Primary Histiocytic Sarcoma of the Central Nervous System
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Histiocytic sarcoma is a type of lymphoma that rarely involves the central nervoussystem (CNS). Its rarity can easily lead to a misdiagnosis. We describe a patient withprimary CNS histocytic sarcoma involving the cerebral hemisphere and spinal cord,who had been initially misdiagnosed as demyelinating disease. Two biopsies werenecessary before a correct diagnosis was made. A histologic examination showedbizarre shaped histiocytes with larger nuclei and nuclear atypia. The cells werepositive for CD68, CD163, and S-100 protein. As a resection was not feasible due tomultifocality, 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 theonset of symptoms. Since few patients with this condition have been described andhistopathology is difficult to diagnose, suspicion of the disease is essential.
Abstract
Histiocytic sarcoma is a type of lymphoma that rarely involves the central nervoussystem (CNS). Its rarity can easily lead to a misdiagnosis. We describe a patient withprimary CNS histocytic sarcoma involving the cerebral hemisphere and spinal cord,who had been initially misdiagnosed as demyelinating disease. Two biopsies werenecessary before a correct diagnosis was made. A histologic examination showedbizarre shaped histiocytes with larger nuclei and nuclear atypia. The cells werepositive for CD68, CD163, and S-100 protein. As a resection was not feasible due tomultifocality, 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 theonset of symptoms. Since few patients with this condition have been described andhistopathology is difficult to diagnose, suspicion of the disease is essential.
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