Journal d'ophtalmologie clinique et expérimentale
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A 47-Year-Old Woman with a History of Tularemia and Regression of an Untreated Juxtapapillary Amelanotic Choroidal Malignant Melanoma More than Four Decades Following Initial Diagnosis

Nils Andreas Eide, Per Syrdalen, Jesintha Navaratnam*

Background: We report a case of a symptomatic small Uveal Melanoma (UM) followed for 4 decades, the most common primary intraocular tumor that regressed almost completely after an infectious disease, Tularemia, caused by Francisella tularensis.

Case presentation: In 1981, a 47-year old nurse was diagnosed with a left-sided amelanotic juxtapapillary lesion presumed to be a melanoma. In 1985, the visual acuity decreased to 0.5. The tumor became increasingly pigmented and Sub-Retinal Fluid (SRF) was observed. Ultrasound B-scan demonstrated findings characteristic of a UM. Enucleation was not recommended due to small size and useful vision. The lesion was evaluated annually with fundus images and ultrasound B-scan. In 1993, an epidemic nephropathy presented with anuria. The serum tests, Immunoglobulin M (IgM) and IgG, were positive for Tularemia. Her vision deteriorated to 0.04 in 1995. A central depigmented zone, disappearance of SRF and minimal growth in height were interpreted as signs of regression. Metastatic work-up revealed no pathology. Neither a Fine Needle Aspiration Biopsy (FNAB) nor brachytherapy was recommended.

In 2023, the Best Corrected Visual Acuity (BCVA) was finger counting in her left eye. The area of the previous mass was atrophic except for a small, pigmented area in the upper central corner of the primary lesion, and the lesion was flat on the ultrasound B-scan.

Conclusion: Tularemia most likely initiated the almost complete spontaneous regression of UM.