220                                                                                                                             Retinoblastoma

44. Retinoblastoma

General considerations

There has been a trend away from enucleation (removal of the eye) and external beam radiation therapy towards focal treatment. Enucleation is now generally restricted to retinoblastoma that fills most of the eye and when there is concern for tumor invasion into the optic nerve or chorioid. External beam radiotherapy remains a treatment option for those less advanced retinoblastomas with diffuse vitreous or subretinal seeding.

Small- to medium-sized tumors can be controlled with focal plaque radiotherapy.

Cyotherapy, photocoagulation and more recently thermotherapy are used for selected small tumors with excellent results.

Chemoreduction is often employed to reduce the tumor volume to an extent that allows for focal treatment (cryotherapy, thermotherapy, plaque radiotherapy) of tumor residues. Other clinical settings in which chemotherapy is considered are a high risk for or presence of metastatic disease and extraocular extension of the tumor.

Literature: for review

FINGER et al, Drugs 58 (1999): 983-996 (role of chemotherapy) SHIELDS and SHIELDS , J. Pediatr. Ophthalmol. Strabismus 36 (1999):8– 18

SINGER, J. Ophthalmic Nurs. Technol. 19 (2000): 148 – 149 ZUCKER et al, Eur. J. Cancer 34 (1998): 1045 – 1049

44.2 CADO

Cyclophosphamide 20 – 40 mg/kg * i.v. ( 1 h inf) d 1

Doxorubicin 0.67 mg/kg i.v. ( 1 h inf) d 1 – 3 **

Vincristine 0.05 mg/kg i.v. (bolus) d 1

* week 0 = 40 mg/kg

week 3, 6, 9, 12, 15, 18, 21 = 20 mg/kg

weeks 24, 27, 30, 33, 36, 39, 42, 45, 48, 51, 54, 57 = 30 mg/kg

** Stopped after week 21

Literature:

SCHVARTZMAN et al, J. Clin. Oncol. 14 (1996): 1532 – 1536

44.3 Etoposide+ carboplatin

Etoposide 100 mg/m2 i.v. ( 1 h inf) d 1-5

Carboplatin 160 mg/m2 i.v. ( 1 h inf) d 1-5

To be repeated every 3 – 4 weeks

Literature:

DOZ et al, J. Clin. Oncol. 13 (1995): 902 – 909

or

Etoposide 150 mg/m2* i.v. ( 1 h inf) d 1-3

Carboplatin 200 mg/m2 i.v. ( 1 h inf) d 1-3

To be repeated every 3 – 4 intervals

* In children younger than one year or weighing less than 10 kg , The doses

were adapted to weight (5 mg / kg for etoposide and 6.7 mg/kg for carboplatin

Retinoblastoma                                                                                                                             221

Literature:

NENADOV BECK et al, J. Clin. Oncol. 18 (2000): 2881 – 2887 (first-line chemotherapy with local treatment to prevent external- beam irradiation and enucleation in low-stage intraocular disease)

44.4 VEC

Carboplatin 560 mg/m2 * i.v. ( 1 h inf) d 1

Etoposide 150 mg/m2* i.v. ( 1 h inf) d 1+2

Vincristine 1.5 mg/m2* i.v. (bolus) d 1

(max 2 mg)

To be repeated every 4 weeks ( 6 cycles )

For patients ≤ 36 months of age 18.6 mg/kg , 5 mg/kg , and 0.05 mg/kg ,

respectively

Literature:

FRIEDMAN et al, J. Clin. Oncol. 18 (2000): 12 – 17 (chemoreduction and local ophthalmic therapy to avoid enucleation and external-beam radiation therapy)

44.5 Cyclophosphamide + vincrestine

Cyclophosphamide 300 mg/m2 i.v. wk x 6, thereafter

200 mg/m2 i.v. wk for one year

Vincristine 1.5 mg/m2 i.v. wk x 6, thereafter

1.0 mg/m2 i.v. wk for one year

Literature:

WHITE, Am. J. Ped. Hematol. Oncol. 13 (1991): 189 – 201