Non-Melanocytic Tumors Flashcards
T/F: Retinoblastoma is confined to retina
FALSE: can also invade ON or choroid
Pathophysiology of Retinoblastoma
Believed to be undifferentiated rod/cones that metastasize
Color of retinoblastoma
Starts translucent gray/white but as it grows and calcifies, it gains “chalky white” appearance
Most common malignant intraocular tumor in children
Retinoblastoma
T/F: retinoblastoma is generally sporadic
TRUE
In developed countries, retinoblastoma is typically found before age
4 (avg: 18 months)
Most frequent sites of metastasis in children
Skull
Distal Bones
Brain
Spinal Cord
Lungs
Lymph Nodes
Primary workup for Retinoblastoma
FA + B-scan
Main growth patterns of Retinoblastomas
- Intraretinal —> homogenous, dome-shaped white tumor, eventually irregular w/ calcific flecks
- Endophytic —> grows into vitreous cavity and invades inner retina layers as white mass w/ “seeding” of tumor cells
3 Exophytic —> grows into direction of subretinal space as bi-lobed white mass —> can cause RD
What must be ruled out in all cases of childhood uveitis/ocular inflammation?
Retinoblastoma!
Under what conditions is there an increased risk of metastasis of Retinoblastoma?
- If tumor is advanced/large “too big”
- Choroidal/Retrolaminar ON invasion “too back”
- AS involvement “too front”
- Orbital spread “too far”
- Repeated recurrence after conservative treatments ‘too many times”
Uveal layer most common for metastasis? Why?
Choroid; rich blood supply
T/F: diagnosis of choroidal metastasis requires immediate treatment
TRUE
T/F: Retinoblastoma is bilateral, multifocal, and has ON involvement.
Actually false
Only 20% bilateral, 20% MF, and 20% ON involvement
Describe the appearance of choroidal metastasis
Indistinct margins
Elevated mildly —> Flat
Yellow/creamy
Subretinal
What ocular findings if other uveal tissues affected by Choroidal Metastasis? (4)
- Pseudohypopyon (tumor cells, not WBCs)
- Iridocyclitis (inflammatory cells)
- Secondary Angle Closure GLC
- Rubeosis iridium —> hyphema, NVG
Diagnostic tool of choice for Choroidal Metastasis? Why?
Ultrasonography; tells size, shape, and excavation
Results of Ultrasonography w/ Choroidal Metastasis?
A scan — moderate/high internal acoustic reflectivity
B scan — irregular placoid lesion and associated serous RD
Symptoms of CHoroidal Metastasis
- Decreased vision
- VF defects
- Elevated IOP
- Photopsia (flashes)
- Diplopia
but may be asymptomatic
Uveal metastasis implies
Poor prognosis (8-12 months to live)*
- actual survival rate dependent on cancer type
For what diagnosis might Brachytherapy be appropriate?
Retinoblastoma or Choroidal Metastasis
Describe course if Choroidal Metastasis breaks Bruch’s Membrane
Develops mushroom shape
Subretinal fluid invades
Possible serous SD and RPE atrophy
Can cause VF defects and vision loss
T/F: Choroidal Osteoma is a fast-growing benign tumor
Benign, but slow-growing
Who is most susceptible to Choroidal Osteoma?
Females in 2nd-3rd decade of life
T/F: Choroidal Osteoma typically unilateral
TRUE; 75% unilateral
If bilateral, usually not simultaneous
Appearance of Choroidal Osteoma
Distinct margins
Yellow-white
Calcified
Flat —> minimally elevated
What is commonly secondary to Choroidal Osteoma?
choroidal neovascularization is very common
Longstanding Choroidal Osteoma can result in…
RPE atrophy/hyperplasia
What is Choroidal Osteoma?
Deposits forming bony trabeculae in between choroid and choriocapillaris
Choroidal Osteoma results in (rapid/gradual) vision loss
Gradual
T/F: regression in Choroidal Osteoma is possible
Actually true
Management of Choroidal Osteoma
Every 6 months:
DFE, OCT/OCTA
+ occasional A/B-scan
T/F: Vision Loss associated with Choroidal Osteoma is usually treatable
FALSE
Location of Choroidal Osteoma
Posterior Pole / Juxtapapillary
Choroidal Osteoma CT scan shows
Dense plaque like opacity at choroidal level
Choroidal Osteoma Ultrasonography shows
Highly reflective localized area w/ orbital shadowing
Choroidal Osteoma FA shows
early irregular diffuse mottled hyperfluorescence and late staining of the tumor
