Abstract

Objective To provide insight into the clinical manifestation, histopathology characteristics, diagnostic and therapeutic methods of primary histiocytic sarcoma (HS) of central nervous system. Methods The clinical, auxiliary examination and pathological data of one patient with intracranial primary histiocytic sarcoma were presented, and relevant literature were reviewed. Results A 55-year-old female had an 2-month history of hypomnesia and unsteady gait. MRI scans showed multiple mass lesions in brain. Biopsies of the brain lesion showed the tumor cells had polymorphism, and abundant cytoplasm was eosinophilic or clear, with large, pleomorphic nuclei. Immunohistochemically, the tumor cells stained positively with CD45,CD68, CD163, Ki67 and Vimentin, but negatively with pan-cytokeratin, epithelial membrane antigen, CD3, CD20, CD79a, estrogen receptor, progesterone receptor, Melan-A and glial fibrillary acidic protein. Though she had received radiotherapy, the disease progressed and her consciousness deteriorated within 2 months. Conclusions HS should be considered if multiple or single intracranial unexplained lumps are found and the earliest pathological biopsy is required for early diagnosis. There are no effective therapeutic methods for HS and the prognosis is very poor. Key words: Histiocytic sarcoma; Central nervous system neoplasms; Diagnosis

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