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General
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• Very rare
• Often fever, weight loss, hepatosplenomegaly and intestinal obstruction

Case reports
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• Histiocytic sarcoma involving the central nervous system (Am J Surg Pathol 2003;27:258)

Micro description
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• Large groups or sheets of large cells with abundant eosinophilic cytoplasm, pleomorphic vesicular nuclei, distinct nucleoli
• Also necrosis, neutrophils (variable abscesses) and chronic inflammatory cells, hemophagocytosis
• No infiltration of vascular walls

Positive stains
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• CD68, lysozyme, HAM56
• Ki-67 index > 20%
• Variable S100

Negative stains
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• Myeloperoxidase, dendritic markers, ALK1, GFAP, keratin
• CD1a, CD3, CD20, CD21/CD35, CD30

Electron microscopy description
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• Abundant cytoplasm with irregularly folded or multisegmented nuclei, lysosomes
• No Birbeck granules, no interdigitating cell processes, no cell junctions

Molecular / cytogenetics description
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• No t(2;5)
• Polyclonal

Differential diagnosis
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• Other lymphomas and histiocytic neoplasms (use immunohistochemistry)
• Inflammatory processes

Additional references
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• Am J Surg Pathol 2001;25:1372

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