Cbg. Antoneli et al., TREATMENT OF EXTRAOCULAR RETINOBLASTOMA IN BRAZIL, International journal of pediatric hematology/oncology, 2(3), 1995, pp. 217-221
Between January 1986 and December 1990, 120 patients with retinoblasto
ma were admitted at A. C. Camargo Hospital. Among these, 32 (26.6%) we
re extraocular, (24 unilateral and 8 bilateral). The hereditary type w
as present in 9 patients and sporadic in 23. Fifteen patients had opti
cal nerve involvement, 5 scleral vessel and 9 orbital. The cerebrospin
al fluid was positive in 1, and bone marrow was positive in 2 patients
. The mean time before referral was 6 months. Chemotherapy was adminis
tered to all cases after enucleation or eyelid biopsy. This comprised
cisplatin, etoposide, cyclophosphamide, vincristine and adryamicin, an
d intrathecal methotrexate. External irradiation (45 Gy) was initiated
after surgery in patients with optical nerve involvement and in patie
nts with measurable disease after 3 cycles of chemotherapy. Twenty-six
to 32 were evaluated for response. The exclusions criteria were patie
nts that refused treatment, or patients who received one cycle of chem
otherapy and were lost to follow-up, making the evaluation for respons
e impossible to be assess. Among them, 8 are free of disease and off t
reatment, with median follow-up of 14.6 months; 1 is alive with locali
zed disease, and 3 are lost to follow-up. Fourteen patients died of di
sease; the median time of relapse was 6 months (range 1 to 13 months).
We conclude that whereas the current approach yields results similar
to others, more aggressive combined chemotherapy is necessary for the
improvement in the treatment of patients with advanced retinoblastoma.
Late referral may be responsible for the high incidence of advanced c
ases.