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Case Reports
. 2021 Jul 26;9(21):6032-6040.
doi: 10.12998/wjcc.v9.i21.6032.

Rosai-Dorfman disease in the spleen of a pediatric patient: A case report

Hwaseong Ryu  1 , Jae-Yeon Hwang  2 , Yong-Woo Kim  1 , Tae-Un Kim  1 , Joo-Yeon Jang  1 , Su-Eun Park  3 , Eu-Jeen Yang  3 , Dong-Hoon Shin  4
Affiliations
Case Reports

Rosai-Dorfman disease in the spleen of a pediatric patient: A case report

Hwaseong Ryu et al. World J Clin Cases. .

Abstract

Background: Rosai-Dorfman disease (RDD) is a rare histiocytic proliferation of unknown etiology commonly found in children and adolescents. The common manifestation of RDD is massive and painless bilateral cervical lymphadenopathy with extranodal disease. While extranodal involvement in RDD is common, the spleen is an infrequent site of disease.

Case summary: We report a 10-mo-old female infant with RDD presenting multiple splenic masses without cervical lymphadenopathy. She had fever, and blood tests showed leukocytosis, anemia, and elevated erythrocyte sedimentation rate and C-reactive protein. Ultrasound, computed tomography, and magnetic resonance images demonstrated multiple splenic masses. Despite antibiotic therapy, her symptoms were not relived. She underwent diagnostic splenectomy and was discharged with recovery.

Conclusion: In pediatric patients with refractory infectious symptoms or hematological abnormalities, clinicians should suspect RDD, even in patients without significant lymphadenopathy.

Keywords: Case report; Computed tomography; Magnetic resonance imaging; Rosai–Dorfman disease; Sinus histiocytosis with massive lymphadenopathy; Ultrasonography.

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Conflict of interest statement

Conflict-of-interest statement: The authors declare that they have no conflicts of interest.

Figures

Figure 1
Figure 1
Ultrasound images of two solid spleen masses. A and D: B-mode images demonstrated irregular and hypoechoic masses; B and E: Color Doppler images showed similar mass vascularity to that of a normal area of the spleen; C and F: Shear wave elastography showed similar shear wave velocity to that of ad normal area of the spleen.
Figure 2
Figure 2
Computed tomography images of two solid splenic masses. A: Axial image showed smaller hypodense mass; B: Axial image demonstrated larger exophytic hypodense mass; C: Coronal image revealed larger exophytic hypodense mass.
Figure 3
Figure 3
Magnetic resonance images of splenic masses. A-E: Magnetic resonance imaging (MRI) images demonstrated mass in upper level of spleen; F-J: MRI images showed mass in middle level of spleen; K-O: MRI images illustrated mass in lower level of spleen. A, F and K: Splenic masses showed low signal intensity on T2-weighted images; B, G and L: Splenic masses demonstrated various signal intensities on T1-weighted images; C, H and M: Splenic masses showed low signal intensity in the late arterial phase; D, I and N: Splenic masses showed persistent low signal intensity in the portal venous phase; E, J and O: Splenic masses showed still low signal intensity in the 5-min delayed phase.
Figure 4
Figure 4
Pathologic images of splenic masses. A: Resected specimen showed contour bulging splenic mass; B: Cut surface of the resected specimen; C: Hematoxylin and eosin-stained section (200 ×) showed histiocytic proliferation with emperipolesis (arrows); D: Proliferating histiocytes were positive for S-100 protein stained section (400 ×); E: Proliferating histiocytes were positive for CD68 antigen stained section (400 ×); F: proliferating histiocytes were negative for CD1a stained section (400 ×).
See this image and copyright information in PMC

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