Early Diagnosis and Management of Retinoblastoma

Retinoblastoma is a rare tumour: in France, 1 case per 16,000 births or 5 new cases per million children aged 0 to 14 years or 50 cases/year. In sub-Saharan Africa, at least 1,000 cases/year occur annually among 400 million children under 15 years of age.

 

About 40% of retinoblastomas are heritable (transmissible), often reaching both eyes. The average age at diagnosis in France is 2 years for unilateral forms and 1 year for bilateral forms. Diagnosis is often later in developing countries.

 

Definitive cure is achieved in more than 98% of cases in France (centralization of treatments at the Institut Curie, Paris) with most often useful vision maintained, and less than 40% in many low-income countries with a high percentage of blind children among survivors, due to late diagnosis and reduced access to specialized care with dispersed initial care.

 

However, the earlier the diagnosis is made, the sooner the child is cared for in a referral centre, the simpler the treatment, the higher the cure rate and the lower the sequelae due to the tumour and the treatment and the lower the cost of care.

Retinoblastoma is therefore exemplary in many respects: a long local, spectacular evolution, exceptionally high healing possibilities if diagnosis is made early and if access to relatively simple diagnostic and therapeutic means is ensured.

 

AMCC has developed with the teams in Bamako, Mali, a programme to support early diagnosis, access to treatment, including conservative treatment, and rehabilitation of children with retinoblastoma.

 

This program, developed in partnership with the Franco-African Group of Pediatric Oncology, with the support of the Sanofi Espoir Foundation (My Child Matters program), Retinostop, Institut Curie, included training for ophthalmologists (particularly for conservative treatments of at least one eye in bilateral forms), ocularists (for the preparation of prostheses after enucleation), pathologists and assistance in the development of communication tools for early diagnosis.

 

This programme, initiated in Bamako in 2011, was adapted in Lubumbashi (Democratic Republic of Congo) in 2012, Dakar (Senegal) and Abidjan (Ivory Coast) in 2013 and Antananarivo in 2014.

 

It has been demonstrated in Bamako that healing can be achieved in more than 80% of early forms with a locally manufactured prosthesis and that conservative treatments can be successfully implemented in a trained multidisciplinary team.

 

On the basis of these encouraging initial results, a 2019-2028 programme has been established to extend support to all French-speaking sub-Saharan countries and to some English-speaking countries (a priori concerning about 1,000 new cases per a of retinoblastoma), through assistance in the creation of reference centres in sub-Saharan Africa. The centres with the highest recruitment and conservative treatment can soon be certified to form other African teams and will be the backbone of a retinoblastoma network in sub-Saharan Africa.

 

Pierre BEY, Laurence DESJARDINS, Pascal SIRIGNANO