{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,10]],"date-time":"2026-05-10T08:34:57Z","timestamp":1778402097216,"version":"3.51.4"},"reference-count":35,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2009,1,6]],"date-time":"2009-01-06T00:00:00Z","timestamp":1231200000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["pathsocjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The Journal of Pathology"],"published-print":{"date-parts":[[2009,5]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Medulloblastoma is the most frequent malignant paediatric brain tumour. The activation of the Wnt\/\u03b2\u2010catenin pathway occurs in 10\u201015% of medulloblastomas and has been recently described as a marker for favourable patient outcome. We report a series of 72 paediatric medulloblastomas evaluated for \u03b2\u2010catenin protein expression,\n                    <jats:italic>CTNNB1<\/jats:italic>\n                    mutations, and comparative genomic hybridization. Gene expression profiles were also available in a subset of 40 cases. Immunostaining of \u03b2\u2010catenin showed extensive nuclear staining (&gt;50% of the tumour cells) in six cases and focal nuclear staining (&lt;10% of cells) in three cases. The other cases either exhibited a signal strictly limited to the cytoplasm (58 cases) or were negative (five cases).\n                    <jats:italic>CTNNB1<\/jats:italic>\n                    mutations were detected in all \u03b2\u2010catenin extensively nucleopositive cases. The expression profiles of these cases documented strong activation of the Wnt\/\u03b2\u2010catenin pathway. Remarkably, five out of these six tumours showed a complete loss of chromosome 6. In contrast, cases with focal nuclear \u03b2\u2010catenin staining, as well as tumours with negative or cytoplasmic staining, never demonstrated\n                    <jats:italic>CTNNB1<\/jats:italic>\n                    mutation, Wnt\/\u03b2\u2010catenin pathway activation or chromosome 6 loss. Patients with extensive nuclear staining were significantly older at diagnosis and were in continuous complete remission after a mean follow\u2010up of 75.7 months (range 27.5\u2013121.2 months) from diagnosis. All three patients with focal nuclear staining of \u03b2\u2010catenin died within 36 months from diagnosis. Altogether, these data confirm and extend previous observations that\n                    <jats:italic>CTNNB1<\/jats:italic>\n                    \u2010mutated tumours represent a distinct molecular subgroup of medulloblastomas with favourable outcome, indicating that therapy de\u2010escalation should be considered. International consensus on the definition criteria of this distinct medulloblastoma subgroup should be achieved. Copyright \u00a9 2009 Pathological Society of Great Britain and Ireland. Published by John Wiley &amp; Sons, Ltd.\n                  <\/jats:p>","DOI":"10.1002\/path.2514","type":"journal-article","created":{"date-parts":[[2009,1,6]],"date-time":"2009-01-06T08:22:14Z","timestamp":1231230134000},"page":"86-94","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":164,"title":["Beta\u2010catenin status in paediatric medulloblastomas: correlation of immunohistochemical expression with mutational status, genetic profiles, and clinical 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