Multimodality imaging manifestations of Rosai-Dorfman disease

Tanvi Vaidya; Abhishek Mahajan; Swapnil Rane

doi:10.1177/2058460120946719

Multimodality imaging manifestations of Rosai-Dorfman disease

Acta Radiol Open. 2020 Aug 19;9(8):2058460120946719. doi: 10.1177/2058460120946719. eCollection 2020 Aug.

Authors

Tanvi Vaidya¹, Abhishek Mahajan², Swapnil Rane³

Affiliations

¹ Department of Radiodiagnosis and Imaging, Ruby Hall Clinic, Pune, India.
² Department of Radiodiagnosis and Imaging, Tata Memorial Hospital, Mumbai, India.
³ Department of Pathology, Tata Memorial Hospital, Mumbai, India.

Abstract

Background: Rosai-Dorfman disease (RDD) is a rare lympho-histiocytic disorder of indeterminate etiology usually presenting with lymph node involvement, and infrequently with extra-nodal manifestations. The diagnosis of this condition is challenging due to the wide spectrum of disease manifestations.

Purpose: To elucidate the radiologic features of this disease using multimodality imaging in histopathologically proven cases and to identify characteristic features that would enable its differentiation from its mimics.

Material and methods: We retrospectively evaluated imaging studies of 19 patients with histopathologically confirmed RDD presenting to our institute between January 2004 and March 2016. Imaging modalities included magnetic resonance imaging (MRI), computed tomography, FDG-positron emission tomography (PET) CT, mammography, and ultrasonography.

Results: Lymphadenopathy was the most common imaging feature in our study, seen in 11 (57.8%) cases followed by sino-nasal involvement in 7 (36.8%) cases and intracranial masses in 5 (26.3%) cases. Bilateral homogeneously enhancing cervical lymphadenopathy with avidity on FDG-PET scans was the predominant abnormality on imaging. Sino-nasal involvement manifested as homogeneously enhancing soft-tissue masses occupying the paranasal sinuses. Intracranial disease manifested as sellar/suprasellar masses, dural-based lesions along the cerebral hemispheres and choroid plexus enlargement. Unusual disease manifestations included spinal, osseous, and breast lesions.

Conclusion: Due to the high likelihood of multifocal involvement, the recognition of RDD at one site necessitates screening of other sites for disease. Homogeneously enhancing, FDG-avid lymphadenopathy and sino-nasal masses in association with hypointense extra-nodal lesions on T2-weighted MRI are imaging features which could aid the diagnosis of RDD and facilitate its differentiation from pathologies that present in a similar manner.

Keywords: Rosai-Dorfman; computed tomography; lymphadenopathy; magnetic resonance imaging; paranasal sinuses.