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YHFS is caused by pathogenic variants in <jats:italic>TELO2<\/jats:italic>, a gene involved in regulation of the cell cycle. To date, 29 individuals with YHFS have been reported and none of them has been reported to develop tumors. We describe two siblings with YHFS both presenting with bilateral acoustic nerve agenesis, microcephaly, and dysmorphic features. Notably, one sibling developed hepatoblastoma at the age of 7.5\u2009years. Clinical exome sequencing revealed in both siblings compound heterozygous variants in the <jats:italic>TELO2<\/jats:italic> gene. Although the development of hepatoblastoma might be coincidental, given the role of <jats:italic>TELO2<\/jats:italic> in cell cycle, we suspect YHFS might be associated with an increased cancer susceptibility. Further cases are needed to confirm whether YHFS is associated with an increased risk of cancer.<\/jats:p>","DOI":"10.1002\/ajmg.a.63966","type":"journal-article","created":{"date-parts":[[2024,12,20]],"date-time":"2024-12-20T11:23:31Z","timestamp":1734693811000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Expansion of the Phenotype of You\u2010Hoover\u2010Fong Syndrome and Possible Increased Risk of Cancer"],"prefix":"10.1002","volume":"197","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1569-1556","authenticated-orcid":false,"given":"Alessandro","family":"De Falco","sequence":"first","affiliation":[{"name":"Department of Translational Medical Sciences University of Naples Federico II  Naples Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fabiola","family":"De Gregorio","sequence":"additional","affiliation":[{"name":"Department of Pediatric Oncology, Santobono\u2010Pausilipon 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