Long-term evaluation of outcomes and survival of patients treated for retinoblastoma

Long-term evaluation of outcomes and survival of patients treated for retinoblastoma

Original Authors:

Morawski K,

Janosz-Maduzia A,

Haładus T,

Jędrychowska-Jamborska J

Original article date:

April 28, 2020

Plain Language Summary Author(s):

Jeanne McKay,

Helen Dimaras

Plain Language Summary Date:

<PLS Date>

Purpose of the Study

The purpose of this study, by a group in Poland, was to take a retrospective look at 42 patients (58 eyes) who had been treated for retinoblastoma. They described the three goals of treatment as first of all survival of the patient, secondly saving eyes and finally preserving vision. They reviewed the effectiveness of treatment based on survival rate, visual acuity, preservation of the affected eyeball, and tumor reactivation inside and outside of the eye, after initial treatment.

Summary of the Study

The researchers studied outcomes from patients who were diagnosed between 1997 and 2012. All of the patients had been treated at the Department of Ophthalmology and Ocular Oncology at University Hospital Krakow, Poland. The Reese-Ellsworth (RE) Classification for intraocular retinoblastoma, at diagnosis, ranged from I to V. All had received standard chemotherapy drugs vincristine, etoposide and carboplatin (VEC Protocol) as well as other local treatments. Some were bilaterally affected and some unilaterally and there was a mix of males and females.

In information collected from 1997 to 2019, the researchers looked at the types of local treatment the patients received as well as the spread of the disease.

Conclusions from the Study

Early diagnosis and treatment with chemotherapy is very effective in saving lives, eyes and visual acuity. Of the 42 patients, only 1 patient died. The earlier a patient was diagnosed, the more effective chemotherapy was when used with local treatments, there was a much better chance of saving eyes and vision. Even patients with an RE Classification of V had a good survival rate, although eyes were more likely to be enucleated and there was more vision loss. In all cases it was important to have regular examinations and treatment. The one patient who did not survive died because of the spread of disease outside of the eye after initial enucleation. There is no indication that pathology was done after enucleation to check for spread outside the eye and to determine if chemotherapy treatment should be changed.

Personal Comment

This article emphasizes the need for early diagnosis and treatment but also gives hope that even a later diagnosis can save life and vision. The information is not new but it may be the first time the group in Poland has studied their own patients. The RE Classification system for tumors has been replaced by IPC and TNM classification in many centres now. These other classifications take into account more than just the size of the primary tumour.

It is encouraging to know that chemotherapy in conjunction with other local treatments continues to be effective in aiding survival, saving eyes and preserving vision. It also reinforces the need for skilled physicians in understanding the treatment of retinoblastoma.