Rosai–Dorman Disease Causing Cervical Myelopathy

Abstract

Recognizing clinical and laboratory features of this disease may permit earlier diagnosis and limit or avoid surgical intervention in some cases.

Isolated intradural Rosai-Dorfman disease of the spine: report of two cases

“…In most cases, as in the present patients, the signals of the lesion ranged from iso-to hypointensity on T1 weighted images, and iso-to hyperintensity on T2 weighted images, and exhibits an intense and uniform enhancement after gadolinium administration [1,3,[6][7][8][10][11][12][13]. MR images are usually indistinguishable from other more common lesions in the extradural spinal space such as meningioma [7,9,14], lymphoma, and sarcoma [6].…”

“…Typical clinical presentation involves massive painless cervical lymphadenopathy, but extranodal disease is apparent in about 40 % of cases, most commonly involving the orbit, skin, respiratory tract, bones, kidneys, heart, and head and neck region [1,2]. In less than 5 % of the cases, the central nervous system (CNS) is involved, with three fourths in brain and one fourth in spinal cord [3]. We describe two cases of isolated intradural RDD of the spine, and briefly review the literature.…”

Crush Cytology of Rosai-Dorfman Disease of the Central Nervous System

“…CNS RDD mimics meningioma in both clinical and radiologic features; however, a low-signal intensity on T 2 -weighted MRI, reported in several cases 7,13,18,19 and observed in 1 of the current 2 cases, is a very unlikely characteristic of meningiomas except for the fibrous variant. 19 After total or partial resection of the CNS lesions, most patients were either asymptomatic or showed an improvement.…”

Rosai-Dorfman disease with spinal cord compression: a diagnostic challenge

“…RDD can show different characteristics, mimicking other more common conditions such as metastasis, lymphoma, or infection. They are more frequently hypo-or iso-intense on T1-and T2-weighted MRI and enhance intensely and uniformly with contrast [1,7,16,17,39]. Histopathology is decisive in allowing a definitive diagnosis, showing nodular cellular infiltrates composed of histiocytes intermixed with plasma cells and lymphocytes in a background of increased collagen and/or reticulin fibres [3,4].…”

“…It is characterized by an idiopathic polyclonal hypergammaglobulinemia and a reactive process with pathognomonic histological and immunohistochemical features. [4][5][6][7][8][9][10][11][12][13][14] It commonly involves the cervical lymph nodes and less frequently extranodal sites, including, rarely, the central nervous system, mainly intracranially; spinal involvement is unusual [1][2][3][4][13][14][15][16]. Isolated CNS RDD without other body involvement is hardly described [4,13].…”