Document Detail

Primary gastrointestinal clear cell sarcoma: report of 2 cases, one case associated with IgG4-related sclerosing disease, and review of literature.
MedLine Citation:
PMID:  19118779     Owner:  NLM     Status:  MEDLINE    
Clear cell sarcoma (CCS) is a distinctive soft tissue sarcoma that shows melanocytic differentiation. Primary gastrointestinal (GI) CCSs have been rarely reported, but to our knowledge, no association between GI CCSs and immunoglobulin G4 (IgG4)-related sclerosing disease has been described in the literature. We experienced 2 cases of CCS that arose in the small intestine and metastasized to the liver. Histologic features and immunophenotype were typical of CCS. One of them showed a unique peritumoral sclerosing inflammatory reaction, which was highly reminiscent of IgG4-related sclerosing inflammatory disease. Dense lymphoplasmacytic infiltration with extensive sclerosis and obliterative phlebitis was observed in the immediate vicinity of the primary and metastatic tumors, but not in the distant areas from the tumor. The average number of IgG4-positive plasma cells was more than 50 per high-power field. We report 2 cases of primary GI CCS with one case showing a unique peritumoral IgG4-related lymphoplasmacytic sclerosing inflammation.
Mee Joo; Sun Hee Chang; Hanseong Kim; Jerad M Gardner; Jae Yun Ro
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2008-12-12
Journal Detail:
Title:  Annals of diagnostic pathology     Volume:  13     ISSN:  1532-8198     ISO Abbreviation:  Ann Diagn Pathol     Publication Date:  2009 Feb 
Date Detail:
Created Date:  2009-01-02     Completed Date:  2009-04-03     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9800503     Medline TA:  Ann Diagn Pathol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  30-5     Citation Subset:  IM    
Department of Pathology, Inje University Ilsan Paik Hospital, Goyang, South Korea.
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MeSH Terms
Ileum / pathology
Immunoglobulin G / blood,  immunology*
Jejunal Neoplasms / pathology*,  radiography
Liver Neoplasms / immunology,  pathology*,  radiography
Lymphatic Metastasis
Middle Aged
Sarcoma, Clear Cell / immunology,  pathology*,  radiography
Reg. No./Substance:
0/Immunoglobulin G

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