A 33-year-old Hispanic woman with no history of interest complained of progressive visual loss in her left eye (LE).
Examination revealed a visual acuity (VA) of 0.05 in the left eye.
A high polypoid papillary mass, intensely pigmented, as well as traces of mobile pigment in the vitreous cavity is observed in the eye fundus (FO) of the left eye.
The rest of the exploration was normal.
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A screen effect was observed on fluorescein angiography (FAG).
Ultrasound showed a solid lesion.
In mode A, very high internal reflectivity was observed without spontaneous vascular movement.
At that time, several experts were consulted.
Some patients were diagnosed with malignant melanoma and others with sickle cell disease.
The decision was to continue observing the patient.
Over the course of nineteen years, the lesion progressively increased in size, with no rapid changes in its configuration. Consequently, the etiology of the disease remained the most appropriate.
The patient was admitted due to loss of vision of the left eye and funduscopy became unfeasible, initially due to the vitreous pigment and later due to cataract.
19 years after the first visit, the patient began to complain of severe pain due to acute glaucoma with anterior chamber inflammation and pupillary occlusion without rubeosis, so enucleation was decided.
Anatomopathological examination of the eyeball : the camerular angle is open, contains abundant macrophages filled with pigment in the posterior ciliary mesh, surrounding the Schlemm canal, capturing the anterior ciliary surface.
The crystalline lens is cataratose.
Emerging from the optic nerve head and extending through the juxtapapillary retina, a densely pigmented tumor composed of large polyhedral cells with abundant cytoplasm and picnotic nuclei is observed.
Most cells are anucleolated but a few have small nucleoli.
The tumor shows foci of necrosis on its surface, with sowing of pigment-filled macrophages on the internal limiting membrane and on the vitreous.
In some areas intensely pigmented spindle cells are observed.
Pigmented cells in many areas extend along the inner limiting membrane of the retina reaching the mountainous shore.
The discolored preparations of the tumor show benign cytological characteristics typical of the neoplasm cells.
The tumor does not show mitotic activity, there is no evidence of malignant melanoma or extraocular extension.
