*4.2.3 Metastasis*

3.Plaque brachytherapy—has up to 87% chance if tumor control after local

5.Enucleation—for diffuse, recurrent tumors or eyes with intractable glaucoma

resection

*Melanoma*

4.Proton beam therapy

*4.1.5 Differential diagnosis*

1.Primary iris cyst

3.Essential iris atrophy

5.Peripheral anterior synechiae

*4.1.6 Factors predictive of metastasis*

1. Increased age at diagnosis [16, 17]

5.Previous surgical intervention before referral prognosis [14]

1.Diminution of vision due to astigmatism or lens dislocation

2.Painless visual field loss or pain due to acute glaucoma

4.Unexplained relatively low intraocular pressure

Prognosis is better than ciliary body or choroidal melanoma with a 10-year metastasis of 7% as compared to 25% in choroidal melanoma and 34% for ciliary

It is relatively a rare uveal tumor and is reported in one of 10 cases of all

3.Elevated intraocular pressure

4.Extraocular extension

**4.2 Ciliary body melanoma**

intraocular melanomas [18, 19].

3.Episcleral sentinel vessels

*4.2.1 Clinical presentation*

**38**

4. Iris foreign body

6. Iris metastasis

2.Angle invasion

body melanoma.

2. Iris nevus

Hematogenous metastasis is faster in ciliary body melanoma as a result of continuous contractions of the ciliary muscle and rich vascularization.


*AJCC 8th edition classification of iris melanoma [20].*


#### **Table 3.**

*The prognostic factors for ciliary body melanoma.*


**Table 4.** *Predisposing factors.*

#### *4.2.4 Prognosis*

The prognostic factors are listed in **Table 3**.

#### **4.3 Choroidal melanoma**

Choroidal melanoma is the most common uveal melanoma and constitutes about 90% of all uveal melanomas. This is usually seen in an elderly age group at around 60 years and there is no gross gender predilection. It is seen predominantly in Caucasians (98%), as compared to other races. It has a pronounced tendency to metastasize resulting in high mortality [21]. Predisposing factors are listed in **Table 4**.

#### *4.3.1 Clinical presentation*

It can be incidentally detected in asymptomatic patients on routine ocular examination. Most of the patients, however, manifest with diminution of vision, floaters, photopsia, visual field loss, or pain due to impingement of posterior ciliary nerve or angle closure glaucoma. It can metastasize to liver (89%), lung (29%), and bone (17%). Median survival after metastasis is 6–12 months [22]. Males have a poor prognosis than females. The lower metastatic rate in females can be explained due to the inhibitory action of estrogen on the growth of micrometastases within the liver [23, 24].

#### *4.3.2 Classification*

Choroidal melanoma can be broadly classified into diffuse (**Figure 2**) and circumscribed (**Figure 3**). The circumscribed variant can either be dome-shaped (75%) or mushroom-shaped (20%). Diffuse choroidal melanoma is seen in 3–17% cases and has a substantial risk of metastasis despite its flat appearance. The poor prognostic factors include delayed diagnosis, greater proportion of epitheloid cells, and a tendency for extraocular extension [25].

The most common precursor lesion for choroidal melanoma is the preexisting

The following are used to differentiate a choroidal nevus from a melanoma

choroidal nevus (**Figure 4**), followed by oculodermal melanocytosis.

(pneumonic: to find small ocular melanoma using helpful hints daily):

1.Thickness > 2 mm

2.Fluid

**41**

**Figure 2.**

**Figure 3.**

**Figure 4.** *Choroidal nevus.*

*Circumscribed choroidal melanoma.*

*Diffuse choroidal melanoma.*

*Ocular Melanoma*

*DOI: http://dx.doi.org/10.5772/intechopen.93760*

AJCC Classification has already been mentioned under the section of iris melanoma (**Table 2**).

**Figure 2.** *Diffuse choroidal melanoma.*

*4.2.4 Prognosis*

*Predisposing factors.*

**Table 3.**

*Melanoma*

**Table 4.**

Local/general signs Local extension Presence of metastasis Age of the patient Dysplastic nevi

Light colored eyes Fair skinned

*The prognostic factors for ciliary body melanoma.*

**Table 4**.

**4.3 Choroidal melanoma**

*4.3.1 Clinical presentation*

the liver [23, 24].

*4.3.2 Classification*

melanoma (**Table 2**).

**40**

and a tendency for extraocular extension [25].

The prognostic factors are listed in **Table 3**.

**Clinical Macroscopic Microscopic**

**Host factors Environment factors**

Size of the tumor <11 mm—small 11–15 mm—medium >15 mm—large

Epitheloid and necrotic cellular patterns

Melanophagic, lymphocytic infiltrate

Intermittent ultraviolet exposure to arc welding

Necrosis

Chronic UV exposure Occupational sunlight exposure

Intense pigmentation

Choroidal melanoma is the most common uveal melanoma and constitutes about 90% of all uveal melanomas. This is usually seen in an elderly age group at around 60 years and there is no gross gender predilection. It is seen predominantly in Caucasians (98%), as compared to other races. It has a pronounced tendency to metastasize resulting in high mortality [21]. Predisposing factors are listed in

It can be incidentally detected in asymptomatic patients on routine ocular examination. Most of the patients, however, manifest with diminution of vision, floaters, photopsia, visual field loss, or pain due to impingement of posterior ciliary nerve or angle closure glaucoma. It can metastasize to liver (89%), lung (29%), and bone (17%). Median survival after metastasis is 6–12 months [22]. Males have a poor prognosis than females. The lower metastatic rate in females can be explained due to the inhibitory action of estrogen on the growth of micrometastases within

Choroidal melanoma can be broadly classified into diffuse (**Figure 2**) and circumscribed (**Figure 3**). The circumscribed variant can either be dome-shaped (75%) or mushroom-shaped (20%). Diffuse choroidal melanoma is seen in 3–17% cases and has a substantial risk of metastasis despite its flat appearance. The poor prognostic factors include delayed diagnosis, greater proportion of epitheloid cells,

AJCC Classification has already been mentioned under the section of iris

**Figure 3.** *Circumscribed choroidal melanoma.*

**Figure 4.** *Choroidal nevus.*

The most common precursor lesion for choroidal melanoma is the preexisting choroidal nevus (**Figure 4**), followed by oculodermal melanocytosis.

The following are used to differentiate a choroidal nevus from a melanoma (pneumonic: to find small ocular melanoma using helpful hints daily):

1.Thickness > 2 mm

2.Fluid


#### *4.3.3.1. Ultrasonography*

It has 95% accuracy and is useful to estimate tumor size for periodic observation and to evaluate for extraocular extension.

*4.3.3.5. Optical coherence tomography*

*DOI: http://dx.doi.org/10.5772/intechopen.93760*

*4.3.3.6. Magnetic resonance imaging*

*4.3.3.7. Fine needle aspiration cytology*

chemotherapy, and immunotherapy.

+sunitinib, and fotemustine.

subretinal fluid.

*Ocular Melanoma*

the affected eye.

intraocular masses.

*4.3.4 Management*

*4.3.5 Histopathology*

1.Spindle cell nevi

2. Spindle cell melanoma

4.Epitheloid cell melanoma

5.Mixed cell melanoma

3.Necrotic melanoma

histopathology.

**43**

Dome-shaped choroidal mass with overlying outer retinal thickening and

Pigmented melanomas can be seen as T1 Hyperdense and T2 hypodense

Although reliable, it is technically challenging and requires expertise.

The various newer treatment modalities under evaluation are:

2.Targeted therapy with crizotinib, sunitinib, and valproic acid.

Modified Callenders's classification describes various patterns on

3. Immunotherapy with Ipilimumab with nivolumab.

1.Chemotherapy with dacarbazine+interferon alpha, cisplatin, tamoxifen

The most common treatment modality is the episceral plaque brachytherapy. Plaque brachytherapy is suitable for tumors up to 16 mm in diameter and up to 6 mm thickness with Ruthenium-106 and up to 8 mm thickness with Iodine-125. The dose to the tumor apex should be 10,000 cGy and almost up to 90% tumor control can be achieved. Enucleation is an option for tumors beyond the scope of plaque brachytherapy. Orbital exenteration might be required in tumors with orbital invasion. The proton beam irradiation has a higher chance of eye salvage but the availability and affordability are the considerable limitations. The other treatment modalities include laser photocoagulation, transpupillary thermotherapy,

Optical coherence tomography angiography shows reduced capillary density in

The characteristic features on A-scan are:

1. Initial prominent spike

2.Low to medium internal reflectivity with diminishing amplitude

3.Fine oscillation of internal spiking pattern (vascular pulsations)

The characteristic features on B-scan are:

1.Low to medium internal reflectivity


#### *4.3.3.2. Autofloroscence*

Hyperautofluorescence of orange-colored lipofuscin pigment.

#### *4.3.3.3. Fundus fluorescein angiography*

Small melanoma: Hypofluorescence (blocked fluorescence) Large melanoma: Patchy pattern of early hypofluorescence and hyperfluorescence followed by late intense staining. Double circulation—internal vascularity

#### *4.3.3.4. Ultrasound biomicroscopy*

It helps to differentiate anterior tumors from those of ciliary body origin. Although the tumor margins and extent is well delineated by UBM, the resolution of internal tumor details is limited.
