Eye
Orbit and optic nerve
Lymphoma / leukemia of orbit

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

Revised: 16 May 2018, last major update February 2014

Copyright: (c) 2004-2018, PathologyOutlines.com, Inc.

PubMed Search: Lymphoma [title] orbit

Cite this page: Pernick, N. Orbit and optic nerve: lymphoma / leukemia of orbit. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/eyeorbitlymphoma.html. Accessed July 21st, 2018.
Definition / general
  • Tumors composed of small lymphocytic proliferations confined to orbit are usually indolent and associated with long survival, even with minimal treatment
  • MALT lymphoma:
    • Uniform and monotonous proliferation of small lymphocytes infiltrating into orbital fat, blood vessels and nerves
    • May have plasmacytoid features with Dutcher bodies (intranuclear inclusions) and serum paraproteinemia
    • Light chain restriction
Radiology images

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Homogeneous tumor
that molds to ocular
structures causing
proptosis

Case reports
  • 5 year old girl with an angiocentric orbital lesion with an immature natural killer cell immunophenotype (Hum Pathol 2001;32:339)
  • Nasal-type NK/T cell lymphoma of the orbit with distant metastases (Hum Pathol 2003;34:290)
Clinical images

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Burkitt lymphoma - bilateral

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Lymphoplasmacytic lymphoma - bilateral

Microscopic (histologic) images

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Low grade tumor
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Well differentiated
lymphoma of orbit,
conjunctiva and cornea

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Irregularly shaped mass

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Uniform population of well differentiated lymphocytes

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Small cleaved cells

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Small population of
T cells (UCHL-1 /
CD45RO positive)


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Numerous CD20+ B cells

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Burkitt lymphoma
has lymphoblasts
and numerous
mitotic figures

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Granulocytic leukemia - Leder stain positive

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Lymphoplasmacytic lymphoma - lymphoid cells, some with plasmacytic differentiation

Positive stains
  • Monotypic immunoglobulins
Differential diagnosis
  • Lymphoid hyperplasia: polymorphic lymphocytes, vascular proliferation, prominent follicles with germinal centers