Malignant Rhabdoid & Small Cell Undifferentiated Liver Tumors: A Rational for Uniform Classification
Updated: Feb 22, 2022
LIVER SURGERY RESEARCH HEIDELBERG
FOR IMMEDIATE RELEASE
A Systematic Review and Meta-Analysis of Malignant Rhabdoid and Small Cell Undifferentiated Liver Tumors: A Rational for a Uniform Classification
Jan. 6, 2022
HEIDELBERG, GERMANY – On Jan. 6, 2022, Liver Surgery Research Heidelberg’s new research, “A Systematic Review and Meta-Analysis of Malignant Rhabdoid and Small Cell Undifferentiated Liver Tumors: A Rational for a Uniform Classification,” authored by Juri Fuchs, Anastasia Murtha-Lemekhova, Markus Kessler, Fabian Ruping, Patrick Günther, Alexander Fichtner, Dominik Sturm, and Katrin Hoffmann, was published by the journal Cancers.
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-Meier 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. To learn more about this study, visit https://doi.org/10.3390/cancers14020272.
About Liver Surgery Research HD
Liver Surgery Research Heidelberg (LSRHD) is a research group, located at Heidelberg University in Heidelberg - Germany, that specializes in research investigating liver regeneration after hepatectomy (liver resection) and post-hepatectomy liver failure, HCC development and treatment (especially in the context of MAFLD [metabolically associated fatty liver disease]), and rare liver lesions and diseases. LSRHD is the founder and leader of the RELIVE (RarE LIVEr) Initiative; a multidisciplinary project to establish evidence-based therapies for rare liver diseases. LSRHD is currently working on over 20 research projects and has over 100 peer reviewed published authorships. To learn more about LSRHD, visit www.liversurgeryresearch.com.
Liver Surgery Research Heidelberg
Fuchs, J., Murtha-Lemekhova, A., Kessler, M., Ruping, F., Günther, P., Fichtner, A., Sturm, D., & Hoffmann, K. (2022). A Systematic Review and Meta-Analysis of Malignant Rhabdoid and Small Cell Undifferentiated Liver Tumors: A Rational for a Uniform Classification. Cancers, 14(2), 272. https://doi.org/10.3390/cancers14020272