Abstract
BackgroundXanthoma 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.Case presentationWe 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.ConclusionsIntracranial XD without systemic involvement was extremely rare, which was supposed to be considered in differential diagnosis with other neoplasms of histiocytic origin or gliomas.
Highlights
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
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
XD is always a systemic disease [3], involvement of the central nervous system (CNS) is quite rare, and until now less than 100 cases in the literature written in English have been reported
Summary
We presented the case of intracranial XD without systemic involvement. MR imaging displayed multiple heterogeneous masses with intense enhancement. The tumor cells were absent of atypia and positive for histiocytic markers immunohistochemically. Such case was extremely rare, which was supposed to be considered in differentiation with other neoplasms of histiocytic origin or gliomas. All authors read and approved the final manuscript. Consent for publication Written informed consent was obtained from the patient for publication of this Case Report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal. Ethics approval and consent to participate A statement of ethics approval for this case report by the General Hospital of Beijing Military Command is available on request
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