Disease Characteristics, Radiologic Patterns, Comorbid Diseases, and Ethnic Differences in 32 Patients With...

Rosai-Dorfman disease (RDD) is a rare and idiopathic nonneoplastic disease of histiocytes that is characterized by lymphadenopathy and extranodal disease. In this study, we documented anatomical preferences, imaging findings, comorbid diseases, and ethnic differences in 32 RDD patients.

We conducted a retrospective review of pathologically confirmed cases seen at our institution from 1998 to 2016. These cases were analyzed for (a) anatomical locations, (b) radiologic appearance, (c) comorbid diseases, and (d) differences between ethnic groups.

We found 32 patients with RDD, 18 were women and 14 were men. There were 51 lesions in all patients, 23.5% of which were nodal, involving 11 lymph node regions, and 76.5% were extranodal. Cervical lymph nodes and maxillofacial area were the most common affected nodal and extranodal locations, respectively. Only 4 (12.5%) of 32 patients had pure nodal involvement, whereas 20 (62.5%) of 32 had pure extranodal disease and 8 (25%) of 32 had mixed nodal and extranodal disease.Anatomically, RDD affected multiple organs in our cohort, including the lymphatic system, maxillofacial area (glandular and nonglandular tissues), superficial soft tissue, central nervous system, breast, peritoneum, gastrointestinal tract, and lungs.Radiologically, RDD presentation was variable from an organ to another. However, most lesions were hypermetabolic on 18F-fluorodeoxyglucose positron-emission tomography/computed tomography and isointense on T1-weighted magnetic resonance imaging. Computed tomographic findings were extremely variable between organs.Comorbid diseases were found in 11 patients. Those patients had 17 comorbid diseases; the most common were autoimmune diseases, viral diseases, and cancer.The organ distribution of RDD was slightly different between ethnic groups. The most frequent disease location for African Americans was lymph nodes; for whites, central nervous system and nonglandular maxillofacial (27.3% each); for Asians, lymph nodes, subcutaneous tissue, and nonglandular maxillofacial (25% each); and for Hispanics, lymph nodes and glandular maxillofacial (33.3% each).

Rosai-Dorfman disease represents a wide-spectrum disease not limited to lymph nodes. Radiologically, RDD has diverse imaging findings. However, most lesions were hypermetabolic on 18F-fluorodeoxyglucose positron-emission tomography/computed tomography and isointense on T1-weighted imaging. Patients with RDD have a high rate of comorbid diseases including autoimmune disease, viral infections, and cancer.