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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 2  |  Issue : 2  |  Page : 81-82

Lymphoma presenting as subcutaneous periorbital nodules


1 Department of Ophthalmology, SUNY Downstate Medical Center, Brooklyn, NY, USA
2 Department of Pathology, The New York Eye and Ear Infirmary of Mount Sinai, New York, NY, USA

Date of Web Publication10-Jul-2018

Correspondence Address:
Roman Shinder
Department of Ophthalmology, SUNY Downstate Medical Center, 541 Clarkson Ave, E Bldg, 8th Floor, Suite C, Brooklyn, NY 11203
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/CDR.CDR_44_17

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  Abstract 

A 67-year-old female presented with a several month history of painless enlarging right periorbital growths. Examination showed multiple nontender subcutaneous nodules superior to the right eyebrow. Magnetic resonance imaging and incisional biopsy confirmed subcutaneous marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT). Subcutaneous involvement sparing the epidermis is a rare presentation in patients with prior noncutaneous marginal zone lymphomas. Recent studies have demonstrated that subcutaneous dissemination is associated with MALT lymphoma of the breast and ocular adnexa. Systemic workup is therefore critical in patients who present with subcutaneous MALT lymphoma. Radiation therapy has proven to be effective in cutaneous marginal zone B-cell lymphomas.

Keywords: Lymphoma, mucosa-associated lymphoid tissue, subcutaneous


How to cite this article:
DeMaria LN, Iacob CE, Shinder R. Lymphoma presenting as subcutaneous periorbital nodules. Clin Dermatol Rev 2018;2:81-2

How to cite this URL:
DeMaria LN, Iacob CE, Shinder R. Lymphoma presenting as subcutaneous periorbital nodules. Clin Dermatol Rev [serial online] 2018 [cited 2018 Nov 18];2:81-2. Available from: http://www.cdriadvlkn.org/text.asp?2018/2/2/81/236341


  Introduction Top


Marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) has a propensity for extranodal organs due to interactions of mucosal structures such as α4 β7 integrins with endothelial venules.[1] Primary cutaneous marginal zone B-cell lymphoma (PCMZL) is a low-grade MALT lymphoma that originates in the skin without extracutaneous disease and represents 10% of cutaneous lymphomas.[2] Most present as multifocal lesions on the trunk and extremities but uncommonly (~10%) in the head and neck region, which is a contrast from cutaneous follicle center lymphomas.[3] An association with Borrelia burgdorferi infection has been investigated, with antibiotic use having a mixed effect on the regressions of lesions.[4]


  Case Report Top


A 67-year-old female with a past history of only well-controlled hypertension presented with a several month history of painless enlarging right periorbital growths. She denied any past similar lesions or any history of cancer. Examination showed multiple nontender subcutaneous nodules superior to the right eyebrow [Figure 1]a. Magnetic resonance imaging confirmed the superficial location of the lesions [Figure 1]b. Since the differential diagnosis was broad including both benign and malignant entities; a biopsy was suggested. Incisional biopsy revealed a dense infiltration of the dermis by monotonous small lymphocytes [Figure 1]c that stained with CD20 and CD43 [Figure 1]d confirming a diagnosis of extranodal marginal zone B-cell lymphoma of MALT. Systemic workup with full body positron emission testing was negative, and radiation therapy is currently planned.
Figure 1: (a) Clinical photograph demonstrating multiple subcutaneous nodules superior to the right eyebrow. (b) Axial T-1 weighted orbital magnetic resonance imaging confirming subcutaneous location of lesions (green arrow). (c) Histopathology of biopsy specimen demonstrating small monotonous lymphocytes infiltrating the dermis (H and E, ×400) with strong staining for CD43 (d), (×400)

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  Discussion Top


Subcutaneous involvement sparing the epidermis is a rare presentation in patients with prior cutaneous marginal zone lymphomas. Recent studies have demonstrated that subcutaneous dissemination, particularly in the head-and-neck region, is associated with MALT lymphoma of the breast and ocular adnexa.[1],[5] Systemic workup is, therefore, critical in patients who present with subcutaneous MALT lymphoma. The proposed hypothesis is a specific homing pattern for these ectodermal-derived organ systems. The possible association of hepatitis C infection with subcutaneous MALT also suggests a chronic antigenic stimulation component to its development.[6] Patients with subcutaneous dissemination usually have similar prognoses compared to those with extranodal lesions.[1]

PCMZLs are usually treated with radiotherapy, excision, and possible chemotherapy if multifocal. The dosage of radiation is still unknown, with success occurring at 3000-rad doses. Chemotherapy with agents such as chlorambucil has proven safe and effective, but multiagent use does not appear superior to preventing cutaneous relapses.[3]


  Conclusion Top


Subcutaneous MALT lymphoma can rarely present as painless periorbital nodules and clinicians should include this entity in their differential diagnosis to allow for timely diagnosis and management.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Jonak C, Troch M, Kiesewetter B, Lukas J, Müllauer L, Jäger U, et al. Subcutaneous dissemination pattern in extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue lymphoma. Haematologica 2012;97:766-70.  Back to cited text no. 1
    
2.
Lima M. Cutaneous primary B-cell lymphomas: From diagnosis to treatment. An Bras Dermatol 2015;90:687-706.  Back to cited text no. 2
    
3.
Hoefnagel JJ, Vermeer MH, Jansen PM, Heule F, van Voorst Vader PC, Sanders CJ, et al. Primary cutaneous marginal zone B-cell lymphoma: Clinical and therapeutic features in 50 cases. Arch Dermatol 2005;141:1139-45.  Back to cited text no. 3
    
4.
Cerroni L, Zöchling N, Pütz B, Kerl H. Infection by borrelia burgdorferi and cutaneous B-cell lymphoma. J Cutan Pathol 1997;24:457-61.  Back to cited text no. 4
    
5.
Gerami P, Wickless SC, Querfeld C, Rosen ST, Kuzel TM, Guitart J, et al. Cutaneous involvement with marginal zone lymphoma. J Am Acad Dermatol 2010;63:142-5.  Back to cited text no. 5
    
6.
Paulli M, Arcaini L, Lucioni M, Boveri E, Capello D, Passamonti F, et al. Subcutaneous 'lipoma-like' B-cell lymphoma associated with HCV infection: A new presentation of primary extranodal marginal zone B-cell lymphoma of MALT. Ann Oncol 2010;21:1189-95.  Back to cited text no. 6
    


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Abstract
Introduction
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Discussion
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