Assessment of Vitrectomy Specimens in the Diagnostic of Primary Intraocular Lymphoma

¹Coupland S., ²Bechrakis N. E., ³Anastassiou G., ⁴Foerster A. M. H., ⁵Heiligenhaus A., ⁵Lommatzsch A., ⁶Pleyer U.,
¹Freie Universität Berlin, Klinikum Benjamin Franklin, Institut für Pathologie (Berlin)
²Freie Universität Berlin, Klinikum Benjamin Franklin, Augenklinik (Berlin)
³Universität-Gesamthochschule Essen, Zentrum für Augenheilkunde (Essen)
⁴Universität zu Köln, Universitätsaugenklinik (Köln)
⁵St. Franziskus-Hospital, Augenabteilung (Münster)
⁶Humboldt-Universität zu Berlin, Charité Campus Virchow-Klinikum, Augenklinik (Berlin)

Purpose: Primary intraocular lymphoma (PIOL) is a rare diffuse large cell B-cell lymphoma, which involves the retina, the vitreous and/or the optic nerve. It can occur independently or in conjunction with a primary central nervous system lymphoma. PIOL often presents as a masquarade syndrome in the form of a steroid-resistent uveitis. Establishment of the diagnosis is often very difficult, requiring conventional and immunocytological examinations of vitrectomy specimens. The cellular content of the latter can be sparse or of poor morphological quality, so that false negative or false positive diagnoses are not uncommon. Representing a „quality control“, the current study reviewed the diagnoses of vitrectomy specimens and the corresponding clinical course of patients.
Methods: 80 vitrectomy cases were collected from the consultation files of the Pathology Department of University Hospital Benjamin Franklin, Berlin. Most specimens had been sent unfixed; these were centrifuged, and smears prepared for conventional and immunocytology. Stains included May-Grünewald-Giemsa, and for B-cells (CD79a, PAX5), T-cells (CD3) and macrophages (CD68). Diagnoses, made on the basis of morphology and immunophenotype, included “reactive cellular infiltrate”, “malignant lymphoma”, “suspicious of lymphoma”, and “insufficient for diagnosis”. The corresponding clinical data with regard to treatment and subsequent clinical course was collected, and compared with the diagnosis.
Results: The patients’ age range varied from 21-100 yrs (average, 64 yrs), with a F:M ratio of 1.5:1. Fifty-eight specimens were diagnosed as “reactive”, 7 as definite “malignant lymphoma”, 10 as “suspicious of lymphoma”, and 5 specimens were considered “insufficient for diagnosis”. Comparison of the established diagnoses with clinical data resulted in accordance in 70 cases and discrepancies in 5. Most patients with reactive infiltrates were treated with steroids. Those patients diagnosed with lymphoma (n=14), were treated with either radiotherapy, chemotherapy or a combination of both; 5 developed cerebral lymphomatous manifestation, 3 were still alive at last follow-up, and 11 had succumbed to their disease.
Conclusions: The diagnosis of PIOL is difficult, requiring sufficient material of good quality and the experienced morphological interpretation of conventional and immunocytological investigations. Rapid transport of fresh material to the cytological laboratory is essential. Only when additional material is available should supplementary investigations, such as PCR, be considered.

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