Uvea: Uveal Tumours

Question 1

Describe iris lesions/tumours?

Answer 1

• Iris naevus
• Pigmented, usually flat iris lesion
• Can be seen anywhere on iris
• 15% risk of malignant transformation – should be monitored
• Pupil architecture may be affected

Question 2

Describe iris nodules?

Answer 2

• Can be present in inflammatory conditions and systemic disease
• Usually benign, don’t usually require tx and tx is normally of underlying condition
• Fuchs Hetereocromic Cyclitis (FHC) – Busacca and Koeppe nodules
• Lisch nodules – Neurofibromatosis
• Brushfield spots – Down’s syndrome

Question 3

Describe iris melanoma?

Answer 3

• Similar appearance to iris naevus
• Can distort iris architecture and pupil margin
• May invade into angle
• Will grow gradually
• Risk factors:
  o Fair skin/iris colour
  o Iris naevi
  o Numerous cutaneous naevi – lots of freckles on skin
  o Sunlight exposure
• Any growth of a lesion is suspicious and needs referred URGENTLY for suspicion of cancer

Question 4

Describe ciliary body melanoma?

Answer 4

• Difficult to diagnose
• Often present late with visual symptoms – by time got visual symptoms it’s quite late
• When starts to increase, can press on lens and iris – when distorts lens & iris, the vision itself can become distorted
• Risk factors – as for iris melanoma
• Treatment:
  o Radiotherapy
  o Enucleation
  o Chemotherapy if metastasis (usually liver)
• Need to be extremely vigilant
• Sentinal vessel – BV that is supplying the tumour – as the tumour increases in size, it causes pressure on the vessel  vessel itself becomes very engorged
• Can get thick sausage shaped BVs as a sign
• Top picture – tumour invading/ covering lens  this is massive tumour & really too late

Question 5

List the types of choroidal tumours?

Answer 5

• Choroidal Naevus
• Choroidal Melanoma
• Choroidal Haemangioma (circumscribed & diffuse)
• Choroidal Osteoma
• Choroidal metastasis of other cancers

Question 6

Describe choroidal naevus including signs & treatment?

Answer 6

• Present in 5-10% of population
• Incidental finding – not really any symptoms
• Grow during childhood but not adulthood
• Any change is suspicious
• Usually flat – up to 2mm is benign
• Must monitor life long for conversion
• Signs:
  o Pigmented retinal lesion with indistinct margins
  o Over lying drusen
  o Flat (<2mm in height)
  o Depigmented halo – area of almost pallor around it)
  o Suspicious signs of it becoming melanoma:
   Lipofuscin (orange pigment on surface)
   Any Sub-Retinal Fluid (SRF)
   Any symptoms – v suspicious there is something else going on
   Within 3mm of optic disc
   >2mm height and 5mm diameter
• Treatment:
  o Monitoring with serial photography
   Ideally give the px a picture of what the naevus looks like
  o 6 monthly initially then annually
  o If any concerns then refer to HES
  o Patients referred back to community need lifelong monitoring (through photos at their sight tests)

Question 7

Describe choroidal melanoma including signs & management?

Answer 7

• Commonest intraocular malignancy but still rare
• Risk factors as for iris melanoma:
  o Fair skin
  o Fair iris colour
  o Sun exposure
  o Family hx
• Suspicious signs:
  o Lipofuscin (orange pigment on surface)
  o Any SRF
  o Any symptoms
  o Within 3mm of optic disc
  o >2mm height and 5mm diameter
• Usually asymptomatic, incidental finding
• Metastasis can occur to liver
  o Mortality is 50% at 10 years
  o Even if remove eye the risk of metastasis does not go away
• Signs:
  o Solitary elevated (dome shaped) pigmented mass
  o “collar stud” appearance – base of lesion is narrower than top of lesion
  o Lipofuscin – orange pigment overlying the lesion
  o Localised retinal detachment
  o Vitiris (advanced) – usually because can get a breakthrough of cancer cells from the lesion into the vitreous & generates an inflammatory response and can get vitritis
  o Sentinal vessels, rubeosis, choroidal folds – due to congestion that the mass causes on the venous outflow of blood
• Management:
  o URGENT referral

Question 8

What does early detection in uveal tumours lead to?

Answer 8

Early detection may allow px to keep their eye
Early detection may allow better visual outcome
Symptoms suggest advanced disease & poor prognosis
Always dilate all patients