diffuse contrast enhancement in the leptomeninges or on the ventricular surface in one-third of the patients at presentation.…”

Section: Introductionmentioning

confidence: 99%

Human neural stem cells target and deliver therapeutic gene to experimental leptomeningeal medulloblastoma

et al. 2007

View full text Add to dashboard Cite

Medulloblastomas are highly malignant neuroectodermal cerebellar tumors of children. One of the reasons for the difficulty for the treatment of medulloblastomas is their inherent tendency to metastasize through the cerebrospinal fluid (CSF) pathway leading to leptomeningeal dissemination. Recently, genetically modified neural stem cells (NSCs) were shown to have the capability of selectively migrating into glioma mass and delivering therapeutic agents with significant therapeutic benefits. In the present study, we applied the NSC strategy to target medulloblastomas, particularly their leptomeningeal dissemination. We used NSCs that were retrovirally transduced with the cytosine deaminase gene (CD-NSCs). In vitro studies demonstrated that CD-NSCs had sufficient migratory activity toward medulloblastoma cells and exerted a remarkable bystander effect on these cells following the application of 5-fluorocytosine (5-FC). It is noteworthy that neutralization of the hepatocyte growth factor blocked their migration In animal studies using our leptomeningeal dissemination model, CD-NSCs implanted directly into CSF space were shown to distribute diffusely within the disseminated tumor cells and could provide remarkable antitumor effect after intraperitoneal administration of 5-FC. Furthermore, CD-NSC treatment followed by 5-FC administration prolonged survival periods significantly in experimental animals. Our data suggest that the CD-NSC strategy can also be applied to target leptomeningeal dissemination of medulloblastomas.

show abstract

Medulloblastoma: is the 5-year survival rate improving?

Cited by 108 publications

References 55 publications

Survival and prognostic factors in a series of adults with medulloblastomas

Survival and prognostic factors in a series of adults with medulloblastomas

Expression of Matrix Metalloproteinases and Their Inhibitors in Medulloblastomas and Their Prognostic Relevance

Human neural stem cells target and deliver therapeutic gene to experimental leptomeningeal medulloblastoma

Contact Info

Product

Resources

About