Retinoblastoma patients with high risk ocular pathological features: who needs adjuvant therapy?
OBJECTIVE
To describe the outcome of patients with non-metastatic unilateral retinoblastoma with high risk histopathological features after primary enucleation, and to clarify the need and results of adjuvant therapy.


METHODS
From 1980 to 2001 adjuvant therapy was recommended only to patients with scleral involvement, post-laminar optic nerve involvement (PLONI) with either a positive margin or associated choroidal involvement, or (before 1994) isolated PLONI.


RESULTS
108 of 224 patients had at least one high risk feature (choroidal, scleral, anterior chamber, and/or PLONI). Patients with isolated choroidal (n = 55) or anterior chamber (n = 2) invasion, and most with PLONI without other risk factors (n = 21) were not treated; three relapsed but are long term survivors after intensive therapy. Four with isolated PLONI received adjuvant chemotherapy and none relapsed. Three of 11 with PLONI and concomitant choroidal or scleral involvement who received adjuvant therapy relapsed, versus two of four not treated. Two of five with scleral disease relapsed. All 12 with cut end involvement received adjuvant treatment and none relapsed. In the total group, all four patients who relapsed after adjuvant therapy died.


CONCLUSIONS
Relapsing patients can be rescued with intensive therapy. Those with isolated choroidal or PLONI have a good prognosis without adjuvant therapy. Patients with PLONI with a positive margin have a good prognosis if treated with combined therapy. Those with scleral involvement or PLONI with concomitant choroid disease may benefit from adjuvant therapy.
Chantada
G L
GL
Department of Hematology/Oncology, Hospital JP Garrahan, Combate de los Pozos 1881, C1245AAL Buenos Aires, Argentina. gchantada@garrahan.gov.ar
Dunkel
I J
IJ
de Dávila
M T G
MT
Abramson
D H
DH
D000867:Anterior Chamber; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D002830:Choroid Neoplasms / Q000473:pathology; D003131:Combined Modality Therapy / Q000379:methods; D015353:Eye Enucleation / Q000379:methods*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009361:Neoplasm Invasiveness / Q000473:pathology; D009364:Neoplasm Recurrence, Local; D019574:Optic Nerve Neoplasms / Q000473:pathology; D019572:Retinal Neoplasms / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery*; D012175:Retinoblastoma / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery*; D012189:Retrospective Studies; D012307:Risk Factors; D015422:Scleral Diseases / Q000473:pathology; D016896:Treatment Outcome