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Titolo:
TUMORIGENICITY OF CEREBELLAR PRIMITIVE NEURO-ECTODERMAL TUMORS IN ATHYMIC MICE CORRELATES WITH POOR-PROGNOSIS IN CHILDREN
Autore:
VASSAL G; TERRIERLACOMBE MJ; LELLOUCHTUBIANA A; VALERY CA; SAINTEROSE C; MORIZET J; ARDOUIN P; RIOU G; KALIFA C; GOUYETTE A;
Indirizzi:
INST GUSTAVE ROUSSY,DEPT PEDIAT,RUE CAMILLE DESMOULINS F-94805 VILLEJUIF FRANCE INST GUSTAVE ROUSSY,DEPT ANAT PATHOL F-94805 VILLEJUIF FRANCE INST GUSTAVE ROUSSY,LAB PHARMACOTOXICOL,CNRS,URA 147 F-94805 VILLEJUIF FRANCE INST GUSTAVE ROUSSY,LAB PHARMACOGENET,CNRS,URA 147 F-94805 VILLEJUIF FRANCE INST GUSTAVE ROUSSY,CLIN & MOLEC PHARMACOL LAB F-94805 VILLEJUIF FRANCE INST GUSTAVE ROUSSY,ANIM EXPERIMENTAT LAB F-94805 VILLEJUIF FRANCE HOP NECKER ENFANTS MALAD,NEUROPATHOL LAB PARIS FRANCE HOP NECKER ENFANTS MALAD,DEPT PEDIAT NEUROSURG PARIS FRANCE
Titolo Testata:
International journal of cancer
fascicolo: 2, volume: 69, anno: 1996,
pagine: 146 - 151
SICI:
0020-7136(1996)69:2<146:TOCPNT>2.0.ZU;2-W
Fonte:
ISI
Lingua:
ENG
Soggetto:
C-MYC ONCOGENE; CELL-LINES; NUDE-MICE; AMPLIFICATION; MEDULLOBLASTOMA; DIFFERENTIATION; XENOGRAFTS; EXPRESSION; BLASTOMA; BRAIN;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
26
Recensione:
Indirizzi per estratti:
Citazione:
G. Vassal et al., "TUMORIGENICITY OF CEREBELLAR PRIMITIVE NEURO-ECTODERMAL TUMORS IN ATHYMIC MICE CORRELATES WITH POOR-PROGNOSIS IN CHILDREN", International journal of cancer, 69(2), 1996, pp. 146-151

Abstract

The histogenesis of medulloblastoma, also described as a cerebellar primitive neuro-ectodermal tumor (PNET), remains controversial and unresolved. In addition, genetic markers which characterize cerebellar PNETs with poor prognosis in not been identified, Since xenografts can befor better understanding the genetic events involved in cerebellar PNETs, small fragments of tumor samples from 17 children with newly diagnosed cerebellar PNETs were transplanted s.c. into female athymic Swiss mice. Eleven were non-metastatic and 6 were metastatic PNETs. Eight tumors (47%) were tumorigenic. Histological analysis showed 6 typical medulloblastomas, 1 PNET with melanin pigment and 1 PNET with a rhabdoid phenotype, Wide heterogeneity was observed in tumor growth, with a doubling time ranging from 8 to 81 days after the first passage, Tumorigenicity was correlated with the metastatic phenotype of the tumor (p< 0.001). All the patients but one with a tumorigenic tumor relapsed and died. The survival of patients with a non-tumorigenic PNET (67%) was significantly higher than that of patients with a tumorigenic PNET (13%) (P < 0.02). None of the xenografts or tumors from patients exhibited N-myc-gene alteration. Only one xenograft showed c-myc amplification, with an abnormal 15-kilobase fragment. None of the 17 tumors frompatients showed amplification or c-myc-gene rearrangement. In conclusion, tumorigenicity of cerebellar PNETs strongly correlates both with the metastatic phenotype of the tumors and with the disease-free survival of the patients. In addition, genetic events other than c-myc-geneamplification might be involved in cerebellar PNETs with poor prognosis. (C) 1996 Wiley-Liss, Inc.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 02/12/20 alle ore 04:38:13