Primary histiocytic sarcoma of the central nervous system

Hoonsub So, Sun A. Kim, Dok Hyun Yoon, Shin Kwang Khang, Jihye Hwang, Chong Hyun Suh, Cheolwon Suh

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19 Scopus citations


Histiocytic sarcoma is a type of lymphoma that rarely involves the central nervous system (CNS). Its rarity can easily lead to a misdiagnosis. We describe a patient with primary CNS histocytic sarcoma involving the cerebral hemisphere and spinal cord, who had been initially misdiagnosed as demyelinating disease. Two biopsies were necessary before a correct diagnosis was made. A histologic examination showed bizarre shaped histiocytes with larger nuclei and nuclear atypia. The cells were positive for CD68, CD163, and S-100 protein. As a resection was not feasible due to multifocality, he was treated with highdose methotrexate, but showed no response. As a result, he was switched to high dose cytarabine; but again, showed no response. The patient died 2 months from the start of chemotherapy and 8 months from the onset of symptoms. Since few patients with this condition have been described and histopathology is difficult to diagnose, suspicion of the disease is essential.

Original languageEnglish (US)
Pages (from-to)322-328
Number of pages7
JournalCancer Research and Treatment
Issue number2
StatePublished - 2015

Bibliographical note

Publisher Copyright:
© 2015 by the Korean Cancer Association.


  • Central nervous system
  • Cytarabine
  • Histiocytic sarcoma
  • Methotrexate


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