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Idiopathic retroperitoneal fibrosis mimicking malignant lymphoma
Author(s) -
van Bommel Eric F. H.,
de Mol Mark,
Langerak Anton W.,
Westenend Pieter J.
Publication year - 2011
Publication title -
pathology international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.73
H-Index - 74
eISSN - 1440-1827
pISSN - 1320-5463
DOI - 10.1111/j.1440-1827.2011.02718.x
Subject(s) - pathology , lymphoma , immunohistochemistry , biopsy , population , medicine , fibrosis , pathogenesis , biology , environmental health
We present a case of atypical idiopathic retroperitoneal fibrosis (iRPF) presenting as a large pelvic tumor, for which it proved difficult to exclude T‐cell malignant lymphoma. Histopathological examination of biopsy material showed collagenous tissue and fat with an exuberant and predominant T‐cell infiltrate, largely consisting of CD4 + cells expressing the IL‐2 receptor‐α chain (CD25). Focal plasma cells were negative for the immunoglobulin G4 (IgG4) isotype. T‐cell receptor gene rearrangement (TRGR) pattern showed a Gaussian distribution, in keeping with a polyclonal T‐cell population. Awareness of the sometimes exuberant and predominant T‐cell infiltrate in iRPF should lead to earlier consideration of this disorder. This is particularly the case where there is an atypically localized and/or extensive mass, for which early exclusion of monoclonality with TRGR may provide helpful. Immunohistochemical findings suggest that CD4 + CD25 + cells, which are part of a naturally occurring population of regulatory T‐cells, may be involved in the pathogenesis of iRPF.