Teaching Neuro Image : Isolated intracranial Rosai-Dorfman disease mimicking a meningioma

Abstract

A 56-year-old woman presented with involuntary left arm and leg movements. MRI with gadolinium revealed a heterogeneously enhancing, extra-axial mass at the right frontoparietal convexity, causing mass effect, vasogenic edema, and dural thickening (figure). Intraoperatively the mass was noted to invade nearby cortex, sulci, and the superior sagittal sinus. Meningioma was suspected. Pathology revealed a lymphohistiocytic infiltrate with histiocytes positive for CD 68 and S100 demonstrating emperipolesis (lymphocytophagocytosis) consistent with Rosai-Dorfman disease (figure). The patient had no CNS recurrence or systemic involvement after 12 months of follow-up.Rosai-Dorfman, sinus histiocytosis with massive lymphadenopathy, is a rare disease of the lymphoid tissues with systemic involvement in up to 25% of cases. Neurologic involvement is rare, occurring in 4% of patients in one series. There are approximately 80 cases of CNS involvement in the literature, and the majority of these cases are brain lesions presenting with focal deficits, seizures, or headaches due to mass effects. Sagittal and axial T1-weighted MR images, hematoxylin and eosinstained specimen, and S100-stained specimen Sagittal (A) and axial (B) T1-weighted MR images revealing a contrast-enhancing extra-axial mass lesion with indentation of the cortex, dural thickening, underlying vasogenic edema, and a projection into an intersulcal region. Hematoxylin and eosin-stained specimen (C) shows a lymphohistiocytic infiltrate with histiocytes demonstrating emperipolesis (lymphocytophagocytosis). S100-stained specimen (D) shows histiocytes positive for S100.

Isolated intracranial Rosai-Dorfman disease mimicking meningioma in a child: a case report and review of the literature

“…Neurological involvement is rare and occurs in less than 5% of RDD patients [2]. Only 80 cases of RDD with CNS involvement have been reported in the literature [3].…”

“…The typical imaging appearance of intracranial RDD is a dural-based enhancing mass mimicking meningioma [2,3]. Although it usually presents as a solitary lesion, multiple intracranial masses have also been reported [2].…”

“…Although extranodal involvement is known to occur, isolated intracranial RDD is very rare. Out of 80 reported intracranial RDD cases, only 5 were in children [3][4][5][6][7][8].…”

Rosai Dorfman disease: Case with extensive dural involvement and cerebrospinal fluid pleocytosis

“…Also noted was extension of the intracranial disease to bilateral orbits and internal acoustic meatus resulting in both vision and hearing loss. Radiologically, meningioma is an important differential diagnosis for dural based Rosai Dorfman disease [12,13]. Other conditions with similar imaging features include tuberculoma and sarcoidosis.…”

Intracranial Rosai-Dorfman Disease: pathophysiology, diagnosis and treatment

“…On T1-weighted imaging with gadolinium contrast dural tail sign is often present [42] . RDD lesions are iso-or low hyperintense on T2-weighted MRI [10,40,[42][43][44][45][46] . The macrophages producing free radicals during phagocytosis might be the reason of presence T2weighted areas with low intensity [47] .…”