Idiopathic retroperitoneal fibrosis mimicking malignant lymphoma

EFH Bommel, M (Mark) de Mol, Ton Langerak, PJ (Pieter) Westenend

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Abstract

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-alpha 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.
Original languageUndefined/Unknown
Pages (from-to)672-676
Number of pages5
JournalPathology International
Volume61
Issue number11
DOIs
Publication statusPublished - 2011

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  • EMC MM-02-72-01

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