case_images: PMC7180427_01
case_images: Abdominal CT scan showing symmetrical, bilateral perirenal rind-like lesions (arrows) measuring 2.5 cm in thickness, so-called hairy kidney sign.
case_images: CRIPA2020-3081824.001.jpg
case_images: PMC7180427_01_CRIPA2020-3081824.001.jpg
case_images: fig1
case_images: After a thorough clinical history yielded negative results, the patient was sent for an abdominal pelvic CT scan where the identification of rind-like bilateral perirenal masses by the radiologist resulted in the presumptive diagnosis of Erdheim-Chester disease (Figure 1).
case_images: Needle biopsy of perirenal lesion showing (a) collision tumor of ECD with low grade B cell lymphoma; (b) foamy histiocytes, fibrous stroma, and scattered inflammatory cells of ECD; (c) foamy histiocytes admixed with small monomorphous lymphocytes; and (d) small lymphocytic lymphoma.
case_images: CRIPA2020-3081824.002.jpg
case_images: PMC7180427_01_CRIPA2020-3081824.002.jpg
case_images: fig2
case_images: An atypical lymphoid infiltrate with interstitial and nodular architecture accompanied the histiocytosis (Figure 2).
case_images: Immunohistochemical findings of perirenal mass. (a) CD79a, (b) CD3, (c) CD5, (d) CD68. (e) Flow cytometric findings of perirenal mass typical of SLL (CD19+, Kappa+, CD5+, CD23+, CD10-, and FMC7-).
case_images: CRIPA2020-3081824.003.jpg
case_images: PMC7180427_01_CRIPA2020-3081824.003.jpg
case_images: fig3
case_images: A sample of the biopsy was sent for flow cytometric assessment and showed monoclonal B lymphocytes with kappa light chain restriction and typical SLL phenotype (Figure 3).