JOURNAL OF MEDICAL AND SURGICAL RESEARCH - Vol. I, n 1 , June, 2014

Pages: 22-23
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Bilateral MALT (Mucosal Associated Lymphoid Tissue) Lymphoma of the Conjunctiva. A Rare Case Report

Author: N. Alami Drideb, H. Eddou, K. Doghmi, M. Mikdame

Category: JMSR Oncology

Abstract:

MALT (Mucosal Associated Lymphoid Tissue) lymphomas of the conjunctiva belong to the extranodal marginal zone B-cell lymphomas. This site, while standard, is uncommon. We present the observation of a 65 year's old man who presented a pink papular tumor developed on both lower eyelids. The CT-scan showed thickeni ng of soft tissues associated with bilateral eyelid and sub diaphragmatic nodes. A chemotherapy protocol based on eight courses of R-CHOP was established with a complete remission. Twelve months later, another bilateral eylid swelling reappeared. Treatment with local radiotherapy to 24 Gy was proposed with good outcome 28 months later.

Keywords: Conjunctiva, Lymphoma, MALT , Rituximab

Full Text:

MALT (Mucosal Associated Lymphoid Tissue) lymphomas of the conjunctiva belong to the extranodal marginal zone B-cell lymphomas. This site, while standard, is uncommon.

OBSERVATION

A 65 year's old man presented a pink papular tumor developed on both lower eyelids. He had a history of hypertension, dyslipidemia, carrying a pacemaker for atrial fibrillation, thyroid dysfunction, chronic smoking, and i ntolerance to carbohydrates. He noted an appearance of a pink swelli ng of both lower eyelids, gradually increasing in volume.

Figure 1 : pink swelling of both lower eyelids

There was no general clinical signs. A biopsy was performed and histological exami nation was suggesti ve of a B-cell lymphoma of MALT -type edge area. The orbital CT-scan showed thickening of soft tissues associated with bilateral eyelid. A CT scan of lung, abdomen and pelvis revealed sub diaphragmatic nodes.

Figure 2: thickening of soft tissues associated with bilateral eyelid.

A chemotherapy protocol based on eight courses of R-CHOP was established with a complete remission. Twelve months later, another bilateral eyelid swelli ng reappeared. A new staging was performed with bilateral eyelid thickening without nodes or other location. Treatment with local radiotherapy to 24 Gy was proposed with good outcome 28 months later.

DISCUSSION

The connective -orbital lymphoma remai ns a rare disease of the orbit and represents 5 to 10% of tumors of the orbit [1]. The frequency of extra nodal non hodgking lymphoma of low-grade MALT type from the orbital non-Hodgkin lymphoma has not often been studied because of its more recent identification [1, 2]. MALT lymphomas often recognize specific environmental factors responsible for their development and progression [2]. Recent data have shown the possible association between these lymphomas and Chlamydia psittaci [3]. In our case this infection was absent. All authors agree that this type of location have good prognosis [4]. Bilateral lesions may be a factor of recurrence. According to Gaucher and al [5], The MALT lymphoma has a better prognosis when isolated to the orbit, with a survival rate of about 75 % at 10 years after treatment. The therapeutic approach differs by immunohistochemical typing between forms of high-grade and low grade [6,7]. For Voegtle and al [6], radiotherapy may be used in cases of isolated MALT orbital typed lymphomas. If there is a second location, chemotherapy is recommended [D]. New treatments based on anti-CD20 monoclonal immunotherapy antibody (rituximab, Mabthera ® ) in combination with chemotherapy , improves the response rate , survival i n B-cell lymphomas and represents a major advance in the therapeutic management of these patients [8]. However, regular and long-term follow-up is required.

CONCLUSION

Lymphomas are the most common neoplasms of the orbit and periocular tissues. The most common histological type is the MALT lymphoma. Most often locali zed and with good prognosis, it is characteri zed by its curability, with little aggressive treatments, which does not exclude a regular long-term follow-up.

References:

1. Benabid. L et al. Les lymphomes malins non hodgkiniens conjonctivo-orbitaires. J Fr. Ophtalmol., 2005; 28, 10, 1058-1064.

2. Mannami T and al. Clinical, histopathological, and immunogenetic analysis of ocular adnexal lymphoproliferative disorders: characterization of malt lymphoma and reactive lymphoid hyperplasia. Mod Pathol 2001;14:641-9.

3. Francesca Collina and al. Chlamydia psittaci in ocular adnexa MALT lymphoma: a possible role in lymphomagenesis and a different geographical distribution. Infectious Agents and Cancer 2012, 7:8.

4. Raderer M and al. Importance of extensive staging in patients with mucosa-associated lymphoid tissue (MALT)-type lymphoma. Br J Cancer 2000;83:454-7.

5. Gaucher D and al. MALT-type B-cell lymphoma masquerading as scleritis or posterior uveitis. J Fr Ophtalmol. 2005 Jan;28(1)

6. Voegtle R and al. MALT lymphoma of the lacrimal apparatus (mucosa-associated lymphoid tissue). J Fr Ophtalmol. 1999 Oct;22(8):884-7.

7. Benabid L et al. Nouvelle approche thérapeutique du lymphome malin non hodgkinien orbitaire : à propos de deux cas traités par rituximab. J Fr Ophtalmol, 2005;28:769-71.

8. Davies A and al. Pharmacokinetics and safety of subcutaneous rituximab in follicular lymphoma (SABRINA): stage 1 analysis of a randomised phase 3 study. Lancet Oncol. 2014 Feb 7. pii: S1470-2045(14)70005-1