Primary central nervous system histiocytic sarcoma

Abstract

Rationale:Primary central nervous system histiocytic sarcoma (PCNSHS) is a rare lymphohematopoietic tumor with a histiocytic cell origin. To our knowledge, only 28 cases have been published in English and 2 cases in Chinese.Patient concerns:A 49-year-old Asian female presented to the hospital with a 2 month history of hypomnesia, odynophagia, and gait disorder. Physical examination demonstrated decreased lower extremity muscle strength. The patient denied a history of malignancy.Diagnoses:Radiology demonstrated a lesion in parietal lobe with uniformenhancement. Histologic analysis showed pleomorphic tumor cells with a loose arrangement, effacing the normal brain tissue. The tumor cells exhibited abundant eosinophilic cytoplasm, highly atypical nuclei and predominant nucleoli. Immunohistochemistry revealed positive immunoreactivity for CD45, lysozyme, CD68, and CD163, and negative for pan-cytokeratin (CK), epithelial membrane antigen (EMA), glial fibrillary acidic protein (GFAP), CD3, CD20, CD1a, CD79a, CD138, oligodendrocyte transcription factor (olig2), CD15, melan-A, CD30, CD21, CD35, Human Melanoma Black-45 (HMB45), and anaplastic lymphoma kinase-1 (ALK-1). The diagnosis of PCNSHS was rendered.Interventions:The patient underwent complete surgical resection and adjuvant radiotherapy.Outcomes:Follow-up information shows the patient died 8 months following the initial diagnosis.Lessons:PCNSHS is extremely rare with an aggressive clinical course. Immunohistiochemistry is necessary to make this diagnosis and to exclude other primary intracranial and lymphohematopoietic tumors. Further research is required to improve the outcome of patients with PCNSHS.

3D bioprinted glioma microenvironment for glioma vascularization

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Histiocytic Sarcoma Involving Cervical Vertebra: A Case Report and Review of the Literature

“…It involves other sites much less frequently. The involvement of the central nervous system has been reported in 30 cases [ 11 ]. The primary involvement of the vertebral column by HS is extremely rare.…”

Clinical Reasoning: A 42-Year-Old Woman With Mysterious Monocytic Meningitis

“…As a subtype of primary CNS lymphoma, primary CNS histiocytic sarcoma (PCNSHS) has been documented only a few dozen times. 6,7 PCNSHS can present with one or more lesions in the cerebrum, cerebellum, meninges, and spinal cord. Clinical symptoms can range from headache to frank neurologic deficits.…”

“…6,8 Prognosis is generally poor with reported average survival of 19-29 months. 6 Treatment courses are nonstandardized and have included combinations of surgical resection, radiation, and chemotherapies, as well as interferons, without any successful treatments developed to date. 6,8 Study Funding…”