{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,2]],"date-time":"2025-08-02T05:28:01Z","timestamp":1754112481089},"reference-count":21,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2013,12,5]],"date-time":"2013-12-05T00:00:00Z","timestamp":1386201600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J of Med Genetics Pt A"],"published-print":{"date-parts":[[2014,2]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:label \/><jats:p>Trisomy 13 (T13) is a rare autosomal aneuploidy. Greater than 90% of patients die during the first year of life. Malignancies reported in association with T13 include two cases of Wilms tumor and one case of pilocytic astrocytoma. There is no previous report of hepatoblastoma in patients with T13. We report a unique case of hepatoblastoma in a 15\u2010month\u2010old female with constitutional T13. Our patient was born at 38 weeks gestation and was noted to have phenotypic features consistent with T13. Genetic testing confirmed an abnormal karyotype of 47,XX,+13 in all cells. At fifteen months of age she was noted to have a right hepatic lobe mass on a routine follow\u2010up renal ultrasound for hydronephrosis. Serum alpha\u2010fetoprotein level was 55,300\u2009ng\/ml. Staging work\u2010up revealed the absence of metastases. She underwent a complete surgical resection with right hepatic lobectomy. Histopathology was consistent with hepatoblastoma, mixed epithelial and mesenchymal type. She had a protracted postoperative course complicated by <jats:italic>Enterobacter aerogenes<\/jats:italic> urosepsis, a significant biloma, chronic pancreatitis, and apneic episodes of uncertain etiology. She received four courses of doxorubicin monotherapy without any severe or unexpected toxicity. She continues to be in remission 8 months following diagnosis. This is the first reported case of hepatoblastoma in a child with constitutional T13. This may represent a non\u2010random association, as somatic trisomy of chromosome 13 in hepatoblastoma tumors has been previously described in the literature. Prolonged survival may have allowed for hepatoblastoma to present in our patient. \u00a9 2013 Wiley Periodicals, Inc.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ajmg.a.36271","type":"journal-article","created":{"date-parts":[[2013,12,5]],"date-time":"2013-12-05T20:36:51Z","timestamp":1386275811000},"page":"472-475","source":"Crossref","is-referenced-by-count":8,"title":["Hepatoblastoma in a 15\u2010month\u2010old female with trisomy 13"],"prefix":"10.1002","volume":"164","author":[{"given":"Rachana","family":"Shah","sequence":"first","affiliation":[{"name":"Division of Hematology and Oncology Children's Hospital Los Angeles Los Angeles California"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hung Chi","family":"Tran","sequence":"additional","affiliation":[{"name":"Division of Hematology and Oncology Children's Hospital Los Angeles Los Angeles California"},{"name":"Department of Pediatrics Keck School of Medicine University of Southern California Los Angeles 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