Rosai–Dorfman disease (RDD) is a rare, benign, non-Langerhans cells histiocytosis with massive lymphadenopathy of uncertain aetiology. It is commonly characterized by massive, painless, non-tender, bilateral cervical lymphadenopathy. Extra-nodal involvement is usually seen in 50% of patients, with the brain being affected in only 5% of cases, usually as dural-based lesions. Clinical presentation is heterogeneous and strongly dependent on the localization of the lesions. Although the histopathological findings are essential for the final diagnosis, brain magnetic resonance imaging (MRI) currently represents the first-line strategy for the detection of the lesions across the central nervous system (CNS); moreover, it may provide additional elements for the differential diagnosis versus other more common lesions. We performed a case-based literature review to highlight possible aetiologic and pathogenetic theories of this disease, along with imaging features of RDD, with a particular focus on the MRI characteristics of the CNS involvement (CNS–RDD). Finally, we provided a novel insight on the current therapeutic approaches, either surgical or medical.

MR imaging of cerebral involvement of Rosai–Dorfman disease: a single-centre experience with review of the literature

Corridore A.;Tommasino E.;Bruno F.;Di Sibio A.;Splendiani A.;Di Cesare E.;Masciocchi C.
2020

Abstract

Rosai–Dorfman disease (RDD) is a rare, benign, non-Langerhans cells histiocytosis with massive lymphadenopathy of uncertain aetiology. It is commonly characterized by massive, painless, non-tender, bilateral cervical lymphadenopathy. Extra-nodal involvement is usually seen in 50% of patients, with the brain being affected in only 5% of cases, usually as dural-based lesions. Clinical presentation is heterogeneous and strongly dependent on the localization of the lesions. Although the histopathological findings are essential for the final diagnosis, brain magnetic resonance imaging (MRI) currently represents the first-line strategy for the detection of the lesions across the central nervous system (CNS); moreover, it may provide additional elements for the differential diagnosis versus other more common lesions. We performed a case-based literature review to highlight possible aetiologic and pathogenetic theories of this disease, along with imaging features of RDD, with a particular focus on the MRI characteristics of the CNS involvement (CNS–RDD). Finally, we provided a novel insight on the current therapeutic approaches, either surgical or medical.
File in questo prodotto:
File Dimensione Formato  
2020 rad med varrassi.pdf

solo utenti autorizzati

Tipologia: Documento in Post-print
Licenza: Dominio pubblico
Dimensione 1.41 MB
Formato Adobe PDF
1.41 MB Adobe PDF   Visualizza/Apri   Richiedi una copia

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/11697/147875
Citazioni
  • ???jsp.display-item.citation.pmc??? ND
  • Scopus 6
  • ???jsp.display-item.citation.isi??? 6
social impact