title: Disseminated intracranial xanthoma disseminatum: a rare case report and review of literature.
abstract: BACKGROUND
  Xanthoma disseminatum (XD) is a rare benign histiocytic proliferating disease of non-Langerhans cell origin, which is clinically mainly characterized by cutaneous or mucous lesions. Although XD is acknowledged of one systematic disease, involvement of the central nervous system is quite rare.
  
  
  METHODS
  We presented one 34-year-old Chinese female with disseminated intracranial XD without cutaneous or oral mucosal papules and masses of the other organs. MR imaging displayed multiple heterogeneous masses with intense enhancement in the right frontal lobe, temporal lobe, corpus callosum, left cuneus, suprasellar region, and right cerebellum. Pathological examination showed a neoplastic lesion composed of plentiful epitheloid or spindle cells. The cell had pink cytoplasm of vacuolation and foam with deviated nucleus absent of atypia and mitosis. The histiocytic markers including CD163, CD11c, Mac387 and CD68 were positive, whereas S-100, CD1a, GFAP, CD21, CD23 and so on were negative immunohistochemically.
  
  
  CONCLUSIONS
  Intracranial XD without systemic involvement was extremely rare, which was supposed to be considered in differential diagnosis with other neoplasms of histiocytic origin or gliomas.
authors.affiliation: Department of Pathology, the General Hospital of Beijing Military Command of PLA, Nan Men Cang, No. 5, Dong Si, Dongcheng District, Beijing, 100700, People's Republic of China.
authors.forename: Guang-Zhi
authors.initials: GZ
authors.lastname: Yang
authors.affiliation: Department of Pathology, the General Hospital of Beijing Military Command of PLA, Nan Men Cang, No. 5, Dong Si, Dongcheng District, Beijing, 100700, People's Republic of China. li_jing971326@yahoo.com.
authors.forename: Jing
authors.initials: J
authors.lastname: Li
authors.affiliation: Department of Pathology, the General Hospital of Beijing Military Command of PLA, Nan Men Cang, No. 5, Dong Si, Dongcheng District, Beijing, 100700, People's Republic of China.
authors.forename: Lu-Ping
authors.initials: LP
authors.lastname: Wang
mesh_terms: D000328:Adult; D015415:Biomarkers / Q000032:analysis; D001921:Brain / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology; D001927:Brain Diseases / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology; D003937:Diagnosis, Differential; D005260:Female; D015616:Histiocytosis, Non-Langerhans-Cell / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging; D011237:Predictive Value of Tests
chemical_list: D015415:Biomarkers
keywords: Disseminated; Histiocytic neoplasm; Immunohistochemistry; MR; Xanthoma disseminatum