Disease Characteristics, Radiologic Patterns, Comorbid Diseases, and Ethnic Differences in 32 Patients With Rosai-Dorfman Disease

Abstract

Objective 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. Methods 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. Results 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). Conclusions 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.

Case Report: Rosai-Dorfman Disease Involving Sellar Region in a Pediatric Patient: A Case Report and Systematic Review of Literature

“…Other common symptoms include subcutaneous masses, bone pain, constitutional symptoms, and abdominal pain ( 8 ). Surprisingly, extra nodal presentations were more common than nodal lesions ( 4 , 8 ).…”

“…Rosai-Dorfman disease was first reported in 1969 as sinus histiocytosis with massive lymphadenopathy, which was a benign and progressive lymphoproliferative disorder presenting with lymphadenopathies and polyclonal hypergammaglobulinemia ( 1 , 2 ). RDD is more frequently seen in children and young adults than in older adults ( 3 ), while it seems not to present a explicit sex predilection ( 4 ). In addition, extranodal involvement has been reported in 43% of RDD, with the most common sites being the skin and the central nervous system (CNS) ( 5 , 6 ).…”

Rosai-Dorfman Disease of Bone and Soft Tissue

“…10,13,16,21,22 On magnetic resonance imaging, the lesions usually show well-demarcated borders and have heterogeneous intermediate to low signal on T1-weighted images and bright signal on T2-weighted images (Figure 1, C and D). [10][11][12]17,20,30 On bone scan, the lesions demonstrate increased radiotracer uptake, which is very helpful for the diagnosis of multifocal lesions. 10,17,27 The lesions are hypermetabolic on positron emission tomography-computed tomography, which is another useful modality for the detection of multifocal asymptomatic lesions and evaluation of treatment response.…”

“…10,17,27 The lesions are hypermetabolic on positron emission tomography-computed tomography, which is another useful modality for the detection of multifocal asymptomatic lesions and evaluation of treatment response. 20,[30][31][32] Whole-body diffusion-weighted magnetic resonance imaging has also been used to detect multifocal bone disease and to evaluate response to therapy without the need for radiation or intravenous contrast agents. 33…”

“…In the soft tissues, RDD more commonly involves the subcutaneous adipose tissue and less commonly the deep soft tissue. 5,6,18,19,30 Subcutaneous lesions may extend to the overlying reticular dermis. 6 Red-brown skin discoloration has been described in subcutaneous lesions with dermal involvement.…”