{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,1]],"date-time":"2026-08-01T18:48:13Z","timestamp":1785610093602,"version":"3.56.0"},"reference-count":45,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2022,1,6]],"date-time":"2022-01-06T00:00:00Z","timestamp":1641427200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cancers"],"abstract":"<jats:p>Background: Rhabdoid liver tumors in children are rare and have a devastating prognosis. Reliable diagnosis and targeted treatment approaches are urgently needed. Immunohistochemical and genetic studies suggest that tumors formerly classified as small cell undifferentiated hepatoblastoma (SCUD) belong to the entity of malignant rhabdoid tumors of the liver (MRTL), in contrast to hepatoblastomas with focal small cell histology (F-SCHB). This may have relevant implications on therapeutic approaches. However, studies with larger cohorts investigating the clinical relevance of the histological and genetic similarities for patients are lacking. Purpose: To analyze possible similarities and differences in patient characteristics, tumor biology, response to treatment, and clinical course of patients with MRTL, SCUD and F-SCHB. Applied therapeutic regimens and prognostic factors are investigated. Methods: A systematic literature search of MEDLINE, Web of Science, and CENTRAL was performed for this PRISMA-compliant systematic review. All studies of patients with MRTL, SCUD and F-SCHB that provided individual patient data were included. Demographic, histological, and clinical characteristics of the three subgroups were compared. Overall survival (OS) was estimated with the Kaplan\u2013Meier method and prognostic factors investigated in a multivariable Cox regression model. Protocol registered: PROSPERO 2021 CRD42021258760. Results: Fifty-six studies with a total of 118 patients were included. The two subgroups MRTL and SCUD did not differ significantly in baseline patient characteristics. However, heterogenous diagnostic and therapeutic algorithms were applied. Large histological and clinical overlap between SCUD and MRTL could be shown. Two-year OS was 22% for MRTL and 13% for SCUD, while it was significantly better in F-SCHD (86%). Chemotherapeutic regimens for hepatoblastoma proved to be ineffective for both SCUD and MRTL, but successful in F-SCHB. Soft tissue sarcoma chemotherapy was associated with significantly better survival for MRTL and SCUD, but was rarely applied in SCUD. Patients who did not undergo surgical tumor resection had a significantly higher risk of death. Conclusions: While F-SCHB is a subtype of HB, SCUD should be classified and treated as a type of MRTL. Surgical tumor resection in combination with intensive, multi-agent chemotherapy is the only chance for cure of these tumors. Targeted therapies are highly needed to improve prognosis. Currently, aggressive regimens including soft tissue sarcoma chemotherapy, extensive resection, radiotherapy or even liver transplantation are the only option for affected children.<\/jats:p>","DOI":"10.3390\/cancers14020272","type":"journal-article","created":{"date-parts":[[2022,1,7]],"date-time":"2022-01-07T01:15:26Z","timestamp":1641518126000},"page":"272","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["A Systematic Review and Meta-Analysis of Malignant Rhabdoid and Small Cell Undifferentiated Liver Tumors: A Rational for a Uniform Classification"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2484-8615","authenticated-orcid":false,"given":"Juri","family":"Fuchs","sequence":"first","affiliation":[{"name":"Department of General, Visceral and Transplantation Surgery, University Hospital Heidelberg, 69120 Heidelberg, Germany"},{"name":"Generating Evidence for Diagnosis and Therapy of RarE LIVEr Disease: The RELIVE Initiative for Systematic Reviews and Meta-Analyses, University Hospital Heidelberg, 69120 Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1294-7459","authenticated-orcid":false,"given":"Anastasia","family":"Murtha-Lemekhova","sequence":"additional","affiliation":[{"name":"Department of General, Visceral and Transplantation Surgery, University Hospital Heidelberg, 69120 Heidelberg, Germany"},{"name":"Generating Evidence for Diagnosis and Therapy of RarE LIVEr Disease: The RELIVE Initiative for Systematic Reviews and Meta-Analyses, University Hospital Heidelberg, 69120 Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Markus","family":"Kessler","sequence":"additional","affiliation":[{"name":"Generating Evidence for Diagnosis and Therapy of RarE LIVEr Disease: The RELIVE Initiative for Systematic Reviews and Meta-Analyses, University Hospital Heidelberg, 69120 Heidelberg, Germany"},{"name":"Department of General, Visceral and Transplantation Surgery, Division of Pediatric Surgery, University Hospital Heidelberg, 69120 Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabian","family":"Ruping","sequence":"additional","affiliation":[{"name":"Department of General, Visceral and Transplantation Surgery, Division of Pediatric Surgery, University Hospital Heidelberg, 69120 Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrick","family":"G\u00fcnther","sequence":"additional","affiliation":[{"name":"Generating Evidence for Diagnosis and Therapy of RarE LIVEr Disease: The RELIVE Initiative for Systematic Reviews and Meta-Analyses, University Hospital Heidelberg, 69120 Heidelberg, Germany"},{"name":"Department of General, Visceral and Transplantation Surgery, Division of Pediatric Surgery, University Hospital Heidelberg, 69120 Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9939-4059","authenticated-orcid":false,"given":"Alexander","family":"Fichtner","sequence":"additional","affiliation":[{"name":"Generating Evidence for Diagnosis and Therapy of RarE LIVEr Disease: The RELIVE Initiative for Systematic Reviews and Meta-Analyses, University Hospital Heidelberg, 69120 Heidelberg, Germany"},{"name":"Department of Pediatrics I, Division of Pediatric Gastroenterology, University Children\u2019s Hospital Heidelberg, 69120 Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dominik","family":"Sturm","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hematology and Oncology, Heidelberg University Hospital, 69120 Heidelberg, Germany"},{"name":"Hopp Children\u2019s Cancer Center (KiTZ), 69120 Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katrin","family":"Hoffmann","sequence":"additional","affiliation":[{"name":"Department of General, Visceral and Transplantation Surgery, University Hospital Heidelberg, 69120 Heidelberg, Germany"},{"name":"Generating Evidence for Diagnosis and Therapy of RarE LIVEr Disease: The RELIVE Initiative for Systematic Reviews and Meta-Analyses, University Hospital Heidelberg, 69120 Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,1,6]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"925","DOI":"10.1002\/gcc.22390","article-title":"Small cell undifferentiated (SCUD) hepatoblastomas: All malignant rhabdoid tumors?","volume":"55","author":"Vokuhl","year":"2016","journal-title":"Genes Chromosomes Cancer"},{"key":"ref_2","doi-asserted-by":"crossref","unstructured":"Fazlollahi, L., Hsiao, S.J., Kochhar, M., Mansukhani, M.M., Yamashiro, D.J., and Remotti, H.E. 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