Ocular tumour part 1 and 2

Question 1

What does the term tumour mean ?

Answer 1

abnormal swelling

-any mass of tissue formed by new cells or swelling due to inflammation

Question 2

What does neoplasm mean?

Answer 2

‘new growth’

Question 3

What is a neoplasm (real definition)?

Answer 3

An abnormal tissue that grows by cellular proliferation more rapidly than normal and continues to grow after the stimuli that initiated the new growth cease. Neoplasms show partial or complete lack of structural organization and [lack] functional coordination with the normal tissue”

-May be malignant or benign

Question 4

What does benign mean?

Answer 4

lacks the ability to invade neighbouring tissue or metastasize
self limiting e.g mole
- does not spread

Question 5

What does malignant mean?

Answer 5

• Anaplasia (poor cellular differentiation, lack specialisation of mature cells),
• Invasive with capacity for metastasis e.g. melanoma (invades local tissues and grows)
• capacity to spread throughout the body

Question 6

What is the differential diagnosis on basis of a benign and malignant tumour ?

Answer 6

Anaplasia
Rate of Growth
Local Invasion
Metastasis

Question 7

What does the term cancer refer to ?

Answer 7

a malignant neoplasm

Question 8

How do we know tumours are malignant (cancerous) ?

Answer 8

have the prefix ‘car’ at the start or middle

Question 9

What is melanosis ?

Answer 9

a melanoma
which is increased melanin pigmentation
it is the most aggressive form of skin canceras it can:

Grow and spread rapidly
Can develop from normal looking skin, or in a mole
Can grow anywhere in the body - not just areas exposed to the sun
If treated early 95% of melanomas can be cured

Question 10

How can we detect between a benign and malignant melanoma?

Answer 10

ABCDE
Asymmetry
Border
Colour
Diameter
Evolution

Question 11

What is a superficial spreading melanoma?

Answer 11

Characterized by an irregular outline and variable pigmentation.
Often stays in situ for months/years and grows horizontally on skin surface.

Question 12

What is a Nodular melanoma ?

Answer 12

A lump that has been rapidly enlarging

Malignant cells proliferate downwards (vertical growth).

Question 13

What is a Squamous Cell Papilloma?

Answer 13

Common benign epithelial tumour (skin tag)

Narrow or broad based or pedunculated

Question 14

What is a basal cell carcinoma?

Answer 14

Most common type of skin cancer - 75% 
Incidence 0.25% population/year – 3-4x higher in Australia
More prevalent in elderly
90% of BCC affect head and neck – 9% affect the eyelid.
90% of eye lid tumours are caused by BCC
Locally invasive
Slow growing 
Non metastasizing

Question 15

What do basal cell carcinoma appear as?

Answer 15

Usually small, round or flattened spots that are red, pale or pearly in colour
Can bescaly like a patch of eczema
May look like an ulcer or sore that doesn’t heal

Question 16

What are the clinical features of BCC?
signs
features

Answer 16

Signs:
Ulceration
Lack of tenderness
Induration – Hardening of normally soft tissue
Irregular borders
Destruction of lid margin architecture

Features/types
Nodular
Noduloulcerative
Sclerosing

Question 17

What does Nodular BCC look like?

Answer 17

Shiny, firm, pearly nodule with small dilated blood vessels on its surface
Growth is slow and it may take the tumour 1–2 years to reach a diameter of 0.5 cm.

Question 18

What does Noduloulcerative BCC (rodent ulcer) look like?

Answer 18

-Central ulceration with pearly rolled edges and dilated blood vessels at the edges (telangectasia).
With time it may erode a large portion of the eyelid.

Question 19

What does Sclerosing BCC look like?

Answer 19

Infiltrates laterally beneath the epidermis as a hardened plaque
More extensive on palpation than inspection.
Could be mistaken for a localized area of chronic blepharitis

Question 20

What is Squamous Cell Carcinoma?

Answer 20

Less common than BCC - 5-10% of eyelid tumours
More aggressive (20% metastasise)
Prefers lower lid
Increased risk if immunocompromised
More common in older people with fair complexion and history of chronic sun exposure

Question 21

What is the difference between BCC and SCC?

Answer 21

It may resemble a BCC but:

• Surface vascularization is usually absent.
• Growth is more rapid
• Hyperkeratosis is more often present (excess development of keratin)- IMPORTANT SIGN OF SCC

Question 22

What are the features of SCC?

Answer 22

Nodular SCC
Ulcerating SCC
Cutaneous horn

Question 23

What does the Nodular SCC look like?

Answer 23

Characterized by a hyperkeratotic nodule(nodule with excess keratin in it) which may develop crusting erosions and fissures

Question 24

What does the Ulcerating SCC look ike?

Answer 24

• Has a red base and sharply defined, indurated (hardened) and everted borders
• has Pearl margins and telangiectasia are not usually present (unlike noduloulcerative BCC).

Question 25

What is  Cutaneous horn ?

Answer 25

Keratinic projection. | Cutaneous horns are associated with other benign and malignant conditions too. - not just SCC

Question 26

What is Kaposi Sarcoma?

Answer 26

Rare cancer caused by human herpes virus 8 (HHV-8) Mainly seen in immunosuppressed patients (especially HIV) – very fast progressing Also seen in middle aged men of Mediterranean or Ashkenazi Jewish descent (v slow progressing)

Question 27

What is the treatment for EYE LID MALIGNANT TUMOURS ?

Answer 27

``` Biopsy Surgical Excision Reconstruction Radiotherapy For melanoma and SCC general oncological examination and biopsy on lymph nodes in neck to check for metastases. ```

Question 28

What is Conjunctival naevus?

Answer 28

- Most common conjunctival melanocytic tumour - Benign - Risk of becoming malignant – 1% - Usually noticed in 1st or 2nd decade

Question 29

What are signs of Conjunctival naevus?

Answer 29

Solitary, discrete moderately elevated pigmented or partially pigmented lesion Most frequently near limbus Mobile over the sclera Grey brown or black in most cases

Question 30

What is the risk of a conjunctival nevus of becoming malignant increased ?

Answer 30

- Rapid growth or increase in pigmentation - Develop after 2nd decade - Prominent feeder vessel

Question 31

What is a conjunctival melanoma?

Answer 31

rare when occurred it is invasive of stroma and nerves -spreads within the epithelium

Question 32

what is conjunctival squamous cell carcinoma ?

Answer 32

Squamous cell carcinoma with overlying (white) keratinization in the nasal bulbar conjunctiva

Question 33

What is an iris nevus ?

Answer 33

a benign tumour Diameter <3-4mm; thickness <2mm Minimal or no visible vascularity Inferior location in most cases The normal iris architecture is disrupted There may be mild distortion of the pupil A freckle is smaller and does not distort the iris architecture

Question 34

What does an iris freckle look like ?

Answer 34

Flat, brown pigmentation on iris surface, with irregular, feathery edges and not distorting the normal architecture

Question 35

What are some signs of iris nevus ?

Answer 35

can be elevated (show of blurry signs) | ectropion uvae- turning out of iris epithelium

Question 36

What is an iris melanocytoma ?

Answer 36

another type of naevus benign Darkly pigmented nodular mass , mossy granular surface

Question 37

What is an iris melanoma?

Answer 37

8% of uveal melanomas arise in iris 3x more common in blue/grey eyed people Features Pigmented or non pigmented nodule >3mm diameter and > 1mm thick Usually surface blood vessels Pupil distortion/ ectropion uveae May infiltrate the angle and ciliary body Can cause cataract and secondary glaucoma

Question 38

What are iris tumours treatment ?

Answer 38

Sector iridectomy Radiotherapy with radioactive plaque (brachytherapy) External irradiation with proton beam Enucleation for diffusely growing tumours Check for metastases

Question 39

What is a choroidal nevus ?

Answer 39

- Present in 5-10% white Caucasians - Very rare in darker skinned individuals - If growth in adulthood then suspect malignancy - Usually asymptomatic unless at macula and then can cause distortion

Question 40

What are the signs of choroidal nevus ?

Answer 40

Brown to slate grey May contain surface drusen Oval or circular Indistinct margins Diameter usually <3mm (2DD) wide and < 1mm thick Depigmented halo is common Secondary choroidal neovascularisation is rare

Question 41

What is choroidal melanoma prevalence?

Answer 41

Most common intraocular malignancy in adults | 80% of all uveal melanoma; peaks at 60yrs

Question 42

what are the signs of the choroidal melanoma ?

Answer 42

Solitary elevated subretinal grey-brown or amelanotic (colourless) dome shaped mass 60% are within 3mm of the optic disc. Can be larger than naevi (>2DD) May have surface orange lipofuscin

Question 43

What are the symptoms of chorodial melanoma ?

Answer 43

Blurred vision; Visual Field Loss | Floaters; Photopsia

Question 44

What can you see in a Amelanotic Melanoma?

Answer 44

Colourless, dome shaped elevation, close to disc

Question 45

What does a Lipofuscin + Melanoma fundus image look like ?

Answer 45

Elevated, >2DD, light grey, close to disc, orange lipofuscin present

Question 46

What does a Lipofuscin + Melanoma fundus image look like ?

Answer 46

Elevated, >2DD, light grey, close to disc, orange lipofuscin present

Question 47

What can melanomas do to the retina?

Answer 47

perforate the retina and through into the vitreous chamber

Question 48

What features are indicative of a choroidal melanoma ?

Answer 48

``` Thickness greater than 2 mm, (To) Fluid (subretinal) (Find) Symptoms, (Small) Orange pigment (lipofuscin) (Ocular) Margin near disc (Melanoma) Ultrasonographic hollowness (Using) Halo absence (Hints) Drusen absence ```

Question 49

How do we investigate these lesions ?

Answer 49

Photograph- colour fundus photograph OCT – useful to measure lesion thickness Fundus autofluorescence (lipofuscin pigment fluoresces) Ultrasound Baseline photography – reviewed regularly for signs of change, growth

Question 50

What are some treatment for the choroidal melanoma ?

Answer 50

Brachytherapy (episcleral plaque radiotherapy) Plaque is sutured to sclera for several days Charged particle therapy High energy charged protons ionising radiation to tumour. Used for smaller tumours Trans Pupillary Thermotherapy (TTT) IR laser to heat tumour – death by hyperthermia Cryotherapy – has been used in past but not widely used now Enucleation Effective and indicated if tumour invades optic nerve

Question 51

What are metastatic tumours ?

Answer 51

Choroid is the most common site for uveal metastases Common primary sites are breast and bronchus Features Fast growing yellow-ish lesion Multifocal and bilateral Large size

Question 52

What is a benign tumour of the RPE ?

Answer 52

Congenital Hypertrophy of RPE (CHRPE) | -THEY ARE Asymptomatic

Question 53

What are the signs of CHRPE ?

Answer 53

Solitary – dark grey or black lesion with well defined margins Multifocal - smaller lesions – “bear-track” Atypical CHRPE – multiple lesions of variable size with hypopigmented margins (associated with gastrointestinal malignancy/colon cancer)