Lymphoma & related disorders
Splenic hematogenous neoplasms
Histiocytic lymphoma / sarcoma

Topic Completed: 1 March 2013

Minor changes: 1 April 2020

Copyright: 2003-2020,, Inc.

PubMed Search: Histiocytic lymphoma[TIAB] OR histiocytic sarcoma[TIAB] spleen

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Page views in 2020 to date: 601
Cite this page: Mansouri J. Histiocytic lymphoma / sarcoma. website. Accessed August 6th, 2020.
Definition / general
  • Rare to initially involve the spleen; usually arises in GI tract or skin
  • Must exclude diffuse large B cell or T cell lymphomas, anaplastic large cell lymphomas, acute monocytic leukemia
  • Called malignant histiocytosis if disseminated
  • May arise in mediastinal germ cell tumors
Case reports
Gross images

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Splenic histiocytic sarcoma

Microscopic (histologic) description
  • Diffuse sinusoidal infiltration of red pulp by neoplastic histiocytes with extension into cords
  • May be multinucleated giant atypical cells
  • Foci of spindled morphology may be seen
  • Often erythrophagocytosis; inconspicuous white pulp
  • No nodules or tumor masses
Microscopic (histologic) images

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Various images

CD68+, CD163+, lysozyme+

Secondary bone marrow involvement

Electron microscopy description
  • Dilated sinuses lined by large atypical histiocytic cells with abundant lysosomes and prominent erythrophagocytosis
  • No Birbeck granules
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