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Klin Monbl Augenheilkd 2016; 233(07): 824-846
DOI: 10.1055/s-0042-110574
Übersicht
Georg Thieme Verlag KG Stuttgart · New York

Lymphome des peri- und intraokularen Gewebes – klinisch-pathologische Korrelationen

Lymphoma of Ocular and Periocular Tissues – Clinicopathological Correlations
I. Schmack
1   Klinik für Augenheilkunde, Klinikum der Johann Wolfgang Goethe-Universität, Frankfurt am Main
,
H. E. Grossniklaus
2   L.F. Montgomery Laboratory, Emory Eye Center, Emory University, Atlanta, Georgia, United States
,
S. Hartmann
3   Dr. Senckenbergisches Institut für Pathologie, Klinikum der Johann Wolfgang Goethe-Universität, Frankfurt am Main
› Author Affiliations
Further Information

Publication History

eingereicht 06 April 2016

akzeptiert 14 June 2016

Publication Date:
28 July 2016 (online)

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Zusammenfassung

Lymphome der okulären Adnexe und des intraokularen Gewebes stellen ein weites Spektrum neoplastischer lymphoproliferativer Erkrankungen dar. In der überwiegenden Zahl handelt es sich um extranodale Non-Hodgkin-Lymphome (NHL), die sich entsprechend der Klassifikationskriterien der Weltgesundheitsorganisation (WHO) anhand morphologischer, immunophänotypischer und molekulargenetischer Eigenschaften in Lymphome der B- (etwa 80 % aller NHL) und T-Zell-Reihe (etwa 20 % aller NHL) unterteilen lassen. Zu den häufigsten am Auge vorkommenden B-Zell-NHL zählen das extranodale Marginalzonenlymphom (EMZL) des mukosaassoziierten lymphoiden Gewebes (MALT-Typ), das follikuläre Lymphom (FL), das diffus-großzellige B-Zell-Lymphom (DLBCL) sowie das Mantelzelllymphom (MCL). Die klinisch oftmals individuellen, sehr unterschiedlichen Symptome und Befunde ergeben sich i. d. R. aus der Lokalisation, Größe und Ausdehnung der Erkrankung. Niedrigmaligne NHL können aufgrund ihres vorwiegend indolenten Charakters und langsamen Wachstums nicht selten über Jahre hinweg unerkannt bleiben. Demgegenüber zeichnen sich hochmaligne NHL wie das DLBCL oder MCL durch ein aggressives Wachstumsverhalten und eine, trotz frühzeitiger Diagnosestellung, vielfach schlechte Prognose aus. Trotz zahlreicher Fortschritte auf dem Gebiet der Diagnostik bleibt die Histopathologie, unterstützt durch die Immunhistochemie und Molekulargenetik, weiterhin der Goldstandard bei der diagnostischen Einteilung der einzelnen Lymphomformen. Insbesondere für die sich in der Weiterbildung befindlichen Augenärzte erscheint die frühzeitige Vermittlung der der Entstehung und Entwicklung orbitaler und okulärer Lymphome zugrunde liegenden pathophysiologischen sowie klinisch-pathologischen Zusammenhänge wichtig. Hieraus ergeben sich relevante Hinweise nicht nur im Hinblick auf eine optimierte und zeitnahe Diagnosestellung, sondern auch auf die Behandlung, den Therapieerfolg und die weitere Prognose, die in sich stark vom jeweiligen Lymphomsubtyp abhängig sind.

Abstract

Lymphomas of the ocular adnexa and intraocular tissue include a wide range of lymphoproliferative neoplastic disorders. They are predominantly extranodal non-Hodgkin lymphomas (NHL). The World Health Organization (WHO) classification of lymphoid neoplasm and individual morphological, immunophenotypical, and molecular genetic features, indicate that they may be divided into B-cell (approximately 80 % of all NHL) and T-cell lymphomas (approximately 10–20 % of all NHL). The most common forms of ocular NHL are extranodal marginal zone lymphoma (EMZL) of the mucosa-associated lymphoid tissue (MALT-type), follicular lymphoma (FL), diffuse large B-cell lymphoma, and mantel cell lymphoma. The clinical signs and symptoms are usually very unspecific and depend on the location, size, and extent of the underlying lymphoma subtype. Typical low grade lymphomas have an indolent clinical course and often remain unrecognized for many years. On the other hand, high grade NHLs, such as DLBCL or MCL, are frequently aggressive, with rapid tumour growth and poor prognosis, despite early detection. Histopathology is still the gold standard in the diagnosis of ocular lymphomas. Basic understanding of the principal pathophysiological and clinical aspects of the development and progression of orbital and ocular lymphomas seems to be mandatory for optimal diagnosis and treatment and for improving survival and prognosis. Both residents in training and board certified ophthalmologists should be aware of these problems.

Schlüsselwörter

Lymphom - okuläre Adnexe - intraokuläres Lymphom - extranoduläres Lymphom - Non-Hodgkin-Lymphom - B-Zell-Lymphom

Key words

lymphoma - ocular adnexal lymphoma - intraocular lymphoma - extranodal lymphoma - non-Hodgkin Lymphoma - B-cell lymphoma
 

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