Skip to main content

Main menu

  • Home
  • Content
    • Current issue
    • Past issues
    • Early releases
    • Collections
    • Sections
    • Blog
    • Infographics & illustrations
    • Podcasts
    • COVID-19 articles
    • Obituary notices
  • Authors & Reviewers
    • Overview for authors
    • Submission guidelines
    • Submit a manuscript
    • Forms
    • Editorial process
    • Editorial policies
    • Peer review process
    • Publication fees
    • Reprint requests
    • Open access
    • Patient engagement
  • Physicians & Subscribers
    • Benefits for Canadian physicians
    • CPD Credits for CMA Members
    • Subscribe to CMAJ Print
    • Subscription prices
    • Obituary notices
  • Alerts
    • Email alerts
    • RSS
  • JAMC
    • À propos
    • Numéro en cours
    • Archives
    • Sections
    • Abonnement
    • Alertes
    • Trousse média 2023
    • Avis de décès
  • CMAJ JOURNALS
    • CMAJ Open
    • CJS
    • JAMC
    • JPN

User menu

Search

  • Advanced search
CMAJ
  • CMAJ JOURNALS
    • CMAJ Open
    • CJS
    • JAMC
    • JPN
CMAJ

Advanced Search

  • Home
  • Content
    • Current issue
    • Past issues
    • Early releases
    • Collections
    • Sections
    • Blog
    • Infographics & illustrations
    • Podcasts
    • COVID-19 articles
    • Obituary notices
  • Authors & Reviewers
    • Overview for authors
    • Submission guidelines
    • Submit a manuscript
    • Forms
    • Editorial process
    • Editorial policies
    • Peer review process
    • Publication fees
    • Reprint requests
    • Open access
    • Patient engagement
  • Physicians & Subscribers
    • Benefits for Canadian physicians
    • CPD Credits for CMA Members
    • Subscribe to CMAJ Print
    • Subscription prices
    • Obituary notices
  • Alerts
    • Email alerts
    • RSS
  • JAMC
    • À propos
    • Numéro en cours
    • Archives
    • Sections
    • Abonnement
    • Alertes
    • Trousse média 2023
    • Avis de décès
  • Visit CMAJ on Facebook
  • Follow CMAJ on Twitter
  • Follow CMAJ on Pinterest
  • Follow CMAJ on Youtube
  • Follow CMAJ on Instagram
Practice

Yellow swelling around the eyes: periocular lymphoma

H.J. Bovenschen and E.J.F.M de Kruijf
CMAJ June 15, 2010 182 (9) E378; DOI: https://doi.org/10.1503/cmaj.090955
H.J. Bovenschen
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
E.J.F.M de Kruijf
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • Article
  • Figures & Tables
  • Responses
  • Metrics
  • PDF
Loading

A 49-year-old woman presented with gradual onset of swelling and yellowish discoloration around both eyes (Figure 1). Nine years earlier, she had been assessed for persistent lymphocytosis, which was thought to represent stage 0 chronic lymphocytic leukemia, for which no treatment was needed. During follow-up, her peripheral blood cell counts remained stable and no palpable lymphadenopathy had developed. At this presentation, the patient was found to have swollen intranasal mucosa and periocular yellowish subcutaneous nodules, and no erythema or scaling. She had no palpable lymphadenopathy or other abnormalities.

Figure
  • Download figure
  • Open in new tab
  • Download powerpoint

Figure 1: (A) Front view of a 49-year-old patient before treatment, showing periorbital yellowish discoloration with indurated subcutaneous nodules. (B) Front view after combination of chemotherapy and immunotherapy (background colour adjusted for ease of comparison). (C) Side view before treatment. (D) Side view after treatment.

Biopsy of the subcutis of the right upper eyelid showed normal epidermis but there was a massive dermal and subcutaneous infiltrate of B-cells (CD20+, CD79a+, Bcl-2+, κ-chain+) and hemosiderin-loaded macrophages. CD23 and CD10 were negative. Cyclin-D1 was negative, and proliferation marker Ki-67 (MIB-1) was sparingly positive. T-cell markers (CD2, CD3, CD5 and CD7) were negative. This histopathologic and immunophenotypical examination gave results consistent with an extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue.

The colour of the skin lesions was explained by the accumulation of hemosiderin-laden macrophages. There was no evidence of bleeding on examination or in the patient’s history. Results of a bone marrow biopsy were consistent with marginal zone B-cell lymphoma, showing extensive infiltration of B cells that were immunophenotypically identical to the cutaneous B-cell infiltrate. Treatment with a combination of chemotherapy and immunotherapy (cyclophosphamide, vincristine, prednisone and rituximab) resulted in complete remission and resolution of the skin lesions (Figure 1).

Extranodal marginal zone B-cell lymphomas account for up to 80% of periocular lymphomas. The tumours originate in mucosa-associated lymphoid tissue in the ocular adnexa. Most ocular adnexal lymphomas are primary tumours but up to one-third are secondary tumours that develop in patients with disseminated lymphoma. 1 Localized periocular lymphoma can be treated with radiotherapy alone 2 but systemic involvement requires chemotherapy, alone or in combination with immunotherapy. With this approach, the disease can be controlled for years; however, the persistent nature of this lymphoma can often lead to relapse. 3

Footnotes

  • Previously published at www.cmaj.ca

    This article has been peer reviewed.

    Competing interests: None declared.

REFERENCES

  1. 1.↵
    Stefanovic A, Lossos I. Extranodal marginal zone lymphoma of the ocular adnexa. Blood 2009;114:501–10.
    OpenUrlAbstract/FREE Full Text
  2. 2.↵
    Decaudin D, Dendale R, Lumbroso-Le Rouic L. Treatment of mucosa-associated lymphoid tissue-type ocular adnexal lymphoma. Anticancer Drugs 2008;19:673–80.
    OpenUrlCrossRefPubMed
  3. 3.↵
    Suh C, Huh J, Roh JL. Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue arising in the extracranial head and neck region: a high rate of dissemination and disease recurrence. Oral Oncol 2008;44:949–55.
    OpenUrlCrossRefPubMed
PreviousNext
Back to top

In this issue

Canadian Medical Association Journal: 182 (9)
CMAJ
Vol. 182, Issue 9
15 Jun 2010
  • Table of Contents
  • Index by author

Article tools

Respond to this article
Print
Download PDF
Article Alerts
To sign up for email alerts or to access your current email alerts, enter your email address below:
Email Article

Thank you for your interest in spreading the word on CMAJ.

NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.

Enter multiple addresses on separate lines or separate them with commas.
Yellow swelling around the eyes: periocular lymphoma
(Your Name) has sent you a message from CMAJ
(Your Name) thought you would like to see the CMAJ web site.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.
Citation Tools
Yellow swelling around the eyes: periocular lymphoma
H.J. Bovenschen, E.J.F.M de Kruijf
CMAJ Jun 2010, 182 (9) E378; DOI: 10.1503/cmaj.090955

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero
‍ Request Permissions
Share
Yellow swelling around the eyes: periocular lymphoma
H.J. Bovenschen, E.J.F.M de Kruijf
CMAJ Jun 2010, 182 (9) E378; DOI: 10.1503/cmaj.090955
Digg logo Reddit logo Twitter logo Facebook logo Google logo Mendeley logo
  • Tweet Widget
  • Facebook Like

Jump to section

  • Article
    • Footnotes
    • REFERENCES
  • Figures & Tables
  • Responses
  • Metrics
  • PDF

Related Articles

  • No related articles found.
  • PubMed
  • Google Scholar

Cited By...

  • No citing articles found.
  • Google Scholar

More in this TOC Section

  • Vulvar condyloma lata as a first presentation of syphilis
  • Pregnancy in people living with obesity
  • Bedbugs
Show more Practice

Similar Articles

Collections

  • Article Types
    • Clinical Images
  • Topics
    • Dermatology

 

View Latest Classified Ads

Content

  • Current issue
  • Past issues
  • Collections
  • Sections
  • Blog
  • Podcasts
  • Alerts
  • RSS
  • Early releases

Information for

  • Advertisers
  • Authors
  • Reviewers
  • CMA Members
  • CPD credits
  • Media
  • Reprint requests
  • Subscribers

About

  • General Information
  • Journal staff
  • Editorial Board
  • Advisory Panels
  • Governance Council
  • Journal Oversight
  • Careers
  • Contact
  • Copyright and Permissions
CMAJ Group

Copyright 2023, CMA Impact Inc. or its licensors. All rights reserved. ISSN 1488-2329 (e) 0820-3946 (p)

All editorial matter in CMAJ represents the opinions of the authors and not necessarily those of the Canadian Medical Association or its subsidiaries.

To receive any of these resources in an accessible format, please contact us at CMAJ Group, 500-1410 Blair Towers Place, Ottawa ON, K1J 9B9; p: 1-888-855-2555; e: cmajgroup@cmaj.ca

CMA Civility, Accessibility, Privacy

 

Powered by HighWire