Retinoblastoma

Group

Clinical features

Recommended Treatment options

A

All small tumors (3 mm or less) that are only in the retina and are not near important structures such as the optic disc or the foveolar i.e. Not less than 3 mm from the foveolar and 1.5mmfrom the optic disc.

• Focal therapy (cryotherapy or Transpupillary thermotherapy (TTT).
• TTT is indicated for posterior located tumors. TTT use Diode Laser (810nm) or 1064nm or532nm. Tumor is heated until it turns slightly gray. Complete tumor regression can be
  achieved by using 3-4sessions
• Do not exceed 5 minutes per session to avoid complication such as Cataract.
• Cryotherapy is done to a small tumor at equatorial and peripheral retina. Under GA place the probe precisely on the sclera, directly
  behind the intraocular focus of the tumor. apply triple freeze/thaw at 3week intervals until complete tumor regression.

Note: Administer cryotherapy or TTT 3-6 hours prior to chemotherapy if systemic treatment is indicated. Focal therapy synergistically increases drug penetration to the tumor

B

Tumor of > 3mm, close to theoptic disc or foveola No subretinal fluid

• 6cycles of standard dose chemotherapy are indicated to allow adequate tumor reduction.

Note: Standard dose is a combination of Carboplatin, Vincristine and Etoposide (VEC).Do not perform focal therapy if tumors are located in the macular and juxtapupillary areas.

• If focal therapy is indicated chemotherapy should be given within 6hr of focal therapy to increase drug penetration to the eye and tumor

C

Well-defined tumors with small amounts of subretinal or vitreous seeding (tumor cells floating within the vitreous cavity)

NOTE: Vitreous seeding is one of the most challenging situations for eye-preservation therapy and is the primary reason for treatment failure following conservative management of retinoblastoma.

• 6cycle of standard dose Chemotherapy is indicated.
• Chemotherapy should be given within 6 hours of focal therapy (cryotherapy or TTT or Subtenon Carboplatin) depending on site and response to treatment.
• Chemotherapy cycles are given at 3 weeks intervals, every cycle is preceded by EUA and focal therapy.

Note: Do not perform focal therapy if tumors are located in the macular and juxtapupillary areas

• vitreous seeding can be managed by plaque radiotherapy (need license), External beam Radiotherapy, Enucleation or intravitreal chemotherapy (Melphalan). Melphalan salvage about 60% of eyeball with vitreous seeding (8- 10µg is safe dose)

D

Large or poorly defined tumors occupying up to 50% of the globe with widespread vitreous or subretinal seeding. There is retinal detachment up to 50% of the globe

• If unilateral Enucleate.
• If bilateral Start 3 cycles of chemotherapy
• If no response and no visual potential enucleate the worse eye.
• If there is response complete 6 cycles of systemic chemotherapy.
• Local radiation for persistent vitreous seeds may be indicated (plaque therapy or Intravitreal Melphalan)
• Note: Focal therapy should be given prior to chemotherapy for every cycle.
• Chemotherapy should be given within 6 hours of focal therapy to increase drug penetration to the eye and tumor

E

The tumor is very large with one or more of the following features:

• No visual potential
• Tumor in the anterior segment
• Tumor in or on the ciliary body
• Neovascular glaucoma
• When there is no direct visualization of an active
  tumor (Vitreous hemorrhage, cataract or hyphema)
• Phthisical or pre-phthisical eye
• Orbital cellulitis-like presentation
• Total RD of more than 50%

Enucleation with minimal manipulation is indicated for

• All group E with no visual potential,
• If all known effective treatment has failed

Note: Optic nerve length should not be less than 17mm to minimize the chances of leaving tumor at surgical site. Also, when performing enucleation:

• Take care not to perforate the globe
• Use primary orbital implant to achieve excellent cosmetic appearance
• If histopathology results are positive for high risk factors, 6cycles of Chemotherapy is indicated as for Group D above.

Histopathological features in retinoblastoma are considered high-risk factors (HRF) for tumor progression and metastasis, thus their presence becomes an indication for adjuvant chemotherapy (HRF) includes:

i) Scleral extension

ii) Post-laminar optic nerve invasion

iii) Disease at the cut margin of optic nerve
iv) Massive choroidal invasion of more than 3mm

v) Anterior chamber extension

• If no HRF no need of chemotherapy.
• Prosthesis and protective glasses are indicated for all children who have been enucleated

EOE

Orbital RB

• 3–6 cycles of high dose chemotherapy- followed by enucleation if tumor regressed significantly
• External Beam Radiotherapy and adjuvant chemotherapy for a total of up to 12 cycles

NOTE: Exenteration is not encouraged at this stage

CNS involvement RB- where Ct/MRI proved CNS extension or cerebral-spinal fluid is positive for RB cells

Palliative care according to the National Palliative Care Guideline

Palliative care according to the National Palliative Care Guideline