idiopathic retroperitoneal fibrosis
IRF
Idiopathic retroperitoneal fibrosis (IRF) is an uncommon disease of obscure etiology and pathobiology.
Synopsis
spindle-shaped cells that expressed the immunophenotype of a tissue macrophage (8470762)
- Leu 3a,b (CD4)+
- MY7 (CD13)+
- Leu M5 (CD11c)+
- KP-1 (CD68)
- EBM-11 (CD68)+
- human leukocyte antigen (HLA-DR)+
- leukocyte common antigen (CD45)+
- HAM-56+
- MAC387+
A subpopulation of these cells also reacted with an antibody to the "activation" antigen, interleukin 2R (CD25).
Differential diagnosis
fibroblastic tumors and fibrous tumors
- keloids
- desmoid fibromatosis (desmoid tumor, aggressive fibromatosis)
References
Hughes D, Buckley PJ. Idiopathic retroperitoneal fibrosis is a macrophage-rich process. Implications for its pathogenesis and treatment. Am J Surg Pathol. 1993 May;17(5):482-90. PMID: 8470762