Background and Objective: Rosai-Dorfman disease (RDD) are usually misdiagnosed because of rarity and nonspecific
clinical and radiological features. The aim of our study is to explore the clinical and imaging characteristics of RDD
to improve diagnostic accuracy.
Methods: Clinical and imaging data in 10 patients with RDD were retrospectively analyzed. 7 patients were underwent
CT scanning and 3 patients were underwent MR examination.
Results: 8 (8/10) patients presented with painless enlarged lymph nodes (LNs) or mass. 3 cases were involved with LNs,
5 cases were involved with extra-nodal tissues, and the remaining 2 cases were involved with LNs and extra-nodal
tissue simultaneously. In enhanced CT images, enlarged LNs displayed mild or moderate enhancement, and 2 cases
showed heterogeneous ring-enhancement. MR features of 3 patients with extra-nodal RDD, 2 cases showed a mass
located in the subcutaneous and anterior abdominal wall respectively, and 1 case showed an intracranial mass. Besides,
all lesions showed high signal foci on DWI images, and were characterized by marked heterogeneous enhancement
with blurred edge. The dural/fascia tail sign and dilated blood vessels could be seen around all the lesions on enhanced
MRI. Radiological features of 2 cases with LN and extranodal tissue involved, one case presented with the swelling and
thickening of pharyngeal lymphoid ring and nasopharynx, meanwhile with enlarged LNs in bilateral submandibular
area, neck and abdominal cavity, and also companied with osteolytic lesion in right proximal humerus. All these LNs
displayed mild and moderate enhancement on CT images. Another case showed enlarged LNs in bilateral neck
accompanied with soft tissue mass in the sinuses.
Conclusions: RDD occurred commonly in young and middle-aged men and presented with painless enlarged LNs or
mass.RDD had a huge diversity of imaging findings, which varied with different location. The radiological features,
such as small patches of high signal foci in the masses on DWI images, heterogeneous enhancement and blood vessels
around the masses, are helpful in diagnosis of extranodal RDD.
Isolated cerebral Rosai–Dorfman disease presenting as a sole mass protruding into the fourth ventricle: A case report