PMC9846742_01
Images of extranodal lesions in the patient with Rosai-Dorfman disease. (A, B) Pelvic lesion. (A) Computed tomography (CT) scan showing an intra-pelvic mass enhanced predominantly in the peripheral zone. (B) Magnetic resonance imaging (MRI), of the intra-pelvic mass, showing heterogeneous high-intensity signals on diffusion-weighted imaging, accompanied by peritoneal thickening. (C-F) 18F-fluorodeoxyglucose-positron emission tomography (FDG-PET)-CT of the (C) facial skin lesion (arrow), with a maximum standardized uptake value (SUVmax) of 10.7, (D) pelvic lesion (arrow), with an SUVmax of 6.2, and (E, F) peri-atrial lesion spreading from the interatrial septum to the left atrium, with an SUVmax of 25. (G) Electrocardiography-gated contrast-enhanced CT, showing homogeneous wall thickening around the left atrium (arrows). (H-J) Hepatic lesion. (H) Ultrasonography, showing a homogeneous hypoechoic nodule (arrow). (I) Contrast-enhanced CT imaging, showing a hypervascular nodule in the left lateral hepatic segment. (arrow). (J) Gadolinium ethoxybenzyl diethylenetriamine pentaacetic acid-enhanced MRI, showing a homogeneously enhanced nodule in the arterial phase (arrow).
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Systemic CT showed a contrast-enhanced intra-pelvic mass with irregular margins, along with peripheral enhancement ( Figure 1A ), but no other lesions in the heart, liver, or lymph nodes. Magnetic resonance imaging (MRI) showed an intra-pelvic mass with low-intensity signals on T2-weighted images and heterogeneous high-intensity signals on diffusion-weighted images ( Figure 1B ). FDG-PET-CT revealed abnormal FDG uptake in the left cheek; cervical, axillary, and inguinal lymph nodes; periatrium, colon, and pelvis ( Figures 1C-F ). Electrocardiography-gated contrast-enhanced CT revealed homogeneous wall thickening around the left atrium ( Figure 1G ). Abdominal ultrasonography showed a homogeneous hypoechoic nodule ( Figure 1H ). Contrast-enhanced CT and gadolinium ethoxybenzyl diethylenetriamine pentaacetic acid (Gd-EOB-DTPA)-enhanced MRI demonstrated a hyper vascular nodule approximately 10 mm in diameter in the left lateral hepatic segment ( Figures 1I, J ). CD138-positive cells was approximately 10%. CT-guided biopsy of the pelvic mass ( Supplemental Figure 1 ) and re-examination of the skin biopsy of the leg obtained before hospitalization showed similar findings.
Histological findings of the facial skin biopsy. (A, B) Hematoxylin and eosin staining, showing (A) large round cells aggregated diffusely in the upper dermis immediately beneath the epidermis, and (B) emperipolesis, in which lymphocytes and neutrophils are engulfed. (C, D) Immunohistochemical analysis, showing that the large round cells were positive for (C) CD68 and (D) S-100 protein. Original magnifications: x 100 (A), x 400 (B-D). Scale bars: 100 mum (A) and 20 mum (B-D).
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Immunohistochemistry revealed that these histiocytes were positive for CD68 and S-100 protein ( Figure 2 ).
Whole-body 18F-fluorodeoxyglucose (FDG)-positron emission tomography with computed tomography (PET-CT) before and after treatment. (A) FDG-PET-CT before treatment, showing abnormal FDG uptake in the facial skin, peri-atrial, pelvic (arrows), cervical, axillary, and inguinal lymph nodes, and colon. (B) Follow-up FDG-PET-CT after treatment, showing the disappearance of abnormal FDG uptake.
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FDG uptake by the above-mentioned lesions ( Figure 3 ). Furthermore, abdominal ultrasonography revealed that the liver nodule had disappeared.