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Is INI1-retained small cell undifferentiated histology in hepatoblastoma unfavorable?
Shengmei Zhou1, Ellen Gomulia1, Leo Mascarenhas2
1Department of Pathology and Laboratory Medicine, Children's Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, CA 90027.
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.
