Mixed epithelial mesenchymal (MEM) hepatoblastoma with teratoid features is rare histological variant of hepatoblastoma and described in case reports. Growing teratoma syndrome (GTS) is a rare and often unrecognised phenomenon generally associated with less than 5% of germ cell tumour. It is defined by enlarging tumour mass which is generally mature teratoma with normal or significantly decreasing tumour markers during chemotherapy. The treatment outcomes in GTS are dependent on early recognition and complete surgical excision. We describe a rare case of MEM hepatoblastoma with teratoid features with GTS in an infant who had a delay in definitive management due to late diagnosis of GTS.
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http://dx.doi.org/10.1136/bcr-2020-241197 | DOI Listing |
Zhonghua Bing Li Xue Za Zhi
June 2024
Department of Pathology, Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai 200092, China.
To investigate the clinicopathological features of children with metachronous or synchronous primary tumors and to identify related genetic tumor syndromes. The clinicopathological data of 4 children with multiple primary tumors diagnosed in the Xinhua Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China from 2011 to 2023 were collected. The histological, immunophenotypic and molecular characteristics were examined using H&E staining, immunohistochemical staining, PCR, Sanger sequencing and next-generation sequencing (NGS).
View Article and Find Full Text PDFIran J Pathol
June 2023
Department of Pediatric Oncology, Kidwai Memorial Institute of Oncology, Rajiv Gandhi University of Health Sciences, Bangalore, Karnataka, India.
Background & Objective: Hepatoblastoma encompasses 1% of pediatric malignancies and is the most common liver malignancy in children. Ninety percent of cases are younger than 5 years of age. Clinical and pathological risk stratification forms a crucial role in determining the treatment strategy.
View Article and Find Full Text PDFCancers (Basel)
December 2022
Department of Pathology, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH 45229, USA.
Hepatoblastomas (HB) are the most common pediatric liver tumor with several subgroups described, of which teratoid HB is the rarest. The aim of this study is to characterize the histologic and phenotypic spectrum of teratoid HB in order to better understand the biology and behavior of these tumors. A retrospective analysis of all teratoid HB diagnosed at a major pediatric hospital as well as the consultation files of one of the authors (SR) was performed with the available clinical data and surgical pathology material reviewed.
View Article and Find Full Text PDFClin Pathol
October 2022
Pathology Department, Oncology National Institute, Faculty of Medicine and Pharmacy, Mohammed V University, Rabat, Morocco.
Adult hepatoblastoma is a rare tumor whose etiology and mechanisms of development are still incompletely understood. Imaging and biological tests such as AFP and liver enzymes are non-specific. Histologically, there are 2 histological variants: pure epithelial with 5 types (pure fetal, embryonal, small cell undifferentiated, cholangioblastic, and macrotrabecular), a mixed epithelial and a mesenchymal variant with or without a teratoid contingent.
View Article and Find Full Text PDFJ Clin Exp Hepatol
April 2022
Pediatric Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India.
Background: Teratoid hepatoblastoma is an uncommon but well-recognized variant of mixed hepatoblastoma.
Case Report: A one-year female child presented with palpable and progressively increasing right abdominal mass for 3 months. The contrast-enhancing computed tomography (CECT) abdomen revealed a large heterogeneous hepatic mass measuring 12 × 6.
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