Abstract

P 182

Melanoma-associated paraneoplastic retinopathy

Gerhard Welsandt1, Arno Hueber1, Sascha Fauser1, Jochen W. U. Fries2, Ulrich Kellner3, Sigrid Roters1
1Zentrum für Augenheilkunde, Klinikum der Universität zu Köln, Köln, 2Institut für Pathologie, Klinikum der Universität zu Köln, Köln, 3Augenzentrum Siegburg, Siegburg

Objective
Case report of a 43-year-old man with sudden night blindness since august 2008 with colour vision defects and sensations of 'shimmering lights'. Because of paresthesia in the extremities a complete neurological diagnostic screening including magnetic resonance tomography and puncture of cerebrospinal fluid was done and showed no abnormalities.
Methods
Visual acuity was 100% on both eyes. The slit lamp showed a normal anterior segment and funduscopy including papilla appearance was inconspicuous. Optical coherence tomography and autofluorescence showed no structural changes of retinal and pigment epithelial appearance. The visual field measured with kinetic perimetry showed constricted limitation; in automatic static perimetry no stimulus was recognized at all. The dark adaptometry showed an increase of four decimal powers. There was one mistake when using the Ishihara colour test plates. The Farnsworth colour test showed many tritanomal mistakes. No answers were detected in visual evoked potentials; the multifocal electroretinography showed normal answers; the scotopic electroretinography showed a lowering of the b-wave (negative ERG).
Results
With the given results a melanoma-associated retinopathy (MAR) was presumable. Multiple examinations did not detect a cutan or intestinal melanoma. The tumour marker S100 was stable and within normal range. The histological preparation of a swollen inguinal lymph node showed a pigmented malignant metastasis. The most likely primary tumor was a resected melanocytic tumor in august 2007. The secondary appraisal showed a complete regressive melanocytic tumour, only possibly the former melanoma, because tumour cells or mitotic activity could not be detected.
Conclusions
The melanoma associated retinopathy (MAR) is a rare paraneoplastic syndrome and belongs in company with the carcinoma associated retinopathy to the autoimmune retinopathies. Tumour proteins immitate retinal proteins and are responsible for an autoimmunological antigene reaction against retinal cells. In MAR acquired night blindness is a typical symptom and electrophysiology confirms the defect of bipolar cells. The autoantibodies lead to a functional damage but not to cell destruction. In this reported case the primary tumour was not detected, but the previous removed complete regressive melanoma in the drainage area of a metastasis is probably the focus.

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