Document Detail


Rhabdoid Carcinoma of the Colon: A Distinct Entity With A Very Aggressive Behavior: A Case Report Associated With a Polyposis Coli and Review of the Literature.
MedLine Citation:
PMID:  21791485     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Rhabdoid colon tumors (RCTs) are rare lesions whose existence as an independent distinct entity remains controversial. To date, 6 RCTs have been reported. This study reports a novel case associated with polyposis coli in a 73-year-old woman. Histologically, the neoplasia was heterogeneous consisting of an adenocarcinoma associated with rhabdoid features. In rhabdoid component, an intense expression of MSH2 was noted but MLH1 was negative. A BRAF V600E mutation and no KRAS mutations were identified. The promoter regions of subset of genes highly specific to characterize the CIMP status (NEUROG1, IGF2, RUNX3, SOCS1, including MLH1) were hypermethylated, suggesting the presence of CIMP+ and MSI high tumor. In conclusion, all RCTs have similar clinical features. The presence of polyposis and adenocarcinoma component as well as the expression of mesenchymal marker suggests a sarcomatous dedifferentiation. It is argued that RCT could be a very aggressive entity of colon, which could benefit from new biological colonic treatments.
Authors:
Andrea Remo; Caterina Zanella; Enrico Molinari; Alberto Talamini; Filippo Tollini; Paolo Piacentini; Paolo Battaglia; Elisabetta Baritono; Andrea Bonetti; Franco Lanza; Antonio Fasolin; Erminia Manfrin; Roberto Vendraminelli
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-7-26
Journal Detail:
Title:  International journal of surgical pathology     Volume:  -     ISSN:  1940-2465     ISO Abbreviation:  -     Publication Date:  2011 Jul 
Date Detail:
Created Date:  2011-7-27     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9314927     Medline TA:  Int J Surg Pathol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
"Mater Salutis" Hospital, ULSS 21 Legnago, Verona, Italy.
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