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Ophthalmology > Choroidal melanoma > Flashcards

Choroidal melanoma Flashcards

1
Q

Why is choroidal melanoma an important intraocular tumour to know about? 2 reasons

A
  1. Most common primary malignant intraocular tumour
  2. Second most common type of primary malignant melanoma in the body
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2
Q

What ethnic / regional groups are most affected by choroidal melanoma?

A

White people of Northern European descent (Denmark and other Scandinavian countries)

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3
Q

In which part of the world is choroidal melanoma most common and what is the incidence here?

A

Denmark and other Scandinavnian countries: incidence about 7.5 cases / million / year

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4
Q

How does choroidal melanoma affect the sexes differently?

A

Occurs more frequently in men for all age groups except 20-39, when a small predilection exists for women

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5
Q

Which type of eyes does choroidal melanoma most commonly affect?

A

Light-coloured irides

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6
Q

What is the key, most likley contributor to the development of choroidal melanoma?

A

Sunlight exposure

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7
Q

What are 5 predisposing diseases for uveal melanomas?

A
  1. Family history of uveal melanoma
  2. Uveal naevus
  3. Congenital ocular melanocytosis
  4. Dysplastic naevus syndrome
  5. Xeroderma pigmentosum
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8
Q

What is the most common site of choroidal melanoma metastasis?

A

Liver

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9
Q

What are the 3 distinct cell types recognised in uveal melanomas?

A
  1. Spindle A: elongated nuclei, uncommonly have mitotic figures
  2. Spindle B: prominent nucleolus
  3. Epithelioid: anaplastic (unspecialised), poorly cohesive, considerable morphologic variation; resemble epithelial cells, contain frequent mitotic figures
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10
Q

What is the presentation of symptoms often like in choroidal melanoma, and how therefore are they often detected?

A

Often remain asymptomatic for prolonged period, may be found incidentally during ophthalmoscopy

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11
Q

What are 5 symptoms specific to choroidal melanoma (rather than relating to metastasis)?

A
  1. Blurred visual acuity
  2. Paracentral scotoma
  3. Painless and progressive visual field loss
  4. Floaters
  5. Severe ocular pain
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12
Q

What are 4 symptoms that could suggest a primary non-ocular malignancy with choroidal metastasis?

A
  1. Weight loss
  2. Marked fatigue
  3. Cough
  4. Change in bowel or bladder habits
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13
Q

What are 3 infrequent presentations of advanced choroidal melanoma?

A
  1. Painful blind eye
  2. Cataract
  3. Proptosis from tumour transscleral orbital extension
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14
Q

From which examination technique may choroidal melanoma be found incidentally, due to remaining asymptomatic for long periods?

A

Ophthalmoscopy

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15
Q

What factor increases the delay of symptoms of choroidal melanoma?

A

The more anterior the origin, the longer delay of symptoms

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16
Q

What is shown in the image?

A

Choroidal melanoma (see another pic showing dome-shaped choroidal melanoma)

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17
Q

What is the physical appearance of small choroidal melanomas?

A

Nodular, dome-shaped and well-circumscribed mass under the retinal pigment epithelium

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18
Q

How do choroidal melanomas appear as they grow? 3 key things

A
  • May adopt more irregular configurations e.g. bilobular, multilobular, mushroom shapes.
  • May become diffuse choroidal melanoma.
  • Variable colouration
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19
Q

What is diffuse choroidal melanoma?

A

Lateral growth of melanoma throughout choroid with minimal elevation

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20
Q

Describe what is meant by variable colouration of choroidal melanomas.

A
  • Amelanotic to darkly pigmented, some partially pigmented.
  • If light coloured, vascularisation usually can be seen ophthalmoscopically
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21
Q

What type of changes usually overlie choroidal melanomas? Give 3 examples

A

Retinal pigment epithelial changes e.g.:

  1. Drusen
  2. Patches of atrophy
  3. Orange discolouration
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22
Q

What are 3 types of ocular conditions that a choroidal melanoma could remain under?

A
  1. Large exudative retinal detachment
  2. Subretinal haemorrhage
  3. Vitreous haemorrhages
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23
Q

What type of vascular changes may be associated with choroidal melanoma?

A

May show sentinel vessels: dilated episcleral blood vessels vissible through conjunctiva

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24
Q

What type of changes in choroidal melanoma may mean there are visible areas of abnormal hyperpigmentation with the naked eye?

A

Episcleral growth: subconjunctival area of hyperpigmentation (pic shows enucleated eye with this)

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25
Q

What are 5 key investigations to perform in suspected choroidal melanoma?

A
  1. LFTs
  2. Ultrasound
  3. Angiography
  4. Imaging studies
  5. Biopsy
26
Q

Why should you perform LFTs in suspected choroidal melanoma?

A

Liver most common site of choroidal melanoma metastasis

27
Q

What are 4 key things to look at in LFTs performed in suspected choroidal melanoma?

A
  1. ALP (alkaline phosphatase)
  2. Glutamic-oxaloacetic transaminase
  3. LDH (lactage dehydrogenase)
  4. Gamma-GT (gamma-glutamyl transpeptidase)
28
Q

What type of choroidal melanomas is ultrasound useful for?

A

tumours thicker than 2-3mm

29
Q

What can ultrasound be used for in choroidal melanoma?

A

Can use this to evaluate extaocular extension, establish tumour size and plan intervention

30
Q

What does ultrasound characteristically show for choroidal melanomas?

A
  • An initial prominent spike, excavation of underlying uveal tissue, internal vascularity
  • (acoustically quiet at one base due to acoustic shadowing)
31
Q

What 2 typea of angiography are used for suspected choroidal melanoma?

A

FFA and indocyanine green angiography

32
Q

What is the benefit of performing angiography in suspected choroidal melanoma?

A

Don’t show pathognomonic signs of choroidal melanoma but can help point to its diagnosis

33
Q

What are 3 types of imaging studies that may be useful in suspected choroidal melanoma?

A
  1. Chest x-ray to rule out lung mets
  2. CT
  3. MRI of globe and orbit
34
Q

What is the purpose of performing a CT in suspected choroidal melanoma?

A

to visualise extra-ocular extension + differentiate between choroidal or retinal detachment and solid tumour

35
Q

What is the purpose of an MRI of the globe and orbit in suspected choroidal melanoma?

A

To determine extrascleral extension of melanoma and distinguish the surrounding fluid from the tumour

36
Q

What are 2 types of biopsy that can be done in suspected choroidal melanoma?

A
  1. Fine needle biopsy
  2. Incisional biopsy
37
Q

When might a biopsy be required in suspected choroidal melanoma?

A

Not usually required but may be useful in difficult diagnostic cases, and where other tests equivocal

38
Q

What is fine needle biopsy being increasingly used for in suspected choroidal melanoma?

A

Prognostic purposes

39
Q

What has often occurred by the time a patient with choroidal melanoma is diagnosed?

A

Posterior uveal melanomas have already spread through micrometastasis

40
Q

What are 7 factors to take into account when considering the management of choroidal melanoma?

A
  1. Visual acuity of affected eye
  2. Visual acuity of contralateral eye
  3. Size of tumour
  4. Age
  5. General health
  6. Ocular structures involved
  7. Presence of metastatses
41
Q

What are 8 possible management options for choroidal melanoma?

A
  1. Observation
  2. Immunotherapy and targeted therapy
  3. Enucleation
  4. Plaque brachytherapy
  5. External beam irradiation pars plana vitrectomy endoresection
  6. Block excision
  7. Laser photocoagulation and transpupillary thermotherapy
  8. Orbital exenteration
42
Q

When might observation be an acceptable management approach for choroidal melanoma?

A

For posterior uveal tumours where diagnosis is not well-established; if <2-2.5mm and 10mm in diameter, observe until growth documented

43
Q

What does observation as a management option for choroidal melanoma actually mean?

A

Observe until growth documented: photography and sequential US for measuring precise dimensions

44
Q

What is the management of metastatic choroidal melanoma?

A

Immunotherapy and targeted therapy

45
Q

What are the 2 key groups of immunotherapy/ targeted therapy for metastatic choroidal melanoma, and which mutations are they used for?

A
  1. Oral BRAF inhibitors: dabrafenib (tafinlar) and vemurafenib (Zelboraf) for mutated BRAF gene
  2. Oral MEK inhibitors if BRAFV600E or V600K mutation
46
Q

How are different types of immunotherapy for choroidal melanoma usually given?

A

Oral MEK and BRAF inhibitors usually given in combination

47
Q

What are 2 further types of immunotherapy/targeted therapy for choroidal melanoma currently under investigation?

A
  1. KIT inhibitors (in clinical trials)
  2. Tumour-infiltrating lympocyte (TIL)
48
Q

What is enucleation and when is it used?

A

removal of the eye. It is the classic approach for large and complicated tumours, especially if vision affected

49
Q

What is the classic treatment for large and complicated choroidal melanomas?

A

Enucleation

50
Q

What is plaque brachytherapy as a treatment for choroidal melanoma?

A

Uses plaques containing various radioactive isotopes (iridium, cobalt, ruthenium); radiation causes tumour destruction through damage of DNA

51
Q

What type of choroidal melanoma is plaque brachytherapy used to treat?

A

medium-sized posterior uveal melanoma

52
Q

What radioactive isotope is most commonly used for plaque brachytherapy?

A

Iodine-125

53
Q

What is a risk associated with plaque brachytherapy?

A

Risk of iris and anterior chamber angle neovascularisation

54
Q

What type of choroidal melanomas is block excision used for?

A

Reserved for small tumours

55
Q

What is orbital exenteration and when is it used as a treatment in choroidal melanoma?

A
  • Surgical removal of eyeball and surrounding tissues: eyelids, muscles, nerves, fat
  • Used if extraocular local spread
56
Q

Is there effective treatment in existence for metastatic uveal melnaoma?

A

No

57
Q

What is the 10-year mortality post-diagnosis of choroidal melanoma?

A

30-50% (high mortality)

58
Q

What is usually the cause of death from choroidal melanoma?

A

Due to secondary metastases

59
Q

What are 7 features of choroidal melanomas that correlate with increased mortality?

A
  1. Larger size
  2. Anterior location
  3. Transscleral extension
  4. Growth through Bruch membrane
  5. Optic nerve extension
  6. Lack of pigmentation
  7. Mitotic activity and cell type
60
Q

What is usually the result of the primary choroidal melanoma?

A

Partial or total visual loss in affected eye, due to tumour or treatment

Ophthalmology (20 decks)

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