{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,20]],"date-time":"2026-05-20T17:05:07Z","timestamp":1779296707000,"version":"3.51.4"},"reference-count":20,"publisher":"SAGE Publications","issue":"4","license":[{"start":{"date-parts":[[2003,7,1]],"date-time":"2003-07-01T00:00:00Z","timestamp":1057017600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatr Dev Pathol"],"published-print":{"date-parts":[[2003,7]]},"abstract":"<jats:p> Beckwith-Wiedemann syndrome (BWS) patients with chromosome 11p15.5 uniparental isodisomy (UPD) have an increased risk for developing embryonal tumors. UPD in these patients involves maternal loss of heterozygosity (LOH) and paternal duplication, which leads to tissue overgrowth and tumor development. Although 11p15.5 UPD predisposes to tumorigenesis, the events leading to tumorigenesis in UPD patients remains unknown. We have examined two hepatoblastomas in the BWS patients with UPD to determine the sequence of genetic events. Constitutional 11p15.5 LOH was detected in the blood or nonneoplastic liver of the BWS patients with hepatoblastoma. Mutation of \u03b2-catenin gene (CTNNB1) was found in one hepatoblastoma. Although mutations in CTNNB1 were not found in the second hepatoblastoma, nuclear accumulation of \u03b2-catenin was detected. However, mutation of CTNNB1 or nuclear accumulation of \u03b2-catenin was not detected in the tissue with hepatomegaly which contains UPD cells. These data indicate that Wnt signal activation can be involved as a later event in BWS-associated hepatoblastoma involving 11p15.5 UPD. <\/jats:p>","DOI":"10.1007\/s10024-003-1009-1","type":"journal-article","created":{"date-parts":[[2003,12,2]],"date-time":"2003-12-02T21:39:20Z","timestamp":1070401160000},"page":"299-306","source":"Crossref","is-referenced-by-count":31,"title":["Beckwith-Wiedemann Syndrome-associated Hepatoblastoma: Wnt Signal Activation Occurs Later in Tumorigenesis in Patients with 11p15.5 Uniparental Disomy"],"prefix":"10.1177","volume":"6","author":[{"given":"Ryuji","family":"Fukuzawa","sequence":"first","affiliation":[{"name":"Department of Pathology, Keio University, School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jun-ichi","family":"Hata","sequence":"additional","affiliation":[{"name":"National Research Institute for Child Health and Development, 3-35-1 Taishodo, Setagaya-ku, Tokyo 154-8567, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yutaka","family":"Hayashi","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgical Oncology, Tohoku University School of Medicine, 2-1 Seiryo-cho Aoba-ku, Sendai-shi Miyagi 980-8575, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hitoshi","family":"Ikeda","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery, Koshigaya Hospital, Dokkyo University School of Medicine, 2-1-50 Minamikoshigaya, Koshigaya-shi, Saitama 343-0845, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anthony E.","family":"Reeve","sequence":"additional","affiliation":[{"name":"Cancer Genetics Laboratory, Department of Biochemistry, Otago University, P.O. 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