Choroidal melanomas are the most common primary intraocular malignancies in adults. They arise from melanocytes within the choroid and can be pigmented or amelanotic. Risk factors include light iris color and increased sun exposure. Diagnosis is based on clinical appearance, ultrasound, and fluorescein angiography. Prognosis depends on tumor size, cell type, genetic factors, and presence of extrascleral extension. The liver is the most common site of metastasis.

1. CHOROIDAL MELANOMA
DR K HARIPRIYA
SSSIHMS

2. • Choroidal melanomas are the most
common primary intraocular malignancies
in adults.
• It is the second most common type of
primary malignant melanoma in the body
• Choroidal melanoma is a subtype of uveal
melanoma

3. Epidemiology
• Incidence of primary choroidal melanoma
is about 6 cases per 1 million population in
USA.
• Perhaps because of increased sunlight
exposure, there appears to be a higher
incidence of uveal melanoma in the
southern latitudes of the United States
• Other countries has almost same
incidence.

4. Etiology
• Risk factors are people with light-colored
iris, whites, median age-55 yrs, men
• Sunlight exposure is a contributory factor.
• Predisposing diseases
1)family history of uveal melanoma
2) uveal nevus
3)congenital ocular melanocytosis
4)dysplastic nevus syndrome
5) xeroderma pigmentosum.

5. Pathophysiology
• Primary choroidal melanoma arises from
melanocytes within the choroid
• Three distinct cell types are recognized:
(1) spindle A cells
(2) spindle B cells
(3) Epithelioid cells
The last cell type usually has the most
aggressive behavior and carries a poorer
prognosis for the patient’s long-term
survival.

6. • Choroidal melanomas may be darkly
pigmented or amelanotic.
• They are typically dome-shaped.
• As they enlarge, they break through the
Bruch membrane and assumes a
mushroom configuration.
• Other shapes found are bilobular,
multilobular, and diffuse. The diffuse type
is characterized by lateral growth
throughout the choroid with minimal
elevation.

8. • Fig. 12.24 Choroidal melanoma. (A)
Highly pigmented melanoma; (B)
amelanotic melanoma; (C) melanoma with
surface orange pigment; (D) ‘collar-stud’
melanoma with intrinsic vessels; (E)
diffuse melanoma; (F) large melanoma
with subtotal retinal detachment
(Courtesy of B Damato – figs A, C and F);
AD Singh, from Clinical Ophthalmic
Pathology, Elsevier, 2007 – fig. E)
•

9. • Choroidal melanomas affect the retinal
pigment epithelium as they push against it
and deprive it of normal choroidal
circulation.
• Overlying retinal pigment epithelium
usually develops areas of atrophy, drusen,
and localized pigment epithelial
detachments.
• These changes can lead to choroidal
neovascularization over the tumor, with
consequent subretinal exudation,
hemorrhage, and fibrous plaque formation

10. • The tumor disrupts choroidal circulation
leading to ischemia typically cause
degeneration of retinal photoreceptors and
other retinal neurons.
• The retina overlying the tumor can
separate into cystoid spaces and larger
schisis cavities (cystoid macular edema).
• Exudation of fluid into the subretinal
space with consequent retinal detachment.
• Rarely, choroidal melanomas can impinge
into underlying posterior ciliary nerves,
causing severe ocular pain

11. • Tumor grows anteriorly, involving the
ciliary body, trabecular meshwork, and
lens, with consequent ocular hypotension
or hypertension, cataract, iris rubeosis,
vitreous hemorrhage or hyphema.
• Its metastatic potential depends on the
histopathologic aggressiveness of the
tumor cells.
• It can only spread hematogenously,
because there are no lymphatic vessels in
the eye. It most often metastasizes to the
liver, lung, bone, skin, and CNS.

13. • Less frequently, tumor can grow
transsclerally, through emissary channels,
and metastasize locally into the orbit or
rarely the conjunctiva.
• Choroidal melanoma almost never
extends through the optic nerve; when it
does, it is usually in juxtapapillary tumors
or in diffuse choroidal melanomas

14. Histologic Findings
• Histologic evaluation of the tumor after
enucleation can confirm the diagnosis and
determine the prognosis

15. Spindle A cells have elongated
nuclei and uncommonly have
mitotic figures
Spindle B cells have a prominent
nucleolus. They are found more
commonly and also have an elongated
profile but are slightly larger than
spindle A cells.
Epithelioid melanoma cells are
highly anaplastic, poorly cohesive,
polygonal and contain frequent
mitotic figures

17. • Fig. 12.23 Histology of choroidal
melanoma. (A) Spindle cells – tightly
arranged fusiform cells with indistinct cell
membranes and slender or plump oval
nuclei; (B) epithelioid cells – large
pleomorphic cells with distinct cell
membranes, large vesicular nuclei with
prominent nucleoli, and abundant
cytoplasm; (C) fascicular pattern –
vasocentric; (D) necrotic tumour – cell type
cannot be determined; (E) penetration of

18. • AFIP Classification of uveal melanomas.
1)Spindle cell nevi
2)Spindle cell melanomas (mixture of
spindle A and B cells).
3)Mixed cell melanomas in which there is a
mixture of spindle and epithelioid cells.
4)Epitheloid cell melanoma
Last two types has poorer survival prognosis
Other type- necrotic melanoma

19. Adverse prognostic factors
1)Histological features include large
numbers of epithelioid cells, long and wide
nuclei, multiple nucleoli, closed vascular
loops within the tumour and lymphocytic
infiltration.
2)Chromosomal abnormalities: loss of
chromosome 3 and gains in chromosome
8, are associated with a poor prognosis.
Gains in the short arm of chromosome 6
carry a favourable prognosis.

20. 3)Size. Large tumors have a worse
prognosis than small tumors.
4)Extrascleral extension as tumor is more
likely to be advanced and aggressive.
5)Location. Anterior tumors involving ciliary
body have a worse prognosis.
6)Local tumor recurrence after
conservative treatment is associated with
poor survival. This is probably because the
recurrence is an indication that the original
tumor was relatively aggressive

21. Clinical Presentation
Patient history
• Choroidal melanomas remain asymptomatic for long time; they
may be found incidentally during ophthalmoscopy.
• Blurred visual acuity
• Paracentral scotoma
• Painless and progressive visual field loss
• Floaters
• Severe ocular pain
• History of weight loss, marked fatigue, cough, or change in
bowel or bladder habits

22. CLINICAL APPEARANCE- on
ophthalmoscopic examination
• COLOUR
• SHAPE
TYPES- 1) Solid mass leision
2)Flat,diffuse type
-Ring melanoma
ADVANCED STAGES:
a)Extraocular extension
b)Invasion of optic nerve

23. c)glaucoma
d)Uveitis, pthisis bulbi
e)Orbital invasion
f)Metastasis

24. classification
Based on thickness and basal size.
The tumour is termed
-small (<10 mm diameter),
-medium (10-15 mm diameter,<10mm
height)
-large (>15 mm diameter, >10mm height)

25. Diagnosis
• Clinical appearance
• FFA
• Ultrasound
• Radiography
• Laboratory tests
• Transillumination
• Invasive technique
• New diagnostic tests

26. CLINICAL APPEARANCE
Features helpful in diagnosis:
1)Pigmented mushroom shaped tumor
2)Orange pigmentation(due to lipofuscin)
3)Associated elevated retinal detachment
4)Globular elevated mass

27. Differential Diagnosis
Choroidal neoplasms:
• choroidal nevus
• Choroidal Metastasis
• Choroidal hemangioma
• Melanocytoma
• Choroidal lymphoid tumors
Hemorrhagic processes:
• Extramacular disciform degeneration
• Choroidal haemorrhage
• Haemorrhage into retinal cyst
Retinal pigment epithelium
• Retinal pigment epithelial hyperplasia and hypertrophy
• Retinal pigment epithelial Adenocarcinoma

28. Fundus Fluorescein angiography
• Fluorescein angiography and are not diagnostic. They heip to
differentiate between choroidal melanoma and
pseudomelanoma .
• Small choroidal melanomas with intact RPE shows no
changes.
• Larger melanomas with disrupted RPE may show
Arterial phase-mottled hyperfluorescence
Venous phase-pinpoint hyperfluorescence
Late venous phase- late staining due to SRF

29. • Large dome shaped melanoma
Mottled hyperfluorescence in Early venous
phase with late hyperfluorescence
• Mushroom shaped melanoma
During late arterial or early venous phase
the prominent vessels are seen within
dome of the tumour, thereby allowing both
retinal and choroidal vessels seen
simultaneously(double circulation pattern)

31. ultrasonography
A-SCAN:
• A-scan ultrasonography is useful for tumors
thicker than 2-3 mm.
• Choroidal melanoma shows an initial prominent
spike, followed by low-to-medium internal
reflectivity with diminishing amplitude and a
significant echo.
• Performing sequential A-scans, with accurate
dimension measurements, in cases of diagnostic
uncertainty is important.

32. B-SCAN:
• B-scan is a routine test used in the evaluation of
any posterior segment mass.
• It is especially needed in patients with media
opacity.
• B-scan helps in
-establishing the diagnosis,
-to evaluate possible extraocular extension
-to estimate tumor size for periodic observation
-to plan therapeutic intervention.

33. Intraocular melanomas have several distinctive features on B scan:
• Low-to-medium reflectivity
• Excavation of underlying uveal tissue
• Shadowing of subjacent soft tissues
• Internal vascularity
• An acoustic quiet zone at the base of the tumor called acoustic
hollowing

34. Ultrasound biomicroscopy (UBM)
• It can differentiate very anterior choroidal
melanomas from those of ciliary body
origin.
• It is also helpful in assessing angle-closure
glaucoma

35. Radiography
Computed Tomography
• CT scan is more expensive and is not as
sensitive as ultrasonography.
• It is useful for visualizing extraocular
extension and may help differentiate
between choroidal or retinal detachment
and a solid tumor.
• CT scan also is sensitive in detecting
calcium (characteristically choroidal
osteoma).

36. Magnetic Resonance Imaging
• MRI is more expensive and still remains less sensitive .
• Use of surface coil imaging and gadolinium as a contrast
material greatly improves its resolution.
• Pigmented melanomas are seen as a high-density image in T1
and as a low-density image in T2 .
• MRI also can be used to determine extrascleral extension and
distinguish surrounding fluid from the tumor.

37. Laboratory Studies
• Liver enzyme levels are indicated in any patient with uveal
melanoma, because the liver is the most common site of
choroidal melanoma metastasis.
• The most sensitive tests of hepatic function are serum levels of
the following:
-Alkaline phosphatase
-Glutamic-oxaloacetic transaminase
-Lactate dehydrogenase
-Gamma-glutamyl transpeptidase

38. Transillumination
• Rarely done these days.
• Transillumination can be used to find the
borders of the tumor, especially if it is
surrounded by exudative retinal
detachment.
• Its precision is dependent on even tumor
pigmentation and if associated
hemorrhage is present

39. Invasive technique
• Fine-needle biopsy and incisional biopsy
are not usually required but may be helpful
in case where diagnosis is not established.
• particularly for distinguishing amelanotic
melanomas from metastatic tumors.
• Fine-needle biopsy is increasingly being
performed for prognostic purposes
• In opaque medias, ultrasound guided
approach is essential.

40. • It is done by 25 guage needle via
transvitreal or transcleral route.
• Risk of spread of cancerous cells in the
case of fine-needle biopsy is small
• Genetic analysis and karyotyping of biopsy
specimens have gained increasing
attention.
• Other test- radioactive phosphorus uptake
test- used less commonly.

41. New diagnostic tests
• Colour doppler
• Positron emission tomography
• P-31 magnetic resonance spectroscopy
• Monoclonal antibody tagged with a short
lived radioactive technicium-99m tracer

42. Treatment
The methods of patient management
depend on several factors:
-size, location, and extent of the tumor
-visual status of the affected eye and of the
fellow eye
-age and general health of the patient
-patient's wishes and fears

43. • Observation may be acceptable for posterior uveal
tumors where diagnosis is not well established.
• In particular, tumors of less than 2-2.5 mm in elevation
and 10 mm in diameter can be observed until growth is
documented.
• Photography and sequential ultrasonography for precise
measuring of the tumor’s dimensions are usually
necessary.
• Choice of treatment of choroidal melanoma remains
controversial in many respects.
• Although enucleation has been the treatment of choice
in the past, it appears that vision-sparing approaches
might offer similar degrees of ocular and metastatic
tumor control particularly because it is clear that in many
patients at the time of diagnosis, posterior uveal
melanomas already have spread through
micrometastasis.

44. Brachytherapy
• Brachytherapy (episcleral plaque radiotherapy)
with ruthenium-106 or an iodine-125 applicator
is usually the treatment of first choice
1.Indications
-Tumours less than 20 mm in basal diameter in
which there is a reasonable chance of salvaging
vision.
-5 mm thick with a ruthenium plaque
-10 mm thick with an iodine plaque.

45. 2)Technique
a.The tumour is localized by transillumination or
binocular indirect ophthalmoscopy.
b. A template consisting of a transparent plastic
dummy or metal ring with eyelets is sutured to the
sclera with a releasable bow
c.The sutures are loosened and used to secure the
radioactive plaque.
d. The plaque is removed once the appropriate
dose has been delivered, usually within 3–7 days.
At least 80 Gy should be delivered to the tumour
apex.

46. 3)Tumour response is usually gradual
Tumour regression starts about 1–2 months after
treatment and continues for several years,
leaving a flat or dome-shaped pigmented scar.
4)Complications
Excessive irradiation causes cataract,
papillopathy and maculopathy. The irradiated
tumour can cause macular edema , retinal hard
exudates, serous retinal detachment, rubeosis
and neovascular glaucoma (‘toxic tumour
syndrome’).
5)Survival: similar to that following enucleation for
comparable tumors

48. External beam radiotherapy
Irradiation with charged particles such as
protons achieves a high dose in the
tumour with a relatively small dose in the
superficial tissues.
Indications: tumours unsuitable for
brachytherapy either because of large size
or posterior location making positioning of
a plaque unreliable.
Survival results: similar to brachytherapy
or enucleation

49. Transpupillary thermotherapy
• Transpupillary thermotherapy uses an infrared
laser beam to induce tumour cell death by
hyperthermia but not coagulation. It is useful
adjunct to radiotherapy.
Indications :1)Small, pigmented choroidal
tumour(<3mm), away from macula, when
differentiation between naevus and melanoma is
not possible
2)Small choroidal melanoma when radiotherapy
is inappropriate because of poor general health
or reduced life expectancy.
3)After radiotherapy, as a treatment for
exudation threatening vision.

50. Laser photocoagulation
• Is of limited value
• It is used to treat selected small choroidal
melanomas.
• when the lesions are located away from the fovea
and are less than 3.8 mm in thickness,<10mm in
diameter.
SOURCES: xenon arc
krypton laser

51. Enucleation
Indications:
-large tumour size,
-optic disc invasion,
-extensive involvement of the ciliary body or angle,
-irreversible loss of useful vision and
-poor motivation to keep the eye.
Modified Technique:
It is essential to perform ophthalmoscopy after draping
the patient to ensure that the correct eye is treated.
No touch cryosurgical enucleation(minimal trauma
technique)
Pre-enucleation radiotherapy

53. Orbital exenteration
• Reserved for cases with widespread orbital
extension.
• Patients with such advanced melanomas are
likely to have extensive distant metastases
and poor prognoses.
• The procedure should be considered only in
rare cases where marked discomfort is
associated with massive orbital spread of the
melanoma.

54. Other procedures:
Pars plana vitrectomy endoresection
endoresection for posterior choroidal melanomas
Block excision
It is reserved for small tumors covering less than one third of
the globe’s circumference.
Trans-scleral choroidectomy
Indicated for tumours too thick for radiotherapy but
usually less than 16 mm in diameter.

55. • Systemic chemotherapy and
immunotherapy
- No distinct role
• Photoirradiation
• Ferromagnetic hyperthermia

56. Further outpatient care
• Irrespective of the treatment modality chosen, patients with
choroidal melanomas need to be observed carefully for many
years.
• This is particularly true for small tumors, when the diagnosis is
not established clearly.
• Close observation and measurement of the dimensions of the
tumors is critical.
• Repeat examinations usually are performed about every 3
months initially, and if no changes are seen, follow-up care is
performed every 6 months. If growth of the lesion is detected,
consider further treatment.
• The goal of successful treatment is not necessarily reduction in
size but long-term arrest of the tumor’s growth.
• The possibility of intraocular or extraocular tumor recurrence
should be kept in mind.
• Early detection of distant metastases may affect management
and survival.

57. Dr. Finger suggests that you "Think of
Sunglasses as Sun Block for your Eyes" and
start wearing your UV blocking sunglasses.
They make great gifts too!

58. THANK YOU