{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T07:37:16Z","timestamp":1790753836125,"version":"4.1.0"},"reference-count":64,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2022,4,15]],"date-time":"2022-04-15T00:00:00Z","timestamp":1649980800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100010090","name":"Women and Children\u2019s Health Research Institute","doi-asserted-by":"publisher","award":["2096"],"award-info":[{"award-number":["2096"]}],"id":[{"id":"10.13039\/100010090","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Children"],"abstract":"<jats:p>Liver neoplasms are quite rare in childhood. They often involve 6.7 cases per 10 million children aged 18 years or younger. Hepatoblastoma (HB) is the most frequent tumor, but this neoplasm\u2019s rarity points essentially to the difficulty of performing biologic studies and large-scale therapeutic trials. On the pathological ground, HB is separated into an entirely epithelial neoplasm or a mixed neoplasm with epithelial and mesenchymal components. This last category has been further subdivided into harboring teratoid features or not. The \u2018teratoid\u2019 HB includes a mixture of components with heterologous origin. The heterologous components include neuroectoderm, endoderm, or melanin-holding cells with or without mesenchymal components. The most important criterium for the teratoid component is neuroepithelium, melanin, and, more recently, a yolk-sac-like component and neuroendocrine components. The mesenchymal components include muscle, osteoid, and cartilage, which are most often observed mainly in \u2018teratoid\u2019 neoplasms. The teratoid component or mesenchymal components are diagnosed with biopsies. They appear more prominent after chemotherapy due to the response and shrinkage of epithelial elements and non- or low-responsive components of mixed HB. This review focuses on the clinical, radiological, and pathological findings of HB with teratoid features.<\/jats:p>","DOI":"10.3390\/children9040565","type":"journal-article","created":{"date-parts":[[2022,4,16]],"date-time":"2022-04-16T07:42:41Z","timestamp":1650094961000},"page":"565","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["\u2018Teratoid\u2019 Hepatoblastoma: An Intriguing Variant of Mixed Epithelial-Mesenchymal Hepatoblastoma"],"prefix":"10.3390","volume":"9","author":[{"given":"Consolato M.","family":"Sergi","sequence":"first","affiliation":[{"name":"Anatomic Pathology Division, Children\u2019s Hospital of Eastern Ontario, Ottawa, ON K1H 8L1, Canada"},{"name":"Department of Lab. Medicine and Pathology, Stollery Children\u2019s Hospital, University of Alberta, Edmonton, AB T6G 2B7, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marta","family":"Rojas-Vasquez","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hematology-Oncology, Stollery Children\u2019s Hospital, University of Alberta, Edmonton, AB T6G 2B7, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michelle","family":"Noga","sequence":"additional","affiliation":[{"name":"Department of Pediatric Radiology, Stollery Children\u2019s Hospital, University of Alberta, Edmonton, AB T6G 2B7, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bryan","family":"Dicken","sequence":"additional","affiliation":[{"name":"Department of Surgery, Stollery Children\u2019s Hospital, University of Alberta, Edmonton, AB T6G 2B7, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,4,15]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"192","DOI":"10.1053\/j.semdp.2016.12.015","article-title":"Hepatoblastoma","volume":"34","author":"Sharma","year":"2017","journal-title":"Semin. Diagn. Pathol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1053\/j.semdp.2016.12.016","article-title":"Primary mesenchymal liver tumors of childhood","volume":"34","author":"Saeed","year":"2017","journal-title":"Semin. Diagn. Pathol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1020","DOI":"10.1016\/S1470-2045(18)30491-1","article-title":"Carcinogenicity of isobutyl nitrite, beta-picoline, and some acrylates","volume":"19","author":"Kromhout","year":"2018","journal-title":"Lancet Oncol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"490","DOI":"10.1016\/S1470-2045(15)70134-8","article-title":"Carcinogenicity of tetrachlorvinphos, parathion, malathion, diazinon, and glyphosate","volume":"16","author":"Guyton","year":"2015","journal-title":"Lancet Oncol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1637","DOI":"10.1016\/S1470-2045(16)30513-7","article-title":"Carcinogenicity of pentachlorophenol and some related compounds","volume":"17","author":"Guyton","year":"2016","journal-title":"Lancet Oncol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"1003","DOI":"10.1016\/S1470-2045(17)30505-3","article-title":"Some chemicals that cause tumours of the urinary tract in rodents","volume":"18","author":"Grosse","year":"2017","journal-title":"Lancet Oncol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"183","DOI":"10.1038\/nrd.2017.226","article-title":"Drug development in the era of precision medicine","volume":"17","author":"Dugger","year":"2018","journal-title":"Nat. Rev. Drug Discov."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"571","DOI":"10.1038\/nrd884","article-title":"Value of novelty?","volume":"1","author":"Ma","year":"2002","journal-title":"Nat. Rev. Drug Discov."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1038\/nrd2519","article-title":"Genome-wide association studies: Progress and potential for drug discovery and development","volume":"7","author":"Kingsmore","year":"2008","journal-title":"Nat. Rev. Drug Discov."},{"key":"ref_10","first-page":"255","article-title":"Do genome-wide association scans have potential for translation?","volume":"50","author":"Lopes","year":"2011","journal-title":"Clin. Chem. Lab. Med."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1016\/j.ajhg.2011.11.029","article-title":"Five years of GWAS discovery","volume":"90","author":"Visscher","year":"2012","journal-title":"Am. J. Hum. Genet."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"2168","DOI":"10.1002\/1097-0142(19860601)57:11<2168::AID-CNCR2820571115>3.0.CO;2-M","article-title":"Teratoid hepatoblastoma. The nosologic dilemma of solid embryonic neoplasms of childhood","volume":"57","author":"Manivel","year":"1986","journal-title":"Cancer"},{"key":"ref_13","first-page":"409","article-title":"A malignant true teratoma of liver in childhood","volume":"80","author":"Misugi","year":"1965","journal-title":"Arch. Pathol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1097\/MOP.0000000000000046","article-title":"Hepatoblastoma state of the art: Pathology, genetics, risk stratification, and chemotherapy","volume":"26","author":"Czauderna","year":"2014","journal-title":"Curr. Opin. Pediatr."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"472","DOI":"10.1038\/modpathol.2013.80","article-title":"Towards an international pediatric liver tumor consensus classification: Proceedings of the Los Angeles COG liver tumors symposium","volume":"27","author":"Alaggio","year":"2014","journal-title":"Mod. Pathol."},{"key":"ref_16","unstructured":"Gilbert-Barness, E. (2007). Liver including tumors, gallbladder, and biliary tree. Potter\u2019s Pathology of the Fetus, Infant and Child, Mosby-Elsevier. [2nd ed.]."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1010","DOI":"10.1126\/science.1249484","article-title":"Detection of a recurrent DNAJB1-PRKACA chimeric transcript in fibrolamellar hepatocellular carcinoma","volume":"343","author":"Honeyman","year":"2014","journal-title":"Science"},{"key":"ref_18","doi-asserted-by":"crossref","unstructured":"Sergi, C.M. (2015). Hepatocellular Carcinoma, Fibrolamellar Variant: Diagnostic Pathologic Criteria and Molecular Pathology Update. A Primer. Diagnostics, 6.","DOI":"10.3390\/diagnostics6010003"},{"key":"ref_19","unstructured":"Adam, M.P., Ardinger, H.H., Pagon, R.A., Wallace, S.E., Bean, L.J.H., Stephens, K., and Amemiya, A. (1993). Beckwith-Wiedemann Syndrome. GeneReviews((R)), NCBI, University of Washington."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"287","DOI":"10.1007\/s10024-005-1154-9","article-title":"Beckwith-Wiedemann syndrome: Historical, clinicopathological, and etiopathogenetic perspectives","volume":"8","author":"Cohen","year":"2005","journal-title":"Pediatr. Dev. Pathol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"203","DOI":"10.1002\/ajmg.1320270122","article-title":"Trisomy 18 and hepatic neoplasia","volume":"27","author":"Dasouki","year":"1987","journal-title":"Am. J. Med. Genet."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1002\/ajmg.1320330119","article-title":"Trisomy 18 and hepatoblastoma","volume":"33","author":"Mamlok","year":"1989","journal-title":"Am. J. Med. Genet."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"302","DOI":"10.1046\/j.1442-200x.2001.01380.x","article-title":"Hepatoblastoma associated with trisomy 18 syndrome: A case report and a review of the literature","volume":"43","author":"Maruyama","year":"2001","journal-title":"Pediatr. Int."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1016\/j.cancergencyto.2004.04.011","article-title":"Hepatoblastoma in a patient with a partial trisomy 9p syndrome: A case report","volume":"156","author":"Schnater","year":"2005","journal-title":"Cancer Genet. Cytogenet."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"472","DOI":"10.1002\/ajmg.a.36271","article-title":"Hepatoblastoma in a 15-month-old female with trisomy 13","volume":"164A","author":"Shah","year":"2014","journal-title":"Am. J. Med. Genet. A"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"42","DOI":"10.1016\/S0165-4608(99)00033-3","article-title":"Karyotypic analyses of hepatoblastoma. Report of two cases and review of the literature suggesting chromosomal loci responsible for the pathogenesis of this disease","volume":"114","author":"Nagata","year":"1999","journal-title":"Cancer Genet. Cytogenet."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1002\/(SICI)1096-911X(200003)34:3<165::AID-MPO1>3.0.CO;2-T","article-title":"Cytogenetics of hepatoblastoma: Further characterization of 1q rearrangements by fluorescence in situ hybridization: An international collaborative study","volume":"34","author":"Parada","year":"2000","journal-title":"Med. Pediatr. Oncol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1016\/j.pediatrneurol.2003.10.014","article-title":"Brain metastases in hepatoblastoma","volume":"30","author":"Begemann","year":"2004","journal-title":"Pediatr. Neurol."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"757","DOI":"10.1001\/archopht.1996.01100130749023","article-title":"Neonatal hepatoblastoma metastatic to the choroid and iris","volume":"114","author":"Endo","year":"1996","journal-title":"Arch. Ophthalmol."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1007\/s00383-002-0906-0","article-title":"Analysis of treatment outcome for children with recurrent or metastatic hepatoblastoma","volume":"19","author":"Matsunaga","year":"2003","journal-title":"Pediatr. Surg. Int."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"634","DOI":"10.1177\/104063879300500426","article-title":"Hepatoblastoma in an equine fetus","volume":"5","author":"Neu","year":"1993","journal-title":"J. Vet. Diagn. Investig."},{"key":"ref_32","first-page":"383","article-title":"Pleural effusion resulting from malignant hepatoblastoma in a horse","volume":"194","author":"Prater","year":"1989","journal-title":"J. Am. Vet. Med. Assoc."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1354\/vp.44-2-211","article-title":"Three equine cases of mixed hepatoblastoma with teratoid features","volume":"44","author":"Loynachan","year":"2007","journal-title":"Vet. Pathol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"431","DOI":"10.3349\/ymj.2001.42.4.431","article-title":"Teratoid hepatoblastoma: Multidirectional differentiation of stem cell of the liver","volume":"42","author":"Kim","year":"2001","journal-title":"Yonsei Med. J."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"1503","DOI":"10.1016\/j.ejca.2005.02.035","article-title":"The emerging family of hepatoblastoma tumours: From ontogenesis to oncogenesis","volume":"41","author":"Zimmermann","year":"2005","journal-title":"Eur. J. Cancer"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"492","DOI":"10.1002\/mpo.10174","article-title":"Pediatric liver tumors and hepatic ontogenesis: Common and distinctive pathways","volume":"39","author":"Zimmermann","year":"2002","journal-title":"Med. Pediatr. Oncol."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"487","DOI":"10.1002\/mpo.10173","article-title":"Hepatoblastoma with cholangioblastic features (\u2018cholangioblastic hepatoblastoma\u2019) and other liver tumors with bimodal differentiation in young patients","volume":"39","author":"Zimmermann","year":"2002","journal-title":"Med. Pediatr. Oncol."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1016\/S0378-3782(00)00065-7","article-title":"The remodeling of the primitive human biliary system","volume":"58","author":"Sergi","year":"2000","journal-title":"Early Hum. Dev."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"1589","DOI":"10.1016\/S0002-9440(10)65031-6","article-title":"Contribution of apoptosis and apoptosis-related proteins to the malformation of the primitive intrahepatic biliary system in Meckel syndrome","volume":"156","author":"Sergi","year":"2000","journal-title":"Am. J. Pathol."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"568","DOI":"10.1007\/s100240010104","article-title":"Study of the malformation of ductal plate of the liver in Meckel syndrome and review of other syndromes presenting with this anomaly","volume":"3","author":"Sergi","year":"2000","journal-title":"Pediatr. Dev. Pathol."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"1495","DOI":"10.1369\/jhc.4A6297.2004","article-title":"Stem-like cells in human hepatoblastoma","volume":"52","author":"Fiegel","year":"2004","journal-title":"J. Histochem. Cytochem."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"1936","DOI":"10.1002\/1097-0142(19840501)53:9<1936::AID-CNCR2820530922>3.0.CO;2-W","article-title":"Teratoid Wilms\u2019 tumor","volume":"53","author":"Variend","year":"1984","journal-title":"Cancer"},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"45932","DOI":"10.1038\/srep45932","article-title":"Glypican 3 as a Serum Marker for Hepatoblastoma","volume":"7","author":"Zhou","year":"2017","journal-title":"Sci. Rep."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"1029","DOI":"10.7314\/APJCP.2015.16.3.1029","article-title":"Expression of glypican-3 is highly associated with pediatric hepatoblastoma: A systemic analysis","volume":"16","author":"Xiong","year":"2015","journal-title":"Asian Pac. J. Cancer Prev."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"324","DOI":"10.1046\/j.1365-2559.1997.2750870.x","article-title":"Hepatic stem-like cells in hepatoblastoma: Expression of cytokeratin 7, albumin and oval cell associated antigens detected by OV-1 and OV-6","volume":"31","author":"Ruck","year":"1997","journal-title":"Histopathology"},{"key":"ref_46","first-page":"699","article-title":"Identification of bipotential progenitor cells in human liver regeneration","volume":"75","author":"Haque","year":"1996","journal-title":"Lab. Investig."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"476","DOI":"10.1002\/hep.510230312","article-title":"Identification of bipotential progenitor cells in human liver development","volume":"23","author":"Haruna","year":"1996","journal-title":"Hepatology"},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"1249","DOI":"10.1096\/fasebj.10.11.8836038","article-title":"Hepatic stem cells in liver regeneration","volume":"10","author":"Thorgeirsson","year":"1996","journal-title":"FASEB J."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"7920897","DOI":"10.1155\/2016\/7920897","article-title":"Advances in Liver Regeneration: Revisiting Hepatic Stem\/Progenitor Cells and Their Origin","volume":"2016","author":"Sadri","year":"2016","journal-title":"Stem Cells Int."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"306","DOI":"10.1046\/j.1365-2559.2003.01675.x","article-title":"An unusual adult hepatic teratoma","volume":"43","author":"Nirmala","year":"2003","journal-title":"Histopathology"},{"key":"ref_51","first-page":"658","article-title":"Primary mixed germ cell tumour of the liver--a case report","volume":"46","author":"Verma","year":"2003","journal-title":"Indian J. Pathol. Microbiol."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"308","DOI":"10.1097\/00004836-199312000-00009","article-title":"Hepatic teratoma in an adult. Case report with a review of the literature","volume":"17","author":"Winter","year":"1993","journal-title":"J. Clin. Gastroenterol."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1016\/j.suronc.2007.07.002","article-title":"Tumors of the liver in children","volume":"16","author":"Meyers","year":"2007","journal-title":"Surg. Oncol."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1186\/1746-1596-4-37","article-title":"Mixed hepatoblastoma and teratoma of the liver in a 3-year-old child: A unique combination and clinical challenge","volume":"4","author":"Moll","year":"2009","journal-title":"Diagn. Pathol."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"287","DOI":"10.1097\/PAS.0b013e3181ce5f1e","article-title":"Effects of neoadjuvant chemotherapy on hepatoblastoma: A morphologic and immunohistochemical study","volume":"34","author":"Wang","year":"2010","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_56","unstructured":"(1967). Hereditary tyrosinemia. General discussion: Screening aspects. Can. Med. Assoc. J., 97, 1096\u20131098."},{"key":"ref_57","first-page":"311","article-title":"The effect of chemotherapy on hepatoblastoma","volume":"108","author":"Forouhar","year":"1984","journal-title":"Arch. Pathol. Lab. Med."},{"key":"ref_58","first-page":"293","article-title":"Pediatric hepatoblastoma: Diagnosis and treatment","volume":"3","author":"Hiyama","year":"2014","journal-title":"Transl. Pediatr."},{"key":"ref_59","doi-asserted-by":"crossref","unstructured":"Sergi, C., Dhiman, A., and Gray, J.A. (2018). Fine Needle Aspiration Cytology for Neck Masses in Childhood. An Illustrative Approach. Diagnostics, 8.","DOI":"10.3390\/diagnostics8020028"},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"456","DOI":"10.1007\/s12098-017-2301-9","article-title":"Management of Hepatoblastoma: ICMR Consensus Document","volume":"84","author":"Agarwala","year":"2017","journal-title":"Indian J. Pediatr."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"201","DOI":"10.4103\/0971-9261.142001","article-title":"The treatment of hepatoblastoma: Its evolution and the current status as per the SIOPEL trials","volume":"19","author":"Aronson","year":"2014","journal-title":"J. Indian Assoc. Pediatr. Surg."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"240","DOI":"10.1053\/jpsu.2002.30264","article-title":"Strategy for hepatoblastoma management: Transplant versus nontransplant surgery","volume":"37","author":"Pimpalwar","year":"2002","journal-title":"J. Pediatr. Surg."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"1331","DOI":"10.1016\/j.jpedsurg.2011.11.073","article-title":"Changing treatment and outcome of children with hepatoblastoma: Analysis of a single center experience over the last 20 years","volume":"47","author":"Ismail","year":"2012","journal-title":"J. Pediatr. Surg."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"1461","DOI":"10.1002\/pbc.26018","article-title":"Low Frequency of Programmed Death Ligand 1 Expression in Pediatric Cancers","volume":"63","author":"Aoki","year":"2016","journal-title":"Pediatr. Blood Cancer"}],"container-title":["Children"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2227-9067\/9\/4\/565\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T22:54:45Z","timestamp":1760136885000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2227-9067\/9\/4\/565"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,4,15]]},"references-count":64,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2022,4]]}},"alternative-id":["children9040565"],"URL":"https:\/\/doi.org\/10.3390\/children9040565","relation":{},"ISSN":["2227-9067"],"issn-type":[{"value":"2227-9067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,4,15]]}}}