DIS - Ocular Tumours - Week 9 Flashcards by B H

List four types of intraocular tumours you may encounter.

Uveal melanoma
-choroid
-ciliary body
-iris
Retinoblastoma
Rhabdomyosarcoma
Angiomatosis retinae

How well did you know this?

Not at all

Perfectly

What is the most common intraocular tumour? In which eye colour is it most common?

Malignant choroidal melanoma
-more prevalent in lightly pigmented eyes

How well did you know this?

Not at all

Perfectly

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

How well did you know this?

Not at all

Perfectly

What is mortality rate due to malignant choroidal melanoma a function of (4)?

Cell type
-spindle A
-spindle B
-mixed
-epitheloid

How well did you know this?

Not at all

Perfectly

Is a choroidal naevus generally considered benign or malignant? Are there any symptoms associated with it?

A benign melanoma
-no symptoms

How well did you know this?

Not at all

Perfectly

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

How well did you know this?

Not at all

Perfectly

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

How well did you know this?

Not at all

Perfectly

Where are choroidal naevi typically located?

At or around the posterior pole

How well did you know this?

Not at all

Perfectly

Do choroidal naevi tend to have associated blood vessels?

How well did you know this?

Not at all

Perfectly

Do choroidal naevi result in visual field defects?

Possible local defect, but subtle

How well did you know this?

Not at all

Perfectly

Do choroidal naevi cause leakage on fluorescein angiography? What do you expect to see?

No leaking
Only masking

How well did you know this?

Not at all

Perfectly

What do you expect of size progression in choroidal naevi over the years?

Stationary in size for long periods, typically forever

How well did you know this?

Not at all

Perfectly

Do most naevi tend to become malignant?

Most never become malignant

How well did you know this?

Not at all

Perfectly

What percentage of malignant melanomas arise from choroidal naevi? What about the rest?

1/3rd come from naevi
2/3rds come from isolated melanocytes

How well did you know this?

Not at all

Perfectly

List four differential diagnoses for a choroidal naevus.

Malignant melanoma
RPE pigment accumulations (RPE hypertrophy)
Other choroidal tumours
Sub-retinal blood

How well did you know this?

Not at all

Perfectly

List 3 characteristics of RPE hypertrophy.

Darker
Distinct margins
Gaps between pigment

How well did you know this?

Not at all

Perfectly

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

How well did you know this?

Not at all

Perfectly

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

How well did you know this?

Not at all

Perfectly

Are changes in choroidal neaevus size normal or is it always sinister?

It can be normal, but refer for caution

How well did you know this?

Not at all

Perfectly

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

How well did you know this?

Not at all

Perfectly

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

How well did you know this?

Not at all

Perfectly

What tinge is often evident on malignant melanomas and what may it indicate?

An orange tinge - lipofuscin

How well did you know this?

Not at all

Perfectly

What shape do malignant melanomas tend to have (4)?

Irregular, patchy, round, and/or elevated

How well did you know this?

Not at all

Perfectly

What may be seen at the edges of malignant melanomas?

Serous detachment

How well did you know this?

Not at all

Perfectly

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?

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