Marginal Zone Lymphoma of Eyelids: A Clinicopathological Entity. Histopathological and Molecular Analysis of 7 Cases
Antoine de Mascarel, Marie Parrens, Marie Beylot-Barry, Olivier Fitoussi, Frederic Bauduer, Sofia Marouan, Soumaya Rammeh, Bertille de Barbeyrac, Jean Philippe Merlio, Beatrice Vergier. University Hospital, Bordeaux, France, Metropolitan; Hospital of Bayonne, Bayonne, France, Metropolitan; University Hospital of Ibn Rochd, Casablanca, Morocco; Charles Nicolle Hospital, Tunis, Tunisia
Background: The MALT lymphomas are the most frequent lymphomas of the ocular adnexa. These lymphomas are localized above all in the conjunctiva but also in the eyelids.
Design: 29 ocular MALT lymphomas (OML) are recorded between 2000 and 2011. Out of these 29 cases, 7 were located in the eyelids. 30 cutaneous marginal zone lymphomas (CMZL) were studied in parallel. Formalin-fixed biopsies were analyzed by HE stain and immunohistochemistry with CD20, CD5, IgA, IgG, IgM. PCR for the detection of IgH gene rearrangement, MYD88 mutation and Chlamydia Psitacci (Cp) DNA was performed on DNA extracted from formalin-fixed fragments. FISH was performed on sections using the MALT1 DNA split signal probes.
Results: Out of these 7 eyelid lymphomas (EL), 3 were bilateral and one was unilateral but in the upper and lower eyelids. The 7 cases clinically presented only an eyelid edema. The 30 CMZL never localized to eyelid, showed papules but no edema. In 4 out of 7 EL, the first biopsy was negative as it was too superficial, the lymphomatous tumor being in the subcutis. Usually, in the CMZL group, the lymphoma was found located in the superficial dermis. The phenotype of 7 EL, 22 non EL OML and 30 CMZL was CD20+, CD5 -. IgM expression was detected in 4/7 EL, 11/22 OML and 0/30 CMZL. In the 29 OML, 26 displayed IgH rearrangement. In the 3 bilateral EL, the same clone was observed at both sites. The 30 CMZL were all monoclonal. No MYD88 mutation was found in the 7 EL. FISH analysis did not find any MALT1 gene breakpoint (0/18). In all 29 AOL, Chlamidia psittacci was not detected by PCR, even in the 3 bilateral tumors. In the 7 EL, bone marrow was negative but a similar IgH rearrangement was found in the 2 cases where the peripheral blood was studied. By comparaison, the peripheral blood of the 30 CMZL exibited a polyclonal profile. The 3 patients with bilateral EL received 4 cycles of rituximab and are currently in complete remission respectively 1, 3 and 6 years from the diagnosis.
Conclusions: The marginal zone lymphoma of eyelids seems to be a different clinicopathological entity from cutaneous marginal zone lymphomas because of: 1) constant edema often bilateral masking the lymphoma, 2) subcutaneous location requiring a deeper biopsy, compared to the other CMZL, 3) IgM expression which was not observed in CMZL.
Wednesday, March 6, 2013 9:30 AM
Poster Session V # 75, Wednesday Morning