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Neuroradiology

E3112. Role of Imaging in Intracranial Rosai Dorfman Disease

Daruwalla V,  Gunduru M,  Raval K,  Abdelhadi S,  Zuk C. Wayne State University/Detroit Medical Center, Detroit, MI

Address correspondence to V. Daruwalla (vistasp.daruwalla@gmail.com)

Background Information: Rosai Dorfman disease is a nonneoplastic proliferative disease of histiocytes. It is characterized by painless lymphadenopathy, fever, and weight loss with nodal or extranodal involvement. CNS manifestations are extremely rare and patients with intracranial involvement usually present with clinical and radiologic findings suggestive of a meningioma. CT, MRI, and FDG PET are crucial for diagnosing Rosai Dorfman disease. The purpose of this exhibit is to review the various imaging presentations of Rosai Dorfman disease and its differentials. We also review the progression of the disease on imaging.

Educational Goals/Teaching Points: In this exhibit, we review the most important and clinically relevant findings of Rosai Dorfman disease. We include incidence, etiology, and pathophysiology of Rosai Dorfman disease; imaging findings of intracranial lesion on various imaging modalities and understanding its progression; management of Rosai Dorfman disease; and nodal and extranodal involvement throughout the body. We also review the diseases that must be considered in our list of differential diagnosis. Viewers of this exhibit will benefit from understanding the incidence, pathophysiology, intracranial imaging findings on various imaging modalities associated with Rosai Dorfman disease.

Key Anatomic/Physiologic Issues and Imaging Findings/Techniques: We describe the imaging features of intracranial Rosai Dorfman disease and related differential diagnoses on multiple imaging modalities.

Conclusion: Rosai-Dorfman disease is a well-known clinicopathologic disease. The disease may be noted as nodal or extranodal. It usually presents with only extranodal involvement of the skin, orbit, upper respiratory tract, testes, or rarely CNS. Rosai-Dorfman disease should be considered in the differential diagnoses of both dural-based and intraparenchymal lesions of the CNS, where dural-based lesions mimic the radiologic appearance of a meningioma, and intraparenchymal lesions may mimic granulomatous lesions. MRI of the brain and PET scan assist in characterizing such nonspecific lesions and directing an appropriate line of treatment. Rosai Dorfman disease should be considered as one of the differentials even for isolated intracranial lesions.