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OJHAS: Vol. 3, Issue
2: (2004 Apr-Jun) |
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| Orbital MALT Lymphoma: A Case Report |
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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 |
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Address For Correspondence |
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Dr. Shobha G Pai,
Associate Professor, Dept. of Ophthalmology,
Kasturba Medical College Hospital, Attavar,
MANGALORE - 575001, INDIA.
E-mail: drsgpai@rediff.com
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| Ramanakumar
AV. Reviewing disease burden among rural Indian women.
Online J Health Allied Scs.2004;2:3 |
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Submitted: Jun 16,
2004; Revised: Jul 11, 2004; Re-revised: Aug 20, 2004; Accepted: Aug
20, 2004; Published: Aug 31,
2004 |
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| Abstract: |
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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
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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. 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.
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| Figure 1: Mass arising
from the right orbit |
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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.
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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) Figure 3: Eyeball
surrounded by the tumor |
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 Fig 5: Enucleated
eyeball with cut-section of the tumor |
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Figure 4: Posterior view
of the tumor |
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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 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.
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?
- Hardman-Lea S, Kerr–Muir M, Wotherspoon
AC et al. Mucosal-Associated Lymphoid Tissue Lymphoma of the conjunctiva. Arch
Ophthalmol. 1994;112:1207-1212.
- 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
- 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.
- Garner A. Orbital lymphoproliferative
disorders. Br J Ophthal. 1992;76:47-48
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