“…Extranodal structures are affected in around 43% of patients and include central nervous system (CNS), skin, gastrointestinal and respiratory tract, heart, and other [1,4,10,17,18]. Only five to ten percent patients have RDD of the CNS; intraspinal lesions are less common than intracranial lesions [1,4,15,17]. Intracranial disease tends to occur in adult patients, slightly older than RDD in general [19].…”