Zusammenfassung
Das Auge und seine Anhangsgebilde stellen einen seltenen Manifestationsort maligner Non-Hodgkin-Lymphome (NHL) dar. In Abhängigkeit von der anatomischen Lokalisation müssen intraokuläre Lymphome von NHL der Adnexstrukturen wie Bindehaut, Tränendrüsen und Orbita unterschieden werden. Während es sich bei der zweiten Gruppe überwiegend um indolente extranodale Marginalzonenlymphome (MALT-Lymphome [„mucosa associated lymphoid tissue“]) oder um sekundäre Manifestationen systemischer NHL handelt, sind primäre intraokuläre Lymphome meist als diffus großzellige B‑Zell-Lymphome (DLBCL) zu klassifizieren und werden als Sonderform des primären DLBCL des zentralen Nervensystems (ZNS) gewertet. Die häufigste Form ist das primäre vitreoretinale Lymphom, das unspezifische klinische Symptome bietet, oft schwierig von einer Uveitis abzugrenzen ist und daher auch als Masquerade-Syndrom bezeichnet wird. Die Diagnose wird üblicherweise durch die zytologische, immunzytochemische und molekulare Analyse eines Glaskörperaspirats gestellt, wird aber oft durch zytolytische Veränderungen, geringe Materialmengen und pseudoklonale Ergebnisse in der Klonalitätsanalyse erschwert. Neuere Ansätze wie der Nachweis von MYD88-Mutationen erlauben eine deutliche Verbesserung der Sensitivität. Enge Kooperation mit den klinischen Kollegen und rasche Aufarbeitung des Materials sind Grundvoraussetzungen für eine optimale Diagnostik.
Abstract
The eye and the ocular adnexae are rare sites for malignant non-Hodgkin lymphoma (NHL). Based on their anatomical location, intraocular lymphomas must be discerned from NHL of adnexal structures including conjunctiva, lacrimal gland, and orbit. Whereas the latter group mostly consists of indolent extranodal marginal zone B‑cell lymphomas of mucosa-associated lymphoid tissue (MALT) type or secondary manifestations of systemic NHL, most primary intraocular lymphomas are classified as diffuse large B‑cell lymphomas (DLBCL) and are considered a variant of primary DLBCL of the central nervous system. The most common form is primary vitreoretinal lymphoma (PVRL), which presents with nonspecific symptoms and is difficult to discern from uveitis. Diagnosis of PVRL is usually made by cytological, immunocytochemical, and molecular analysis of vitreous aspirates. Degenerative changes, limited material, and the occurrence of pseudoclonality in the molecular analysis of B‑cell clonality can hamper diagnostic assessment. Novel techniques such as detection of MYD88 mutations common in PVRL can increase diagnostic sensitivity. Close cooperation with clinical colleagues and rapid specimen processing are fundamental for successful diagnosis.
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F. Fend, D. Süsskind, C. Deuter und S.E. Coupland geben an, dass kein Interessenkonflikt besteht.
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Fend, F., Süsskind, D., Deuter, C. et al. Maligne Lymphome des Auges. Pathologe 38, 515–520 (2017). https://doi.org/10.1007/s00292-017-0378-6
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DOI: https://doi.org/10.1007/s00292-017-0378-6