Document Detail


Monoclonality of multifocal epithelioid hemangioendothelioma of the liver by analysis of WWTR1-CAMTA1 breakpoints.
MedLine Citation:
PMID:  22429593     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Similar to other vascular tumors, epithelioid hemangioendothelioma (EHE) can have multifocal presentation in up to 50% of cases. However, whether multifocal EHE represents an unusual pattern of metastasis or multiple separate primary tumors remains to be elucidated. Our recent identification of a WWTR1-CAMTA1 fusion as the genetic hallmark of EHE irrespective of anatomic location was used to clarify this question by comparing the similarity of translocation breakpoints. In our previous study, we found variability of the fusion transcripts of the t(1;3)(p36;q25) translocation among different patients with EHE. Thus, we undertook a molecular analysis of six samples from two patients with multicentric hepatic EHE to test our hypothesis that the presence of identical breakpoints in WWTR1 and CAMTA1 support the monoclonal nature of multifocal EHE. Using reverse transcription-polymerase chain reaction (RT-PCR) and subsequent sequencing, we confirmed an identical WWTR1-CAMTA1 fusion transcript product from different nodules in each patient. Our results confirm that multifocal EHE are monoclonal and thus represent metastatic implants of the same neoplastic clone rather than a "field-effect" or synchronous occurrence of multiple neoplastic clones.
Authors:
Costantino Errani; Yun Shao Sung; Lei Zhang; John H Healey; Cristina R Antonescu
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Cancer genetics     Volume:  205     ISSN:  2210-7762     ISO Abbreviation:  Cancer Genet     Publication Date:    2012 Jan-Feb
Date Detail:
Created Date:  2012-03-20     Completed Date:  2012-05-07     Revised Date:  2013-04-01    
Medline Journal Info:
Nlm Unique ID:  101539150     Medline TA:  Cancer Genet     Country:  United States    
Other Details:
Languages:  eng     Pagination:  12-7     Citation Subset:  IM    
Copyright Information:
Copyright © 2012 Elsevier Inc. All rights reserved.
Affiliation:
Department of Surgery, Orthopaedic Surgery Service, Memorial Sloan-Kettering Cancer Center, New York, NY, USA.
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MeSH Terms
Descriptor/Qualifier:
Adult
Base Sequence
Calcium-Binding Proteins / analysis,  genetics*
Chromosome Breakpoints*
Clonal Evolution / genetics*,  physiology
Clone Cells / metabolism,  pathology
Female
Hemangioendothelioma, Epithelioid / genetics,  pathology*
Humans
Intracellular Signaling Peptides and Proteins / analysis,  genetics*
Liver Neoplasms / genetics,  pathology*
Male
Molecular Sequence Data
Neoplasms, Multiple Primary / genetics,  pathology*
Reverse Transcriptase Polymerase Chain Reaction
Sequence Analysis, DNA
Trans-Activators / analysis,  genetics*
Grant Support
ID/Acronym/Agency:
P01 CA047179/CA/NCI NIH HHS; P01 CA047179-15A2/CA/NCI NIH HHS; P50 CA 140146-01/CA/NCI NIH HHS; P50 CA140146/CA/NCI NIH HHS; P50 CA140146-01A1/CA/NCI NIH HHS
Chemical
Reg. No./Substance:
0/CAMTA1 protein, human; 0/Calcium-Binding Proteins; 0/Intracellular Signaling Peptides and Proteins; 0/Trans-Activators; 0/WWTR1 protein, human
Comments/Corrections

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