Maligne epibulbäre Tumoren: Neue Strategien in Diagnostik und Therapie

Zusammenfassung

In dieser Übersichtsarbeit werden die wichtigsten malignen konjunktivalen Neoplasien abgehandelt und die komplexe Diagnostik und multimodale Therapie für die verschiedenen Tumorentitäten zusammengefasst. Das Bindehautmelanom stellt vor allem in seiner Abgrenzung zu benignen melanozytären Tumoren der Bindehaut eine diagnostische Herausforderung dar. Konsequentes chirurgisches Vorgehen gepaart mit einem schlüssigen adjuvanten Therapiekonzept für die jeweilige Tumorsituation sind Basis einer kurativen Therapie. Konjunktivale intraepitheliale Neoplasien (CIN) sind in ihrer mittleren und schweren Form Vorstufen des invasiven Plattenepithelkarzinoms (PE-Ca). Problematisch ist hierbei die häufig großflächige Ausbreitung des invasiven Tumors oder begleitender CINs, die eine R 0-Resektion erschweren oder unmöglich machen. Adjuvanten bzw. begleitenden Therapien kommt daher eine besondere Bedeutung zu, um Tumorkontrolle und Funktionserhalt des Auges zu erreichen. Lymphome sind die häufigsten malignen primären Tumoren der Augenhöhle und der okulären Adnexe. In diesem Zusammenhang können die Lymphome als primäre oder sekundäre Tumoren in den Konjunktiven, der Tränendrüse, dem orbitalen Fett, Augenlid und Tränensack auftreten. Die häufigste Manifestation der okulären MALT-Lymphome sind die Konjunktiven mit 20 – 33 % von allen epibulbären Lymphomen. Mehr als 75 % der okulären Lymphompatienten entwickeln eine einzige lymphomatöse Läsion. Eine Immunphänotypisierung ermöglicht die genaue Differenzierung der Lymphomentitäten. In der Pathogenese werden heute auch infektiologische Faktoren, z. B. durch Chlamydia psittaci berücksichtigt. Zuletzt wird aus strahlentherapeutischer Sicht eine umfassende Übersicht über die an der Konjunktiva sinnvoll einsetzbaren Therapieverfahren gegeben.

Abstract

In this article we discuss the complex diagnostic approaches and therapeutic options for the most important conjunctival malignancies. Conjunctival melanoma can be a diagnostic challenge as it can be difficult to distinguish from benign melanocytic conjunctival tumours. Complete surgical excision accompanied by a coherent adjuvant concept is the key for a curative therapy. Moderate and severe conjunctival intraepithelial neoplasias (CIN) are precancerous lesions and can progress to invasive squamous cell carcinoma. The involvement of large parts of the ocular surface can prevent an R 0-resection. Adjuvant therapeutic concepts are therefore especially important to gain tumour control and preserve the function of the affected eye. Lymphomas are the most common malignant primary tumours of the orbit and ocular adnexa. They can present as primary or secondary tumours of the conjunctiva, the lacrimal gland, the orbital fat, the eye lid or the lacrimal sac. The most common manifestation site of ocular MALT lymphoma is the conjunctiva with 20 – 33 % of all epibulbar lymphomas. More than 75 % of ocular lymphoma patients develop only one lymphomatous lesion. Immunophenotyping allows the exact differentiation between the lymphoma entities. Infectious agents (e. g., Chlamydia psittaci) seem to play a role in the pathogenesis. An overview over radiotherapeutic approaches that are conclusively applicable at the conjunctiva completes the article.

Schlüsselwörter

Bindehautmelanom - Plattenepithelkarzinom/CIN - epibulbäres Lymphom - topische Chemotherapie - Radiotherapie

Key words

conjunctival melanoma - squamous cell carcinoma/CIN - epibulbar lymphoma - topical chemotherapy - radiotherapy

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