Zusammenfassung
Die karzinomassoziierte Retinopathie (CAR) ist ein seltenes paraneoplastisches Syndrom. Innerhalb weniger Monate konnten zwei Patienten mit einer CAR an der Univ.-Augenklinik Tübingen diagnostiziert werden, über die im Rahmen dieser Übersicht berichtet wird.
Am häufigsten wurde die CAR beim kleinzelligen Bronchialkarzinom, in Einzelfällen beim Rhabdomyosarkom, Gebärmutter-, Prostata- und Mammakarzinom beschrieben. Die genaue Pathogenese der CAR ist unbekannt. Eine wichtige Rolle scheint die tumorinduzierte, spezifische Immunreaktion gegen das Photorezeptorprotein Recoverin („23-kd retinal CAR antigen”) zu spielen, die sich nicht bei jedem Patienten nachweisen lässt. Vermutlich sind weitere retinale Antigene an der Pathogenese beteiligt. Symptome machen sich in der Regel bereits vor der Diagnose des Primärtumors bemerkbar. Es kommt zu einer schnell progredienten, meist asymmetrischen Visusminderung, der oft Photopsien und eine vermehrte Blendungsempfindlichkeit vorausgehen. Gesichtsfelddefekte können sehr heterogen sein, häufiger sind Skotome in der mittleren Peripherie oder parazentral. Im ERG zeigen sich typischerweise reduzierte Antworten, obwohl auch von normalen ERG-Befunden bei sonst typischer CAR berichtet wird. Funduskopisch kann sich ein unauffälliger Netzhautbefund zeigen, häufiger sind jedoch Veränderungen des retinalen Pigmentepithels, Einscheidungen der retinalen Gefäße und eine Verengung der retinalen Arteriolen. Die Prognose muss als schlecht eingestuft werden. In den wenigen dokumentierten Verlaufsbeobachtungen in der Literatur kam es ohne Therapie innerhalb von 6 bis 18 Monaten zur Erblindung. Obwohl systemische Kortikosteroide bei einigen Patienten als hilfreich beschrieben wurden, ist ein allgemein anerkanntes und in seiner Wirksamkeit belegtes Therapiekonzept bislang nicht verfügbar.
Als Literatursuche wurden die Datenbanken Medline und Current Contents Search verwendet.
Cancer-associated retinopathy (CAR) is a rare paraneoplastic syndrome. In this survey we report about two further patients with CAR, who were referred to the University Eye Hospital of Tuebingen within a few months. The most common primary tumor associated with CAR is small cell carcinoma of the lung. Case reports about rhabdomyosarcoma, carcinoma of the endometrium, prostate and mamma were also described. The exact pathogenesis of CAR is still unknown. Specific autoantibodies were found against the photoreceptor protein recoverin (23-kd retinal CAR antigen). However, this reaction is not present in all patients, and probably other antigens are also involved. Most of the patients experience symptoms of CAR before the primary tumor is detected. Besides glare sensitivity and flashing lights, a rapidly progressive, often asymmetric visual loss may occur. Although paracentral and midperipheral scotomas can be found frequently, visual field defects are often quite heterogeneous. Typically, the responses in the electroretinogram (ERG) are markedly reduced, but normal ERGs were also described. The fundus picture in CAR shows sheathing of the retinal vessels, narrowing of the arterioles and clumbing of the retinal pigment epithelium. The prognosis is poor. Frequently there is progression to bilateral loss of vision within a few months. Treatment of the primary tumor does not seem to alter the ocular prognosis. Systemic corticosteroids may be helpful in some patients. Nevertheless, no proven therapeutic regimen is currently available.
Literature search was done by Medline and Current Contents Search.
Schlüsselwörter
paraneoplastische Syndrome - karzinomassoziierte Retinopathie - paraneoplastische Optikusneuropathie - Mammakarzinom - Karzinom des Corpus uteri - paraneoplastische Syndrome des zentralen Nervensystems
Key words
paraneoplastic syndrome - cancer-associated retinopathy - paraneoplastic optic neuropathy - mamma carcinoma - carcinoma of the endometrium - paraneoplastic neurological syndromes
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