Rosai-Dorfman Disease Isolated to the Central Nervous System: A Report of 11 Cases

Abstract

Sinus histocytosis with massive lymphadenopathy, also known as Rosai-Dorfman Disease (RDD), is an idiopathic histiocytic proliferation affecting lymph nodes. Although extranodal involvement has been reported in diverse sites, central nervous system (CNS) manifestation, particularly in the absence of nodal disease is uncommon. We report 11 cases of RDD primary to the CNS without evidence of other sites of involvement. The cases included 7 males and 4 females ranging in age from 22 to 63 years (mean: 41 y). The patients presented with headaches, seizures, numbness, or paraplegia. Eight cases involved the cranial cavity and three cases, the spinal canal. Lesions were most often extra-axial and dura based. Only one presented in the CNS parenchyma. Histologically, the lesions consisted of variable numbers of pale-staining histocytes with emperipolesis often overshadowed by extensive lymphoplasmacytic infiltrates and fibrosis in the background. Special stains for organisms were negative. By immunohistochemical analysis, the characteristic histiocytes were positive for S100 protein and CD68 and negative for CD1a. Treatment consisted of surgical biopsy or excision. Follow-up, available for 10 cases with intervals ranging from 5 days to 42 months (mean: 15 mo), disclosed one patient dying of operative complications 5 days after biopsy and nine patients with no evidence of disease progression RDD should be considered in the differential diagnosis of inflammatory lesions of the CNS. Our study suggests that this entity may have been misdiagnosed in the past as plasma cell granuloma or inflammatory pseudotumor.

Spinal epidural Rosai–Dorfman disease preceding by relapsing uveitis: a case report with literature review

“…Regions in the brain include dura, venous sinus, intraparenchymal, intraventricle area. 4 There were 36 cases reported RDD involving eyes were highly accompanied with nasal sinuses involvement. 2 The common ocular manifestations were soft tissue of the orbit and eyelids.…”

Rosai Dorfman Disease in CNS 19 Cases from one Institute

“…Infection 5 has been suggested as an underlying cause, but a definitive agent has never been isolated.…”

“…Molecular studies using polymorphic regions of the human and rogen receptor locus have demonstrated that RDD is a polyclonal disorder 5,8 .…”

Co-occurrence of intracranial rosai-dorfman disease and langerhans histiocytosis of the skull: case report and review of literature

“…Occasional neutrophils and eosinophils may be seen and background fibrosis may be noted in extranodal disease. On immunohistochemical examination, histiocytes are immunoreactive for S-100 protein, HAM 56, α1 antitrypsin, α1 chymotrypsin, lysozyme, Mac 387 and Ki-1, but CD 1a and EMA are negative (2,10,16,20).…”