Histiocytic lymphoma / sarcoma

CNS tumor
Lymphoma and other hematopoietic lesions
Histiocytic lymphoma / sarcoma

Author: Nat Pernick, M.D. (see Authors page)

Revised: 8 September 2017, last major update January 2006

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: Histiocytic lymphoma / sarcoma CNS

Table of Contents

Cite this page: Pernick, N. Histiocytic lymphoma / sarcoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/cnstumorhistiocyticsarcoma.html. Accessed May 21st, 2018.

Definition / general

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

Case reports

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

Microscopic (histologic) description

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

CD68, lysozyme, HAM56
Ki67 index > 20%
Variable S100

Negative stains

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

Electron microscopy description

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

Molecular / cytogenetics description

No t(2;5)
Polyclonal

Differential diagnosis

Inflammatory processes
Other lymphomas and histiocytic neoplasms (use immunohistochemistry)

Additional references

Am J Surg Pathol 2001;25:1372