A case of orbital MALT (mucous associated lymphoid tissue) lymphoma is reported for its rarity. It presented as a large tumor obscuring the whole eye with loss of vision, without any signs of dissemination and remained free of recurrence or metastasis 12 months after undergoing simple surgical excision

Chhablani Kumar, Dr. Jay

Kamath Jagannath, Dr. Sumana

Kamath Mulky, Dr. Manjunath

Pai Gopalakrishna, Dr. Shobha

Rau Rangan, Dr. Aarthi

English

Shobha G Pai

Sumana J Kamath

Manjunath M Kamath

Aarthi R Rau

Jay Kumar Chhablani

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Orbital MALT Lymphoma: A Case Report

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Published Quarter ly
Mangalore, S outh I ndia
I S S N 0972-5997
Volume 3;  I s sue 2;  Apr i l  -  June 2004
Case Report
Orbital MALT Lymphoma: A Case Report
Shobha G Pai, MS, DOMS, Associate Professor*
Sumana J Kamath, MS, Associate Professor*
Manjunath M Kamath, MS, DOMS, Associate Professor*
Aarthi R Rau, MD, Assistant Professor,**
Jay Kumar Chhablani, MBBS, Postgraduate Student*
*Dept. of Ophthalmology, Kasturba Medical College Hospital, Attavar, MANGALORE
**Dept. of Pathology, Kasturba Medical College, MANGALORE
Address for Correspondence:
Dr. Shobha G Pai, MS, DOMS,
Associate Professor, Dept. of Ophthalmology,
Kasturba Medical College Hospital, Attavar,
MANGALORE, INDIA. Pin: 575001
E-mail: drsgpai@rediff.com
Citation: Pai SG, Kamath SJ, Kamath MM, Rau AR, Chhablani JK. Orbital MALT
Lymphoma: A Case Report. Online J Health Allied Scs. 2004;2:3
URL: http://www.ojhas.org/issue10/2004-2-3.htm
Open Access Archive: http://cogprints.ecs.soton.ac.uk/view/subjects/OJHAS.html
Abstract:
A case of orbital MALT (mucous associated lymphoid tissue) lymphoma is reported for its rarity. It
presented as a large tumor obscuring the whole eye with loss of vision, without any signs of
dissemination and remained free of recurrence or metastasis 12 months after undergoing simple
surgical excision.
Key Words: Orbital MALT Lymphoma
Case Report
A 52 years old man presented with a slow
growing mass arising from the right orbit,
(Fig. 1,2) encroaching upon the eyeball
over the last 4 months resulting in loss of
vision. He also had blood stained
discharge from the right eye.
Figure 1: Mass arising from the right orbit
The patient had undergone a combined
surgery (for cataract and secondary open
angle glaucoma) in the same eye 8 years
ago, as per his previous records.
Figure 2: Mass arising from the right orbit
Examination revealed a right orbital mass
filling the interpalpebral area, firm with
irregular surface, non-tender, non-
pulsatile, with pigments present on the
surface of the mass. Right eyeball was not
visible. The other eye was normal on
examination. There were no enlarged
lymph nodes and no significant findings
on systemic examination. Routine blood
investigations were within normal limits.
B scan showed clear orbital cavity with
hyper-echoic shadow surrounding it. An
excisional biopsy was performed.
On gross appearance the mass appeared
white and fleshy. Cut section was white,
homogenous, surrounding the whole of the
anterior part of the eye. Tumour was
extending into the sub-conjunctival space
and anterior chamber.(Figs.3,4,5)
Fig. 3: Eyeball surrounded by the tumor
Histopathological examination revealed
diffusely arranged, densely cellular
lymphoid cells in a sparsely cellular
stroma. Tumour cells were medium sized
with irregular nuclei, clumped chromatin,
inconspicuous nucleoli and abundant pale
cytoplasm. Admixed with these were small
lymphoid cells with oval nuclei and scanty
cytoplasm. A few immunoblasts and
plasmacytoid cells were also seen.
Figure 4: Posterior view of the tumor
Figure 5: Enucleated eyeball with cut-
section of the tumor
Figure 6: Histopathology of orbital
MALT Lymphoma
On the basis of these findings, a diagnosis
of MALT Lymphoma was made.
Patient is under follow-up and has
remained free of recurrence or systemic
dissemination after 6 months of surgery.
Discussion:
MALT lymphoma is part of a group of low-
grade B cell lymphomas presenting in the
gut, lungs, thyroid, salivary gland and
conjunctiva. It may arise in lacrimal gland
affected by Sjogren's syndrome.(1) Clinical
manifestations of MALT Lymphoma vary
from mild photophobia to mild redness
and irritation and orange pink or pale
masses arising from either the upper or
lower fornices. Typical appearance of
MALT Lymphoma shows centrocyte like
cells infiltrating around lymphoid follicles
and intraepithelial B cells.(2)
MALT Lymphoma differs from other
forms of B cell extra-nodal non-Hodgkins
lymphoma in that it is not as aggressive in
behaviour and remains localized to
mucosal surfaces rather than
disseminating systemically. This appears
to account for the better prognosis
observed with MALT Lymphoma
compared to that with non-MALT
Lymphoma arising at the same site.
Petrella et al (3) had also described a case
of unilateral conjunctival lymphoma
(MALT type) which showed no signs of
dissemination at presentation and
remained free of recurrence or metastasis
12 months after undergoing simple
surgical excision.
There has been some speculation on why
orbital lymphomas tend to be confined to
the orbit for long periods before
metastasis. One possibility is that they
are initially reactive lesions and only
subsequently become truly neoplastic. The
absence of lymphatic drainage channels
might also impede dissemination.(4)
The present case had a history of ocular
surgeries 8 years prior to the development
of the MALT Lymphoma. We ask whether
there could be any relationship between
the two?
References
1. Hardman-Lea S, Kerr–Muir M,
Wotherspoon AC et al. Mucosal-
Associated Lymphoid Tissue
Lymphoma of the conjunctiva.
Arch Ophthalmol. 1994;112:1207-
1212.
2. Specht CS, Laver NM. Benign and
malignant lymphoid tumors,
leukemia and histiocytic lesions.
In Albert DM, Jakobiec FA
(Editors). Principles and Practice
of Ophthalmology. 2nd edition.
W.B. Saunders Co. Philadelphia,
2000; p 5150
3. Petrella T, Bron A, Foulet A et al.
Report of a primary lymphoma of
the conjunctiva. A lymphoma of
MALT origin? Pathol Res Pract.
1991;187(1):78-84.
4. Garner A. Orbital
lymphoproliferative disorders. Br
J Ophthal. 1992;76:47-48


Benign and malignant lymphoid tumors, leukemia and histiocytic lesions.

Foulet A et al. Report of a primary lymphoma of the conjunctiva. A lymphoma of MALT origin? Pathol Res Pract.

MucosalAssociated Lymphoid Tissue Lymphoma of the conjunctiva. Arch Ophthalmol.

Orbital lymphoproliferative disorders.

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Orbital MALT Lymphoma: A Case Report

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