Radiology-pathology correlation: Rosai-Dorfman disease

Abstract

Rosai-Dorfman Disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare non-Langerhans cell histiocytic neoplasm. Although the disease classically presents as massive painless lymphadenopathy in young adults, RDD can also involve the central nervous system in some patients. CNS lesions, can cause headaches, neurologic deficits, and even neurologic deficits. The imaging appearance of CNS RDD typically mimics that of meningiomas: well-circumscribed dural-based lesions that often have dural tails. However, some imaging clues also exist that might help a radiologist recognize RDD, even before histopathologic confirmation. This radiology-pathology report of a patient with CNS RDD highlights the most pertinent clinical, imaging, and pathologic features of CNS RDD, and discusses what the neuroradiologist needs to know about the disease.

Keywords: MRI; Tumor; pathology.

Figure 1.

Intracranial involvement of disease. Axial fat-saturated post-contrast T1WI (A and B) show diffuse smooth pachymeningeal enhancement over the convexities (long solid arrows), and falx (short arrows). A nodular mass in the left aspect of the foramen magnum (curved arrow) encases the left vertebral artery (dashed arrow). Coronal post-contrast images (C) demonstrate more nodular enhancement along the cavernous sinuses and suprasellar region (long arrows), as well as enhancement along the falx (short arrow). The nodular arrows are hypointense on T2 weighted images (long arrows, D).

Figure 2.

Extracranial disease. Axial fat-saturated post-contrast T1WI (A) show a large expansile and heterogeneously enhancing mass in the right maxillary sinus that extends into the right inferior turbinate (between straight solid long arrows). On coronal fat-saturated T2WI (B), both the mass (straight solid long arrows) and additional involvement of the left inferior turbinate (dashed straight arrows) are seen. The mass is FDG-avid, seen on both whole-body PET imaging (C) and fused axial PET/CT images (D) (solid curved arrows on both). FDG avidity was also seen in the areas of nodular pachymeningeal disease (not shown). Pachymeningeal enhancement was seen extending from the retroclival region into the ventral and dorsal aspects of the upper cervical spine (short arrows, E). A tiny focus of enhancement along a cauda equina nerve root was seen in the lumbar spine (dashed curve arrow, F).

Figure 3.

The prior skin biopsy (performed 17 years prior to presentation) showed a dense dermal and subcutaneous lymphoplasmacytic infiltrate with numerous large histiocytes (panel A), immunoreactive for S100 (panel B) and the histiocytic marker CD68/PGM1 (panel C). The low power inset in B highlights the nodular arrangement of the histiocytes, which were negative for CD1a (not shown). The recent biopsy from the left C1 cervical meninges showed a dense atypical histiocytic infiltration in a background of marked chronic lymphoplasmacytic inflammation and dense fibrosis (panel D). Panels E & F illustrate, respectively, S100 and the histiocytic marker CD163.

Figure 4.

Schematic of typical intracranial findings in Rosai–Dorfman Disease (RDD). As seen in the index case, RDD is often characterized by diffuse dural thickening and/or dural masses, with particular proclivity for the suprasellar, cavernous sinus, and petroclival regions.