Hepatocellular malignant neoplasm, NOS: a clinicopathological study of 11 cases from a single institution

Autor:	James E. Stein, Larry Wang, Shveta Gupta, Leo Mascarenhas, Kasper S. Wang, Rajkumar Venkatramani, Shengmei Zhou
Rok vydání:	2017
Předmět:	Hepatoblastoma Male Pathology medicine.medical_specialty Carcinoma Hepatocellular Histology Adolescent medicine.medical_treatment Glypican 3 Article Pathology and Forensic Medicine 03 medical and health sciences 0302 clinical medicine Immunophenotyping Biomarkers Tumor medicine Humans Child Retrospective Studies Chemotherapy Lung business.industry Liver Neoplasms General Medicine medicine.disease digestive system diseases medicine.anatomical_structure Child Preschool 030220 oncology & carcinogenesis Hepatocellular carcinoma Female 030211 gastroenterology & hepatology business Immunostaining
Zdroj:	Histopathology
ISSN:	0309-0167
Popis:	Aims The primary aim of this study is to characterize hepatocellular malignant neoplasm, NOS (HEMNOS), a new provisional entity describing a subset of paediatric hepatocellular tumours, which have histological features of neither typical hepatoblastoma (HB) nor hepatocellular carcinoma (HCC). Methods and results The clinicopathological features of 11 patients with HEMNOS were analysed retrospectively. The median age and serum alpha-fetoprotein level at diagnosis was 7 years and 182 000 ng/ml, respectively. Ten patients presented with pretreatment extent of disease (PRETEXT) stages III/IV multifocal tumours, eight with major vascular involvement, three with lung metastases and three with extrahepatic extension. The original pathology diagnoses were: HB in seven patients, HCC in two and HEMNOS in two. Our pathology review of pre-chemotherapy specimens showed that six tumours had equivocal/overlapping histological features of HB and HCC, four had predominant HB histology along with focal HCC-like histology and one had HB histology. Seven of nine post-chemotherapy resection specimens showed predominant HCC-like histology. Beta-catenin, glypican 3 and spalt-like transcription factor 4 immunostaining showed that all the tumours had a mixed HB/HCC immunophenotype. Telomerase reverse transcriptase immunostaining showed nuclear staining in nine of the 11 tumours. All patients received chemotherapy and achieved gross total primary tumour resection. Nine of the 11 patients were treated with established HB chemotherapy regimens. After a median follow-up of 6.1 years (range: 1.2–11.8 years), all patients were in remission. Conclusions HEMNOS is a subtype of HB with focal HCC-like histology, a high-risk clinical profile but favourable outcome following chemotherapy and complete tumour resection.
Databáze:	OpenAIRE
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