Document Detail


Childhood undifferentiated embryonal liver sarcoma: clinical features and immunohistochemistry analysis.
MedLine Citation:
PMID:  18926232     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
PURPOSE: The aim of the study was to report on 3 cases of childhood undifferentiated embryonal liver sarcoma (UELS) and to highlight the clinical features, laboratory findings, diagnosis, and management of this rare disease. METHODS: The patients' age, sex, clinical features, laboratory findings, pathologic results, and therapy were reviewed. Immunohistochemistry analysis was performed on the resected mass sections. RESULTS: In this study, 2 cases were female (aged 8 and 12 years) and 1 was male (aged 9 years). The causes of hospitalization were mainly abdominal pain, mass, or fever. An elevated erythrocyte sedimentation rate was noted in 2 available cases, and alpha fetoprotein (AFP) was within the normal range. Imaging findings indicated a well-defined heterogeneous large mass in the right lobe. Histopathologic evaluation of the mass confirmed the diagnosis of UELS. Immunohistochemical staining showed that vimentin and CD68 antigen were positive in all samples, whereas desmin was positive in one sample. Surgery with chemotherapy was performed in 2 cases. CONCLUSION: The diagnosis of UELS depends mainly on the pathologic findings. Undifferentiated embryonal liver sarcoma should be included in the differential diagnosis of mass in the liver, especially with well-defined heterogeneous imaging findings and normal AFP. Diagnosis and management should be made early as UELS is a potentially treatable tumor.
Authors:
Zhong Gu Wei; Lan Fang Tang; Zhi Min Chen; Hong Fang Tang; Min Ju Li
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of pediatric surgery     Volume:  43     ISSN:  1531-5037     ISO Abbreviation:  J. Pediatr. Surg.     Publication Date:  2008 Oct 
Date Detail:
Created Date:  2008-10-17     Completed Date:  2009-02-23     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0052631     Medline TA:  J Pediatr Surg     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1912-9     Citation Subset:  IM    
Affiliation:
Department of Pathology, The Children's Hospital of Zhejiang University School of Medicine, 57 Zhugan Xiang, Hangzhou 310003, China.
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MeSH Terms
Descriptor/Qualifier:
Antigens, CD / analysis
Antigens, Differentiation, Myelomonocytic / analysis
Antineoplastic Combined Chemotherapy Protocols / therapeutic use
Child
Cisplatin / administration & dosage
Combined Modality Therapy
Cyclophosphamide / administration & dosage
Desmin / analysis
Dexamethasone / administration & dosage
Doxorubicin / administration & dosage
Fatal Outcome
Female
Hepatectomy
Humans
Ifosfamide / administration & dosage
Liver Neoplasms / chemistry,  diagnosis,  drug therapy,  pathology*,  radiotherapy,  surgery
Male
Neoadjuvant Therapy
Neoplasm Proteins / analysis*
Neoplasms, Germ Cell and Embryonal / chemistry,  diagnosis,  drug therapy,  pathology*,  radiotherapy,  surgery
Remission Induction
Sarcoma / chemistry,  diagnosis,  drug therapy,  pathology*,  radiotherapy,  surgery
Tumor Markers, Biological / analysis*
Vimentin / analysis
Vinblastine / administration & dosage
Chemical
Reg. No./Substance:
0/Antigens, CD; 0/Antigens, Differentiation, Myelomonocytic; 0/CD68 antigen, human; 0/Desmin; 0/Neoplasm Proteins; 0/Tumor Markers, Biological; 0/Vimentin; 15663-27-1/Cisplatin; 23214-92-8/Doxorubicin; 3778-73-2/Ifosfamide; 50-02-2/Dexamethasone; 50-18-0/Cyclophosphamide; 865-21-4/Vinblastine

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