title: Is INI1-retained small cell undifferentiated histology in hepatoblastoma unfavorable?
abstract: Small cell undifferentiated (SCU) is a rare epithelial component of hepatoblastoma (HB), generally regarded as a high-risk histology for an adverse outcome. Limited data show that SCU cells in some HBs with poor prognosis had INI1 loss. Of note, SCU cells are usually INI1-retained in more typical HBs. We evaluated the clinical and pathologic characteristics of 7 HBs with SCU. Five patients were Children's Oncology Group stage I and underwent complete resection and adjuvant chemotherapy. Two patients with Children's Oncology Group stage III had neoadjuvant chemotherapy and liver transplantation. Six patients were alive without evidence of disease with a median follow-up of 5.4 years (range, 4-14.1 years). One patient died of transplantation-related complications 2.6 years after initial diagnosis. Small cell undifferentiated cells in 7 patients with HB were all INI-retained. Our results suggest that INI1-retained SCU may be not prognostically unfavorable. Immunohistochemical staining of INI1 should be performed in HBs with SCU.
authors: Department of Pathology and Laboratory Medicine, Children's Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, CA 90027. Electronic address: Szhou@chla.usc.edu.
authors: Shengmei
authors: S
authors: Zhou
authors: Department of Pathology and Laboratory Medicine, Children's Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, CA 90027.
authors: Ellen
authors: E
authors: Gomulia
authors: Division of Hematology, Oncology, and Blood and Marrow Transplantation, Department of Pediatrics, Children's Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, CA 90027.
authors: Leo
authors: L
authors: Mascarenhas
authors: Department of Pathology and Laboratory Medicine, Children's Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, CA 90027.
authors: Larry
authors: L
authors: Wang
mesh_terms: D002454:Cell Differentiation / Q000502:physiology; D017024:Chemotherapy, Adjuvant / Q000379:methods; D002648:Child; D002675:Child, Preschool; D002868:Chromosomal Proteins, Non-Histone / Q000378:metabolism*; D004268:DNA-Binding Proteins / Q000378:metabolism*; D005260:Female; D018197:Hepatoblastoma / Q000175:diagnosis* / Q000473:pathology* / Q000628:therapy; D006801:Humans; D007223:Infant; D008113:Liver Neoplasms / Q000175:diagnosis* / Q000473:pathology* / Q000628:therapy; D016031:Liver Transplantation / Q000379:methods; D008297:Male; D020360:Neoadjuvant Therapy / Q000379:methods; D011379:Prognosis; D000071796:SMARCB1 Protein; D014157:Transcription Factors / Q000378:metabolism*; D016896:Treatment Outcome
chemical_list: D002868:Chromosomal Proteins, Non-Histone; D004268:DNA-Binding Proteins; D000071796:SMARCB1 Protein; C513266:SMARCB1 protein, human; D014157:Transcription Factors
keywords: Hepatoblastoma; High risk histology; INI1; Prognosis; Small cell undifferentiated