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Isolated MALT-lymphoma of the Conjunctiva with Lymphoplasmocytic Differentiation in Childhood

1Bestelmeyer S., 2Claviez A., 3Frahm S. O., 1Reichelt J. A.,
1Christian-Albrechts-Universität Kiel, Klinik für Ophthalmologie (Kiel)
2Christian-Albrechts-Universität Kiel, Klinik für Algemeine Pädiatrie (Kiel)
3Christian-Albrechts-Universität Kiel, Institut für Pathologie (Kiel)

Background: We report on a case of primary extranodal isolated MALT-lymphoma of the conjunctiva in infancy (low-grade non-Hodgkin lymphoma). Physiologically, MALT (mucosa associated lymphoid tissue) is part of the mucosa of the terminal ileum. The occurrence of MALT-lymphoma in tissues which primarily do not express MALT is still unexplained. The conjunctiva of the eye is an example for this condition.
Case report: A ten-year-old girl presented a tumor of the nasal conjunctiva of the left eye existing for one month. Examination demonstrated a painless, smooth, cystic lesion of pale reddish colour. The caruncle was not affected. Visual acuity was 20/20. The anterior segment of the right eye as well as the fundus in both eyes showed regular findings. The tumor was excised. Histology and molecular genetics revealed the diagnosis of MALT-lymphoma with lymphoplasmocytic differentiation, respectively immunocytoma. During follow-up no sign for relapse was noted, the conjunctiva appeared bland. Systemic affection could be excluded. Due to the young age of the patient we restrained from radiotherapy and preferred adjuvant cryotherapy of the conjunctiva.
Discussion: Lymphoplasmocytic immunocytomas typically occur in elder patients in form of systemic disease (Waldenström´s disease). However, these cases do not belong to the entity of MALT-lymphoma and the prognosis is severe. In contrast, in infancy systemic immunocytomas are rare: to our knowledge isolated conjunctival occurrence has not been described so far. Isolated conjunctival MALT-lymphoma in elder people takes a benign course, but does not reveal the differentiation of immunocytoma. Therefore, the question arises if the lymphoma described above may possibly be defined as an own entity.