Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy (SHML), is a rare benign idiopathic proliferative disease that involves phagocytic histiocytes.
The disease predominantly occurs in young adults with a mean age at presentation of 21 years. There may be a slight male predominance 7.
A vast majority of patients (~80%) present with painless massive cervical lymphadenopathy, with associated fever, malaise, weight loss and night-sweats 14.
In individuals with intracranial involvement, headaches and seizures have been described and in many, no systemic manifestations are present 8,14. Additional site-specific signs and symptoms may also be present (e.g. pituitary dysfunction).
The exact cause is unknown but disordered immune regulation and viral infections (e.g. EBV, HHV) are thought to be involved 9.
There is a very wide distribution. The condition can affect a multitude of organ systems which include:
- nodal involvement
- extranodal involvement (~30% 9)
- lungs (~2.5%): nodules and perilymphatic interstitial thickening 9
- nasal cavity
- orbit: ~7%
- intracranial and spinal disease: rare
Histologically, it is characterised by an attenuated infiltration of lymphoplasmacytic cells and histiocytes of varying size. The large, pale histiocytic cells contain what looks like engulfed lymphocytes ("emperipolesis") within their cellular borders.
- S100: positive
- CD11c: positive
- CD68: positive
- L1 antigen: positive
- CD1a: negative
Due to the wide disease spectrum, radiographic features can be variable. Nodal involvement is appreciated as lymphadenopathy on plain film and cross-sectional imaging.
- central nervous system 8
- hyperattenuating meningeal-based mass showing contrast enhancement
- parenchymal oedema surrounding the lesion may be present
- central nervous system 8,14
- T1: isointense to grey matter
- T2: hypointense to grey matter
- T1 C+ (Gd): homogeneous enhancement
- meningeal-based masses
Often shows increased uptake with Gallium scanning and increased metabolism with FDG-PET.
Treatment and prognosis
Rosai-Dorfman disease usually follows a benign and self-limiting course with treatment largely targeted at controlling local manifestations (surgical intervention) 6.
History and etymology
This condition was initially described by Juan Rosai and R F Dorfman in 1969 3.
- grading and histological variants
- grade I
- meningothelial meningioma
- fibrous meningioma
- microcystic meningioma
- psammomatous meningioma
- angiomatous meningioma
- secretory meningioma
- metaplastic meningioma
- lymphoplasmacyte-rich meningioma
- grade II
- grade III
- grade I
- imaging signs
- by location
- Simpson grade (of resection)
- grading and histological variants
- solitary fibrous tumour of the dura
- primary dural lymphoma
- Rosai-Dorfman disease
- EBV-associated smooth muscle tumour
- meningeal melanocytoma
- primary meningeal malignant melanoma
- Erdheim-Chester disease
- dural metastases
- hypertrophic pachymeningitis
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