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Impressum
Primary Uveal Non-Hodgkin`s Lymphoma (NHL) with Extrascleral Extension: Manifestation, Diagnosis and Treatment
1Scheuerle A. F., 2Mechtersheimer G., 3Ott G., 1Holz F.
Departments of Ophthalmology1 and Pathology2, University of Heidelberg, INF 400, 69120 Heidelberg, Dpt of Pathology3, University of Würzburg, 97078 Würzburg
Objective: Primary uveal NHL is a dignostic pitfall because it
masquerades as other uveal tract disorders including choroidal effusion
syndromes and other diffuse malignant unveal tumors. Identification and
biopsy of an extrascleral extension followed by immunohistochemical and
molecular biologic examination are crucial for correct diagnosis and subsequent
therapeutic management.
Methods: Imaging studies including echography and MRI demonstrate
diffuse uveal infiltration and extrascleral extension of the tumor. Immunohistochemical
examination of the biopsy and molecular biological work-up were performed
for classification and to determine clonality.
Results: A 53-year-old man initially presented with acute angle-closure
glaucoma secondary to ciliary body involvement, while a 63-year-old woman
presented with unilateral metamorphopsia. Ocular signs included choroidal
folds and a diffuse dark-reddish discoloration. A biopsy of the retrobulbar
extrascleral extension was obtained. Immunohistochemical examination was
consistent with a low-grade B-cell NHL (CD20-positive, CD21-negative,
Ki67<4%). PCR using appropriate primers for the CDR-III region of the
immunoglobulin heavy chain gene revealed a monoclonal expansion of B-lymphocytes.
Staging examinations showed no extraocular manifestation. Complete regression
of uveal thickenig and visual recovery was noted after fractionated radiation
therapy (45 gray) of the retrobulbar mass and the globe. There was no
evidence of recurrence over the period of one and four years, respectively.
Discussion: The favourable clinical course of primary uveal NHL
is in sharp contrast to the dismal prognosis of oculocerebral NHL. As
a retrobulbar biopsy is easier to perform and less invasive compared to
a choriodal biopsy, it appears prudent to detect extrascleral extensions
for establishing the diagnosis. External radiotherapy may result in complete
resolution of uveal thickening and improvement of visual function.