DIS - Ocular Tumours - Week 9 Flashcards
List four types of intraocular tumours you may encounter.
Uveal melanoma
-choroid
-ciliary body
-iris
Retinoblastoma
Rhabdomyosarcoma
Angiomatosis retinae
What is the most common intraocular tumour? In which eye colour is it most common?
Malignant choroidal melanoma
-more prevalent in lightly pigmented eyes
What is the typical onset for malignant choroidal melanoma? Does it have a high or low mortality rate?
40 to 60 years
High mortality rate
What is mortality rate due to malignant choroidal melanoma a function of (4)?
Cell type
-spindle A
-spindle B
-mixed
-epitheloid
Is a choroidal naevus generally considered benign or malignant? Are there any symptoms associated with it?
A benign melanoma
-no symptoms
What is a choroidal naevus? What colour and what appearance does it have initially? What shape do they generally have? What is the typical diameter and thickness?
It is a pigmented lesion
Slate grey colour with greenish tinge
Initially non-pigmented, acquires colour age 6-10
Round or oval
-average 1-2DD in diameter
Usually <1mm thick
Does the size of a choroidal naevus affect the probability of malignant melanoma or is there no association?
The larger the lesion, the greater the probability
Where are choroidal naevi typically located?
At or around the posterior pole
Do choroidal naevi tend to have associated blood vessels?
No
Do choroidal naevi result in visual field defects?
Possible local defect, but subtle
Do choroidal naevi cause leakage on fluorescein angiography? What do you expect to see?
No leaking
Only masking
What do you expect of size progression in choroidal naevi over the years?
Stationary in size for long periods, typically forever
Do most naevi tend to become malignant?
Most never become malignant
What percentage of malignant melanomas arise from choroidal naevi? What about the rest?
1/3rd come from naevi
2/3rds come from isolated melanocytes
List four differential diagnoses for a choroidal naevus.
Malignant melanoma
RPE pigment accumulations (RPE hypertrophy)
Other choroidal tumours
Sub-retinal blood
List 3 characteristics of RPE hypertrophy.
Darker
Distinct margins
Gaps between pigment
What is the management for choroidal naevi (4)? What is the review schedule and what should you do on review?
Observation
Monitoring
Photography
Visual fields
Review in 1-2 years
-carefully investigate any changes
When should you refer a choroidal naevus (2) and to who?
Refer to an ophthalmologist if there are new symptoms or a change in size
Are changes in choroidal neaevus size normal or is it always sinister?
It can be normal, but refer for caution
List signs and symptoms for a small (1) and large malignant melanoma (3).
Small
-no symptoms if small and/or peripheral
Large
-metamorphopsia
-photopsia
-visual field loss
What colour appearance do malignant melanomas tend to have (4)? What does a pale/translucent appearance indicate?
Grey
Brown
Dark red
Yellow
Pale/translucent - amelanotic
What tinge is often evident on malignant melanomas and what may it indicate?
An orange tinge - lipofuscin
What shape do malignant melanomas tend to have (4)?
Irregular, patchy, round, and/or elevated
What may be seen at the edges of malignant melanomas?
Serous detachment
True or false
Malignant melanomas have no retinal striae/folds
False, they do
Do malignant melanomas tend to be highly or poorly vascularised? Where do they get their supply from? When is this vasculature only visible (2)? What may you see on the surface of tumours and what test would you want to do?
Highly vascularised
-choroidal blood vessels
Only visible if the RPE is thin or tumour breaks through the RPE
May see haemorrhage on the tumour surface
-do angiography
What is common alongside malignant melanoma?
Cystoid macular oedema
What kind of haemorrhage is common with malignant melanomas and from which blood vessels?
Vitreous
-from tumour blood vessels
What should you beware regarding cataract (2)?
Unilateral, rapidly progressing cataract
Describe how a malignant melanoma (or any other intraocular tumour) may cause anterior uveitis.
Tumour necrosis leading to inflammation
Do tumours affect IOP? Explain (2).
Large tumours can
Also if tumours spread to the anterior angle
What is the enlargement of choroidal tumours restricted by (2) and what are they constrained to (what plane)? What appearance do they have when this is so and what is an approximation of their height?
Enlargement restricted by the sclera and bruchs membrane
Constrained within the plane of the choroid
-flatter appearance - height is ~0.5 x diameter
What appearance do choroidal tumours tend to have if they penetrate bruchs membrane?
Collar button effect
Describe systemic metastasis and list major sites of metastasis in percentages (3).
Tumour cells enter the bloodstream via tumour vessels
Liver 34%
Liver and other organs 63%
Other organs 3%
Note the TFSOM-UHHD mneumonic and what it is used for.
Used to differentiate between a choroidal naevus and a malignant melanoma
Thickness (>2mm)
Fluid (subretinal fluid is present)
Symptoms (flashes/floaters/decreased vision)
Orange pigment present
Margins (tumour margin within 3mm of the ONH)
Ultrasonic Hollowness
Halo (absence)
Drusen (absence)
Note the four categories of risk associated with the TFSOM-UHHD mneumonic and what to do for each one.
Based on the number of risk factors:
1-2 - monitor every 4-6 months
3-4 - refer to an experienced centre for oncology evaluation
5-6 - refer to ocular oncology centre for further management
7+ - urgent referral to ocular oncology centre
With how many risk factors according to the TFSOM-UHHD mneumonic is a lesion more likely to be a choroidal melanoma rather than a naevus?
3+
List four other ocular tumours than may be differentials for a malignant melanoma. Note which of these is found on or near the disc.
Melanocytoma (on or near the disc)
Angioma of choroid or retina
Choroidal osteoma
Intraocular lymphoma
List two common primary sites of tumours that can metastasize to the choroid. Where are they usually located (once metastasized) and what colour? What may you often see?
Primary tumour usually in the lung or breast
Typically near the disc and lightly coloured
-may see leukaemic infiltrates
List six non-tumour conditions that can be differentials for a malignant melanoma.
Primary rhegmatogenous retinal detachment
Other secondary retinal detachments
Retinoschisis
Wet AMD (haemorrhagic or serous)
Choroidal and other intraocular haemorrhage
Retinal cyst
What five additional tests should you do or refer for if you suspect malignant melanoma?
Transillumination
Ultrasound
Fluorescein angiography
CT/MRI scan
Intraocular biopsy
When used to assess malignant melanoma, what information does ultrasound provide (3)?
Shape
Depth
Extraocular growth
What is the optometric management of a malignant melanoma and what further tests can you expect to occur once referred (3)?
Refer to an ophthalmologist for further care
-fluoroscein angiogram
-ultrasonograpgy
-hunt for metastasis
Who specifically should you refer to if you have a case of malignant melanoma (ophthalmologist not enough)?
Retinal specialist or ophthalmic oncologist
What are four tests to do when looking for a metastasis?
Blood tests
Liver tests
Physical examination
General examination
-includes scans etc
List 6 treatment options for a malignant melanoma.
Observation
Plaque irradiation
Laser photocoagulation
Proton/helium ion radiotherapy
Local resection
Enucleation
Describe when observation is a treatment option for malignant melanomas.
Small tumours are observed until growth occurs
Describe how plaque irradiation is carried out and how long.
Suture a radioactive plaque to the sclera under the lesion for short periods
List 6 complications of plaque irradiation as a treatment option for malignant melanoma and note which of these is inevitable.
Radiation retinopathy (inevitable)
Radiation cant reach all of the tumour
Tumour regrowth
Neovascular glaucoma
Scleral melting
Enucleation
Compare the effectiveness of enucleation and plaque irradiation for the prevenion of metastatic choroidal melanoma.
Equally effective
Are ciliary body malignant melanomas more or less common compared to choroidal melanomas?
Less common
Are ciliary body melanomas usually detected early or late and via what technique (2)?
Often detected late
-DFE or gonioscopy
What are the symptoms of ciliary body malignant melanoma (3)?
No symptoms
Blurred vision
Pain
List 3 lens anomalies that may be seen with ciliary body malignant melanoma.
Odd astigmatism
Localised cataract
Subluxation
List 7 signs of ciliary body malignant melanoma.
Lens anomalies
Quadrant of dilated episcleral vessels
Subconjunctival pigment
-simulating naevus
IOP changes
Visible extension into the iris and angle
Anterior uveitis (necrosis)
Describe what increased and decreased IOP with ciliary body malignant melanoma can mean.
Increased - extension into the angle, outflow obstruction
Decreased - decreased aqueous production
What is the optometric management for ciliary body malignant melanoma? Compare its prognosis to choroidal malignant melanoma.
Refer any suspicious pigment
Better systemic progress vs choroidal melanoma
List two treatment options for ciliary body malignant melanoma.
Local sectioning (iridocyclectomy)
Enucleation
What is the visibility of an iris malignant melanoma like compared to ciliary body malignant melanoma?
Iris malignant melanoma more visible
What is the average age of detection for iris malignant melanoma?
40 to 50 years
What are the symptoms of iris malignant melanoma (2)?
There may be none
-patient may report a brown spot on their eye
List 5 signs of iris malignant melanoma.
Single pigmented nodule in the lower half of the iris
Invasion of the angle (± glaucoma)
Localised cataract
Pupil distortion
Iris neovascularisation
List three differential diagnoses for iris malignant melanoma.
Iris naevi
Iris freckle
Iris granulomas/cysts
List 4 features of iris naevi.
Flat
Superficial
Avascular
Dark
What is the optometric management for iris malignant melanoma?
Refer any suspicious pigment
List two treatment options for iris malignant melanoma.
Surgical excision (iridectomy or iridotrabeculectomy)
Enucleation
What is the prognosis for iris malignant melanoma with surgical excision?
Good for small, slow growing tumours
What is the most common primary tumour of childhood?
Retinoblastomas
Where do retinoblastomas originate from?
Retinal/glial progenitor cells
Is there a significant risk of morbidity associated with retinoblastomas?
Yes, matastasis via bloodstream
What is the inheritence of retinoblastomas like (2)?
6% autosomal dominant
94% sporadic (no family history)
Are retinoblastomas usually uni- or bilateral? Where else may you see a tumour?
Usually unilateral (60-70%)
May be trilateral with another primary tumour in the pineal gland
What is the average age of onset for retinoblastomas?
2-3 years
List 5 signs of retinoblastoma.
Leukocoria
Strabismus
-important warning sign in children
Uveitis
-secondary to tumour necrosis
Secondary glaucoma causing buphthalmos
Proptosis
-with or without orbital inflammation
List three differential diagnoses for leukocoria aside from retinoblastoma.
Cataract
Retinopathy of prematurity
Persistent foetal vasculature
Describe what the lesion of retinoblastoma looks like including number, elevation, and texture.
White or pink
Elevated
Smooth
Frequently multiple
Describe endophytic and exophyitc and in what context they are used.
Retinoblastomas that grow
-into the vitreous (endophytic)
-into the subretinal space (exophtic)
What does an exophytic retinoblastoma allow for (4)?
Extension into the choroid, optic nerve, meninges, and CNS
List 7 differential diagnoses for retinoblastoma.
Toxoplasmosis
Toxocara
Disciform degeneration (juvenile)
Tuberous sclerosis
Astrocytoma
Other causes of leukocoria
Any other mass lesion
What is the management of retinoblastoma?
Do not risk misdiagnosis
Refer any uncertain cases
Refer all confirmed cases
What is the treatment for retinoblastoma?
Radiotherapy
Photocoagulation/cryotherapy
Enucleation
What is the most common primary orbital tumour of childhood?
Rhabdomyosarcoma
What risk is there with rhabdomyosarcoma?
Risk of spread driectly to the skull from orbit
List 3 signs and symptoms of rhabdomyosarcoma.
Rapid progressive proptosis
Squint
Restriction of gaze
What is a differential diagnosis for rhabdomyosarcoma?
Orbital cellulitis
What is the optometric management for rhabdomyosarcoma?
Refer
What is the treatment for rhabdomyosarcoma (2)? What is there a risk of (4)?
Radiotherapy and chemotherapy
-risk of cataract, radiation retinopathy, corneal opacification, loss of vision
What may be necessary in more advanced cases of rhabdomyosarcoma?
Radical exenteration of orbit
What is an angiomatosis retinae?
Retinal vascular tumour
List 5 signs and symptoms of angiomatosis retinae.
Microaneurysm
Tortuous vessels leading from and to disc
Exudates
Vitreous haemorrhage
Serous retinal detachment
Are tumours of lymphoid tissue common or rare in the eye?
Rare
List 2 signs and symptoms of lymphoid tissue tumours (in the eye).
Vitreous cells
Multifocal intraretinal or subretinal infiltrates
What is the management for lymphoid tissue tumours and is it effective (2)?
Radiotherapy and chemotherapy
-quite effective
What is a choroidal osteoma?
Benign bone-like choroidal tumour
Describe what a choroidal osteoma looks like.
Golden/orange, plate-like lesion of bony nature
What may occur with a choroidal osteoma? What may it disrupt and what is a consequence of this?
May disrupt the RPE and sensory retina causing subretinal neovascularisation
What is the management of chroidal osteoma (2)?
Benign
-may extend under the macula and affect central visiond
Are neurofibromas common or rare?
Very rare
What occurs with a neurofibroma?
The eye develops a melanocytic pigmented hamartoma
