ABSTRACT
Objective To summarize the clinical and imaging characteristics of Rosai-Dorfman disease (RDD) in order to improve the diagnosis of the disease.Methods The clinical and imaging features of 10 patients with RDD proved by pathology were retrospectively analyzed and the related literatures were reviewed.Results Ten patients including 7 males and 3 females,aged 8 to 54 years old,with average of 32 years old.White blood cells (BCA) and erythrocyte sedimentation rate (ESR) in 8 cases were normal.Eight cases manifested as painless enlarged lymph node or mass,of which 4 cases were accompanied with multisystem involvement.Three lesions involved lymph nodes,5 lesions were located in extranodal sites,while 2 lesions involved both lymph node and extranodal sites.RDD occurred in lymph nodes (n=3) manifested as multiple enlarged lymph nodes on CT imaging,showing mild or moderate the enhancement,or a heterogeneous ring-enhancement.Of 5 extranodal RDD lesions,3 cases underwent MR exmination.2 lesions manifested as subcutaneous soft tissue mass on left shoulder and anterior abdominal wall,1 lesion manifested as intracranial mass accompany with localised cranial bone destruction.Lesions in all 3 cases had poor margins and showed scatted areas of high signal on diffusion weighted images.Lesions exhibited remarkable heterogeneous enhancement on Gd-DTPA enhanced MR imaging,dural/fascia tail sign and dilated blood vessels could be found around the lesions.Intrahepatic RDD lesion showed fast washing out phenomenon on CT enhanced imaging.In 2 cases involved both lymph node and extranodal sites,1 case manifested as swelling pharyngeal lymph chain and nasopharynx,enlargement of lymph nodes in bilateral submandibular and cervical region abdominal cavity on CT imaging,the enhancement is mild and moderate,accompany with osteolytic lesions in right proximal humerus on X-ray.For the other case,CT images manifested as bilateral neck lymphadenopathy,accompanied with soft tissue mass in nasal cavity,the lesion showed mild/moderate enhancement.Conclusions RDD mostly appears in young and middle-aged men,and is rarely preoperatively diagnosed.RDD mainly manifests as painless enlarged lymph node or mass.RDD has a broad spectrum of radiographic appearance,varied according to different location of lesion.Scatted higher signal areas on diffusion weighted images in the masses and significantly heterogeneity enhancement with dilated blood vessels around the lesion could be helpful in diagnosing of extranodal RDD.