Lymphoma of the Ocular Adnexa

Abstract

Background: Over the last decades, the incidence of ocular adnexal lymphomas has been increasing. Over the last decade advances in the imaging of ocular adnexal lymphomas have enabled precise biopsies of the tumors. Biopsies are necessary for immunophenotyping, correct molecular classification and the immediate start of the appropriate treatment.

Patients and Methods: In a retrospective study, we collected data from 16 patients (19 eyes) treated for lymphoma of the ocular adnexa between 2006 and 2011 with various tumor sites. We evaluated ocular symptoms of the patients in correlation to the tumor localization on imaging and determined the access of biopsy. Follow-up period (13.7 ± 15.4 months) and therapy were analyzed.

Results: The mean age of study patients was 67.4 ± 13.6 years. Seven patients presented with exophthalmos, 6 with upper lid swelling, 5 with double vision, 5 with reduced visual acuity and one patient with retinal detachment. None of the patients complained of pain. In 7 cases the location of the OAL was superior, in 6 retrobulbar, in 4 patients in the lacrimal gland (1 bilaterally affected), one case with lacrimal sac infiltration and another with isolated subconjunctival lymphoma. Patients with retrobulbar tumors complained of visual loss, whereas patients with anterior orbital tumors showed localized nodular swelling. In 11 cases (58 %) the diagnosis was marginal zone B-cell lymphoma (MALT), in 5 (26 %) follicular lymphoma. 11 patients (69 %) received curative and one HIV-positive patient palliative radiation. Three patients were treated with systemic rituximab due to generalized lymphoma and one of them was complemented with CHOP therapy. One patient received chemotherapy alone (CHOP). During the follow-up the HIV-positive patient died.

Conclusions: All our lymphoma patients showed full tumor regression, without side effects from radiotherapy. In this study we emphasize the need to perform a diagnostic biopsy. The risk of biopsy by a trained surgeon is small. Early and accurate diagnosis is crucial for proper treatment. In unclear situations repeated biopsies might be necessary.

Zusammenfassung

Hintergrund: Die Inzidenz der Lymphome der okulären Adnexe (OAL) hat in den letzten Jahrzehnten zugenommen. Aber auch Fortschritte im Bereich der Bildgebung resultieren in exzellenter Darstellung von OALs und ermöglichen so eine zielgenaue Biopsie. Nur durch die Biopsie kann die verbesserte Molekulardiagnostik und das Immunophenotyping zu akkuraten Diagnosen führen und die präziser und effektiver gewordene Radiotherapie zu einem besseren Outcome verhelfen.

Patienten und Methoden: In einer retrospektiven Studie inkludierten wir Daten von 16 Patienten (19 Augen), die zwischen 2006 und 2011 wegen einem Lymphom der okulären Adnexe behandelt wurden. Die Symptome bei Erstvorstellung wurden mit der Tumorlokalisation korreliert, Zugangswege für die Biopsie festgelegt, Therapie und Follow-up (13,7 ± 15,4 Monate) festgehalten.

Ergebnisse: Das Durchschnittsalter bei Erstvorstellung betrug 67,4 ± 13,6 Jahre. Sieben Patienten präsentierten sich mit Exophthalmus, 6 mit Oberlidschwellung, 5 mit Doppelbildern, 5 mit Visusreduktion und einer mit einer Netzhautablösung. Keiner der Patienten beklagte Schmerzen. Die Tumorlokalisation war bei 7 Patienten superior, bei 6 retrobulbär, bei 4 war die Tränendrüse befallen (bei einem sogar bilateral), bei einem der Tränensack und einer zeigte ein subkonjunktivales Lymphom. Die Beschwerden der Patienten mit retrobulbärer Läsion waren vornehmlich Visusreduktion, wohingegen anterior gelegene peribulbäre Tumoren eher durch lokale Schwellungen und/oder erkennbares knotiges Wachstum auffielen. In 11 Fällen (58 %) war die Diagnose MALT-Lymphom, in 5 (26 %) follikuläres Lymphom. Die Therapie erfolgte bei 11 Patienten (69 %) kurativ mittels Bestrahlung und palliativ bei einer HIV⁺-Person. Drei Patienten mit generalisiertem Lymphom wurden systemisch mit Rituximab behandelt, einer davon nachträglich mittels CHOP-Therapie. Ein Patient erhielt alleinige Chemotherapie (CHOP). Innerhalb der Studienzeit verstarb die HIV⁺-Person.

Schlussfolgerungen: Alle behandelten Patienten zeigten volle Regression des Lymphoms ohne Nebenwirkungen der Bestrahlung. Durch einen erfahrenen Orbitachirurgen ausgeführt, ist das Risiko einer Biopsie minimal. Eine histologische Bestätigung der Diagnose erscheint essenziell, um frühzeitig mit der richtigen Therapie zu beginnen und von den Behandlungsmöglichkeiten optimal zu profitieren. In unklaren Fällen bedarf es einer mehrmaligen Biopsie.

Key words

lymphoma - orbit - biopsy - radiotherapy - radiation - ocular adnexal lymphomas - MALT lymphoma

Schlüsselwörter

Lymphom - Orbita - Biopsie - Strahlentherapie - Radiotherapie - MALT-Lymphom

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