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Lymphoproliferative Läsionen der okulären Adnexe

Differenzialdiagnostische Leitlinien

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Zusammenfassung

Die zweckmäßigsten Einteilungen zur Gliederung der Lymphome der okulären Adnexe (OAL) sind in der neuen WHO-Klassifikation der Tumoren des hämatopoetischen und lymphatischen Gewebes zusammengefasst. Dieser Einteilung folgend, stellt das extranodale Marginalzonen-B-Zell-Lymphom (EMZL) den am häufigsten vorkommenden Lymphomsubtyp der okulären Adnexe dar. Die Mehrzahl der Patienten mit einem OAL haben eine Erkrankung des Stadiums IE (E=extranodal), für das die perkutane Strahlentherapie die empfohlene initiale Behandlungsoption darstellt; eine disseminierte Erkrankung wird mit einer Chemotherapie behandelt. Trotz ihres klinisch in der Regel indolenten Verlaufs sind EMZL für ihre Rezidive in extranodalen Geweben bekannt. Langfristige Nachuntersuchungen in halbjährlichen Abständen sind notwendig. Die wesentlichen prognostischen Faktoren der OAL beinhalten das Alter der Patienten, die anatomische Lokalisation des Tumors, das Erkrankungsstadium bei Diagnose, die Höhe der Serumlaktatdehydrogenase bei Erstdiagnose, den Lymphomsubtyp und die Wachstumsfraktion der Tumorzellen.

Abstract

The ocular adnexal lymphomas represent the malignant end of the spectrum of lymphoproliferative lesions which occur in the conjunctiva, eyelids, lacrimal gland and orbit. The new “W.H.O. Classification of Tumours of Haemopoietic and Lymphoid Tissues” is the most suitable for subdividing the ocular adnexal lymphomas, whereby the extranodal marginal zone B-cell lymphoma (EMZL) represents the most common lymphoma subtype. Management of patients with ocular adnexal lymphomas includes a systemic medical examination to establish the clinical stage of the disease. Most patients have stage IE disease and current recommended therapy for this is radiotherapy, while disseminated disease is treated with chemotherapy. Despite usually demonstrating an indolent course, EMZLs are renowned for recurrence in extranodal sites, including other ocular adnexal sites. Furthermore, blastic transformation of EMZL with a corresponding aggressive clinical course has been described. Long-term follow-up with half-yearly examinations are therefore recommended. Major prognostic criteria for the ocular adnexal lymphomas include the age of the patient, anatomical location of the tumour, stage of disease at first presentation, serum lactate dehydrogenase level at the time of diagnosis, lymphoma subtype as determined using the W.H.O. lymphoma classification and the tumour cell growth rate. The clinical symptoms and the histopathological findings of the differential diagnoses of lymphoproliferative lesions of the ocular adnexa are discussed.

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Meinem Mann Dr. H. Heimann danke ich für die Übersetzung des Manuskriptes aus dem Englischen. Professor Dr. H. Dürkop und Frau Dr. Jähne danke ich für den fachlichen Beistand und die sorgfältige Durchsicht des Artikels.

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  1. Institut für Pathologie, Charité Universitätsmedizin, Campus Benjamin Franklin, Berlin

    S. E. Coupland

  2. Institut für Pathologie, Charité Universitätsmedizin, Campus Benjamin Franklin, Hindenburgdamm 30, 12200, Berlin

    S. E. Coupland

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Coupland, S.E. Lymphoproliferative Läsionen der okulären Adnexe. Ophthalmologe 101, 197–217 (2004). https://doi.org/10.1007/s00347-003-0854-7

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