Data di Pubblicazione:
2008
Citazione:
Evaluation of 1p36 markers and clinical outcome in a skull base chordoma study / M. Longoni, F. Orzan, M. Stroppi, N. Boari, P. Mortini, P. Riva. - In: NEURO-ONCOLOGY. - ISSN 1522-8517. - 10:1(2008), pp. 52-60. [10.1215/15228517-2007-048]
Abstract:
Chordomas are rare embryogenetic tumors, arising from remnants of the notochord, characterized by local invasiveness and variable tendency for recurrence. No molecular markers are currently used in a clinical setting to distinguish chordomas with an indolent or an aggressive
pattern. Among the genetic lesions observed in this tumor, one of the most commonly detected is 1p loss. In a previous study we observed 1p36 loss of heterozygosity(LOH) in 85% of the analyzed chordomas. We studied a group of 16 homogeneously treated skull base chordomas
(SBCs), reporting 1p36 LOH in 75% of them and determining
the expression pattern of eight apoptotic genes
mapped at 1p36. No tumors shared a common expression profile with nucleus pulposus, which is considered the only adult normal tissue deriving from notochord. In particular, tumor necrosis factor receptor superfamily
genes TNFRSF8, TNFRSF9, and TNFRSF14 were differently expressed compared with control in a higher percentage of tumors (40%–53%) than were the remaining
analyzed genes, suggesting that the deregulation of these
three genes might have a role in chordoma tumorigenesis.
The presence/absence of LOH and the expression/
nonexpression of each apoptotic gene were studied in
a survival analysis. Our results suggest that the lack
of 1p36 LOH or the presence of TNFRSF8 expression
might be associated with a better prognosis in patients
with SBCs.
Tipologia IRIS:
01 - Articolo su periodico
Keywords:
1p36 LOH; Apoptotic gene; Clinical outcome; Expression profile; Skull base chordoma
Elenco autori:
M. Longoni, F. Orzan, M. Stroppi, N. Boari, P. Mortini, P. Riva
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