Malignant
melanomas of the uvea are the most common intraocular malignancy in
adults. These tumors may
arise from the iris(#22150), ciliary body(#22154, #22157), or choroid
(#22156, #22163). These
tumors arise de-novo or also arise from pre-existing nevi.
Iris melanomas arise from the anterior layer of iris stromal
tissue. These tend to appear
as a variably pigmented mass on the iris but may also diffusely involve
anterior chamber structures causing glaucoma or uveitis.
Histopathologically, the vast majority of malignant melanomas of
the iris are composed of spindle cells.
These tumors are more benign than malanomas of the ciliary body or
choroid and only rarely metastasize.
Malignant melanomas most commonly arise from the posterior uveal
structures, the choroid and ciliary body.
The presenting symptoms depend on the location of the lesion.
If the lesion is located posteriorly, the patient will note blurred
vision. Often, the mass is
found on routine ocular examination.
Histologically, in the absence of extraocular extension, the major
determinants of prognosis are cell type, tumor size, and location.
Cell type is generally categorized under the Callender
classification system.
Spindle-A cells(#22153,#22169) have slender nuclei with delicate
chromatin, ill-defined or absent nucleoli, and no mitotic activity.
Often a longitudinal fold of the nuclear membrane accounts for a
prominent streak of chromatin.
Spindle-B(#22162,#22171) cells have more plump nuclei containing
small but prominent nucleoli and coarse chromatin.
Mitotic figures are common. Epithelioid
cells(#22172) are variable in size but are usually larger and pleomorphic
or polygonal. They exhibit
variations in size and shape of their nuclei and may be multinucleated.
Chromatin shows coarse clumping.
Mitotic figures are abundant.
Cells show loss of intercellular cohesiveness. Lesions composed of exclusively spindle-A cells are now
regarded to behave almost like nevi.
The designation of "mixed" cell type implies the presence
of a mixture of spindle and epithelioid melanoma cells(#22174). Spindle-B tumors may also form a fascicular pattern with
palisading of the cells.
A final classification is that of a necrotic tumor in which
necrosis precludes identification of a cell type.
Other histologic findings may include rupture of Bruch's membrane
with a mushroom-like out-pouching of the tumor underneath the
retina(#22166). In addition,
there may be an associated exudative retinal detachment and also a cystic
degeneration of the retina overlying the tumor.
These tumors may extend from the eye by invasion of scleral
emissarial channels or vortex veins (#22177,#22175).
Direct invasion of the sclera or optic nerve is much less common.
The tumor may extend into the orbit and may matastasize distantly. The most common site of the metastasis is the liver(#22178).
See below
for other views of malignant melanoma:
-(#22562
[mushroom shaped], #22647, #22650, #22674, #22686, #22689, #22710)
-Choroid
(#22541 [fascicular arrangment],
#22583 [mixed type]).
-Iris
(#22544, #22644, #22656, #22659, #22668
[iris and ciliary body with extrascleral
extension]).
-Invasion
of Optic Nerve Head (#22613, #22616, #22619, #22625).
-Extraocular
extension (#22638, #22662 [through vortex vein], #22695 [through vortex
vein], #22698 [posterior extension], #22701 [exra-bulbar extension],
#22704 [liver metastasis])
-Epithelioid
type (#22553, #22568, #22580, #22716)
-Spindle A
type (#22565, #22665)
-Spindle B
type (#22573, #22577, #22586,
#22589)