Is INI1-retained small cell undifferentiated histology in hepatoblastoma unfavorable?

Shengmei Zhou; Ellen Gomulia; Leo Mascarenhas; Larry Wang

doi:10.1016/j.humpath.2014.12.013

Is INI1-retained small cell undifferentiated histology in hepatoblastoma unfavorable?

Hum Pathol. 2015 Apr;46(4):620-4. doi: 10.1016/j.humpath.2014.12.013. Epub 2015 Jan 14.

Authors

Shengmei Zhou¹, Ellen Gomulia², Leo Mascarenhas³, Larry Wang²

Affiliations

¹ 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.
² Department of Pathology and Laboratory Medicine, Children's Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, CA 90027.
³ 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.

PMID: 25649007
DOI: 10.1016/j.humpath.2014.12.013

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.

Keywords: Hepatoblastoma; High risk histology; INI1; Prognosis; Small cell undifferentiated.

Publication types

Case Reports

MeSH terms

Cell Differentiation / physiology
Chemotherapy, Adjuvant / methods
Child
Child, Preschool
Chromosomal Proteins, Non-Histone / metabolism*
DNA-Binding Proteins / metabolism*
Female
Hepatoblastoma / diagnosis*
Hepatoblastoma / pathology*
Hepatoblastoma / therapy
Humans
Infant
Liver Neoplasms / diagnosis*
Liver Neoplasms / pathology*
Liver Neoplasms / therapy
Liver Transplantation / methods
Male
Neoadjuvant Therapy / methods
Prognosis
SMARCB1 Protein
Transcription Factors / metabolism*
Treatment Outcome

Substances

Chromosomal Proteins, Non-Histone
DNA-Binding Proteins
SMARCB1 Protein
SMARCB1 protein, human
Transcription Factors