A review of the factors influencing the prognosis of medulloblastoma

Caputy, Anthony J.; McCullough, David C.; Manz, Herbert J.; Patterson, Kathleen; Hammock, Mary K.

doi:10.3171/jns.1987.66.1.0080

Cited by 109 publications

(27 citation statements)

References 39 publications

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“…In medulloblastomas, differentiation has been reported to be associated with both a worse and a better prognosis [26,27]. Only one report of supratentorial PNETs has suggested that degree of differentiation may correlate with survival [18].…”

Section: Discussionmentioning

confidence: 99%

Suprateutorial primitive neuroectodermal tumors in children

et al. 1996

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A retrospective review of 36 children diagnosed with a supratentorial primitive neuroectodermal tumor (PNET) at the Hospital for Sick Children was performed for the period 1970-1995. All children but one received their initial treatment at our institution. There were 18 males and 18 females and the median age at diagnosis was 35 months. Twenty-two PNETs were lobar, 3 were deep in the hemisphere, and 10 were located in the pineal region. One child presented with intracranial leptomeningeal disseminated disease. The tumors were mostly undifferentiated although 22 had some evidence of differentiation along one or more neuroepithelial lines. Five children had a biopsy, 24 had subtotal resection, and 7 had gross total resection. Twenty-six children had adjuvant radiotherapy and 13 had chemotherapy. At last follow-up 30 patients were dead and 6 were alive. The median survival was 23 months and the 2, 3, and 5 year survivals were 50%, 34%, and 18% respectively. All of the survivors received craniospinal radiation and 4 received chemotherapy. There was a statistically significantly worse survival in young children. There was a trend to better survival in children treated since 1984, and in children undergoing gross total resection. Because of the extremely poor survival, we recommended that all children undergo gross total resection followed by chemotherapy. For children older than 3 years of age craniospinal radiation should also be given.

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Section: Discussionmentioning

confidence: 99%

Suprateutorial primitive neuroectodermal tumors in children

et al. 1996

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“…Although fatal if untreated, current treatment regimens achieve 50-60% 5-year survival rates for medulloblastomas (Tait et al, 1990). At the present time neither clinical nor histopathological features in medulloblastomas reliably identify which tumours will recur (Caputy et al, 1987), making it difficult to develop new therapeutic strategies. A recent Newcastle study has shown that mitotic index and overexpression of the c-erbB2 protooncogene have some prognostic value in medulloblastomas (Gilbertson et al, 1992), and this led to an examination of additional molecular biological features of PNET's and their use as prognostic markers.…”

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p53 protein overexpression identifies a group of central primitive neuroectodermal tumours with poor prognosis

Jaros

Lunec²,

Perry³

et al. 1993

Br J Cancer

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Summary Primitive neuroectodermal tumours (PNET's) or medulloblastomas are common primary brain tumours of childhood. Current treatment protocols achieve 50-60% cures. However, it has proved difficult to develop better treatment for the remaining patients because prognostic factors are not established. We have investigated the prognostic value of p53 protein expression in 87 PNET's using immunohistochemistry with DO-7 and CM-1 antibodies on biopsy paraffin sections. Eight patients (9%) had intensely reactive tumour cell nuclei, and a significantly reduced survival (P = 0.002); only one survives and this with a recurrent tumour 50 months following diagnosis. Sixty eight per cent of patients had faintly reactive tumour cell nuclei, a reduced survival up to 4 years but a long term survival not significantly different (P = 0.41) from 23% of patients with p53 negative PNET's; the 10 year survival rates were 37% and 40%, respectively. Males had a reduced survival (P = 0.04) with a 2-fold relative risk of death compared to females. Multivariate analysis showed that intense overexpression of p53 protein identifies a group of PNET patients with a 7-fold relative risk of death compared to all other cases, irrespective of sex. This marked difference suggests the involvement of p53 in the pathogenesis of PNET's which have a particularly poor response to treatment, and should help to develop new therapies for this group of patients.

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“…Its potential of differentiation has been largely reported in the literature, including neuronal, glial and mesenchymal elements [6][7][8] . Authors have attempted to correlate some histological characteristics with prognosis and this has lead, sometimes, to different conclusions 4 . We report a case that represents a rare example of cerebellar tumor in adult, exhibiting an extraordinary morphology characterized by an uncommon mixture of mature adipose tissue with medulloblastomatous areas.…”

Section: Discussionmentioning

confidence: 99%

“…It occurs usually in childhood in the first decade of life 1 , being also found in adults [2][3][4] . According to World Health Organization (WHO) it is considered a grade IV neoplasm 5 .…”

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confidence: 99%

Cerebellar liponeurocytoma: a newly recognized clinico-pathological entity

Montagna

Moreira

Vaz

et al. 2002

Arq. Neuro-Psiquiatr.

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-The term "cerebellar liponeurocytoma", recently adopted by the World Health Organization Working Group (WHO), replaced many other different terms used up to now to give name to this rare tumor. To our knowledge, less than 20 cases have been related up to now under different names like as "lipomatous medulloblastoma, lipidized medulloblastoma, neurolipocytoma, medullocytoma and lipomatous glioneurocytoma". The new nomenclature eliminates the word "medulloblastoma", reinforces its benign caracter, and includes it in the category of glioneuronal tumors. We describe an adictional case of this distinct clinico-pathological entity removed from the right cerebellar hemisphere of a 53-year-old woman. With the present case report, we hope to contribute to the knowledge on the diagnostic and prognostic implications derived from the finding of mature adipose-like tissue within a medulloblastomatous tumour.KEY WORDS: liponeurocytoma, medulloblastoma, adipose tissue, mixed tumour. Liponeurocitoma cerebelar: uma nova entidade clínico-patológicaRESUMO -O termo "liponeurocitoma cerebelar" recentemente adotado pela Organização Mundial de Saúde (Classificação de Tumores Cerebrais -versão 2000), surgiu em substituição a vários outros utilizados até então, para denominar esta rara neoplasia. De nosso conhecimento há na literatura menos de 20 casos relatados sob termos diferentes tais como "meduloblastoma lipomatoso, meduloblastoma lipidizado, neurolipocitoma, medulocitoma e glioneurocitoma lipomatoso". A nova nomenclatura elimina a palavra "meduloblastoma", enfatiza seu caráter benigno e o coloca na categoria dos tumores glioneuronais. Descrevemos mais um caso desta rara entidade clínico-patológica, ressecada do hemisfério cerebelar direito em uma mulher de 53 anos. Com este caso esperamos contribuir para o melhor conhecimento sobre o diagnóstico, prognóstico e possibilidades terapêuticas advindas da presença de tecido adiposo em tumor meduloblastomatoso de adultos. PALAVRAS-CHAVE: liponeurocitoma, meduloblastoma, tecido adiposo, tumor misto.

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A review of the factors influencing the prognosis of medulloblastoma

Cited by 109 publications

References 39 publications

Suprateutorial primitive neuroectodermal tumors in children

Suprateutorial primitive neuroectodermal tumors in children

p53 protein overexpression identifies a group of central primitive neuroectodermal tumours with poor prognosis

Cerebellar liponeurocytoma: a newly recognized clinico-pathological entity

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