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Ophthalmology. 2007 Nov;114(11):2083-9. Epub 2007 Apr 24.

Outcome of patients with retinoblastoma and postlaminar optic nerve invasion.

Author information

1
Department of Hemato-oncology, Hospital J. P. Garrahan, Buenos Aires, Argentina. gchantada@yahoo.com

Abstract

PURPOSE:

To evaluate the outcome of patients with retinoblastoma and postlaminar optic nerve invasion (PLONI).

DESIGN:

Retrospective interventional case series.

PARTICIPANTS:

Sixty-one consecutive patients included in 3 successive protocols were analyzed.

METHODS:

Pathologic review was done in each case. Patients were stratified into 2 risk groups: the high-risk group included those with concomitant full choroidal and/or scleral invasion and were given adjuvant chemotherapy. Those without these features were considered low risk and chemotherapy was withheld after 1994.

MAIN OUTCOME MEASURES:

Extraocular relapse and survival according to stratification.

RESULTS:

The probability of event-free survival (pEFS) was 0.91 and the probability of overall survival (pOS) was 0.94 at 5 years. Patients in the high-risk group (n = 22) had pEFS of 0.86. Three had extraocular relapse (involving the central nervous system; all died of disease). Microscopic scleral invasion was associated to extraocular relapse (P = 0.05). Lower risk patients (n = 39) had a pEFS of 0.94 and pOS of 1. Eighteen received postenucleation chemotherapy and none relapsed. Twenty-one received no adjuvant therapy and 2 had a systemic relapse but were successfully retrieved. Relapsing patients had a higher ratio of affected optic nerve (>25% of it overall length; P = 0.02).

CONCLUSIONS:

Patients with PLONI have an excellent outcome with current therapy. Risk stratification according to the presence of concomitant choroidal and/or scleral invasion may help in the decision of giving adjuvant therapy.

PMID:
17459482
DOI:
10.1016/j.ophtha.2007.01.012
[Indexed for MEDLINE]

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