Home > Studies > OOG Collaborative Study - Uveal Melanoma in Young Patients

OOG Collaborative Study - Uveal Melanoma in Young Patients

October 2013:

https://www.surveymonkey.com/s/OOG_Young_Melanoma

 

Aims of the Study:

 

Many retrospective cohorts of UM in young patients have been reported with variable results and short follow-up, therefore we want to establish a collaborative, retrospective OOG study in order to analyses children diagnosed with choroidal and ciliary body melanoma, whom are younger than 18 years of age, by studying their tumour clinical characteristics, treatment out-come, and prognosis in this age group. We will compare these results with a control group which will includes patients whom their age is between 18-25 years of age.

 

Eligibility Criteria and Data Collection:

Based on discussions in the OOG, this study will enroll two group of patients were diagnosed with choroidal and ciliary body melanoma, excluding all patients with iris melanomas.

 

The first group will include all patients which are children and younger than 18 years of age, according to the definition of The European Medicines Agency and European Union of Pediatric in 2007 that, in the European Union (EU), children are defined as people from birth up to 18 years of age.       

The second group will include patients in the age range above 18 but younger than 25 years old, as this will be a control group for comparison with the first group, both groups, the study group and the control group are expected to be about equal in size.

 

The clinical data should include: Age, gender, tumour characteristics as location, height, largest basal diameter, intra ocular pressure, visual acuity at diagnosis and at last visit, pathology, genetic testing, presence of any predisposing factor as congenital ocular melanocytosis, or neurofibromatosis, treatment type whether surgical or irradiation, and survival status, staging the tumour according to the Tumour, Node, Metastasis classification the 7th Edition, and finally if the patient is alive or dead, beside was the death due to metastasis of UM or due to other cause. Any histopathological material can be re-analyzed and BAP1 status of survival patients determined in a second stage if not already done.

 

Analysis and Reporting:

 

This retrospective observational study will report summary data over all cases, the study aims to collect the data by October 2013, so that initial results can be reported and further plans made in the OOG meeting to be held in Nice, EVER 2013.

 

According to the OOG rules, no reports will be published without prior knowledge of all members who contributed data to the study. The sequence of authors will be as follows:


1- Dr. Rana’a Al-Jamal and Dr. Nathalie Cassoux are the first authors as they are the principal investigators.

2- Participating members will be listed in the order of the number of eligible cases submitted in all abstracts and manuscripts.

 

Update 15.10.2013:

 

 

Initial results and updates were reported at the OOG meeting, which was held in Nice, during the EVER 2013 congress. Fortunately there were new centers have agreed to join this study, therefore It was agreed to extend the deadline for data submission until the end of January 2014. After this deadline, we will complete the data analysis before the Krakow meeting in March 2014, also there will be a discussion during OOG business meeting in Krakow, before starting writing the manuscript.

Rana'a Al-Jamal

 

Instruction:

 

Intent to participate

Once the receipt of your intent to participate have been received you can enter the patient data through this survey website

Upon receiving this study proposal, please e-mail to the study coordinators:

This e-mail address is being protected from spambots. You need JavaScript enabled to view it This e-mail address is being protected from spambots. You need JavaScript enabled to view it This e-mail address is being protected from spambots. You need JavaScript enabled to view it . Also a copy to the OOG president This e-mail address is being protected from spambots. You need JavaScript enabled to view it This e-mail address is being protected from spambots. You need JavaScript enabled to view it

 

1. OOG Member name

2. OOG Member centre

3. Name of any co-investigator assigned to this study

 

 

 

Background

 

Uveal melanoma (UM) is exceptional among young children and adolescents; it is extremely rare in children younger than 10 years of age. Singh et al.[1] reported that out of 8.000 patients with UM only 63 (0.8%) were found in patients who were 20 years of age or younger. Shields et al.[2] reported in their study, which consisted on 3.706 patients of UM, that 40 patients (1.1%) were age 20 years or younger at the time of diagnosis

Tumour characteristics and prognosis of UM among children and young adults are less precisely reported, but have been thought to resemble those adult UM.[1-10] Occasionally young patients have known risk factors for developing UM such as oculodermal melanocytosis, neurofibromatosis type I(NF1), and familial atypical mole-melanoma syndrome. It is not know yet if germline BAP1 mutation is associated with UM among children and adolescents.

 

In preliminary reports by two members of the OOG:

 

1. Of 1,185 patients who were diagnosed with choroidal and ciliary body melanoma and were treated at the Ocular Oncology Service, Helsinki University Central Hospital, Finland, between January 1962 to December 2009, 18 patients (1.5%) were younger than 25 years old, 4 of them were younger than 18, and 14 were between 18-25 years old, median follow-up time was 11.7 years (Al-Jamal & Kivelä, EVER 2011).

 

2. Of 4,857 patients treated for UM at l’ Institut Curie – Paris, from August 1990 to January 2011, 77 patients (1.5%) were younger than 25 years of age, out of them 20 patients were younger than 18, and 57 were between 18-25 years old, median follow-up time was 7.6 years. (CASSOUX N et al, EVER 2011).

 

3. In a meta-analysis review of UM among children and adolescents, five retrospective cohorts[2;9;11-13] from different centres had reported the following results:  

Shields et al.[2] reported 30 patients (13 patients <18 years, 17>18 years), with a median follow-up time of 4 years. Pogrzebielski et al.[12] reported 3 patients (2 patients <18 years, 1>18 years) with a median follow-up of 3.7 years. Leonard et al.[6] reported 14 patients (3 patients <18 years, 11>18 years) with a median follow-up of 1.5 years. Verdaguer et al.[9] reported 9 patients (7 patients <18 years, 2>18 years) with a median follow-up of 5 years, and Vavvas et al.[13] reported 17 patients (10 patients <18 years, 7>18 years) with a median follow-up of 16.2 years.

Most tumours in all reports fell in T2; stage II category, according to the latest TNM classification 7th edition. In our study mortality was higher if the patient was 18 years or older, and if ciliary body was involved. (Al-Jamal & Kivelä, OOG meeting, Ivalo, 2013).

 

 

Reference List

 

       (1)           Singh AD, Shields CL, Shields JA, Sato T. Uveal melanoma in young patients. Arch Ophthalmol 2000;118:918-923.

            (2)       Shields CL, Shields JA, Milite J, De PP, Sabbagh R, Menduke H. Uveal melanoma in teenagers and children. A report of 40 cases. Ophthalmology 1991;98:1662-1666.

            (3)       Barr CC, McLean IW, Zimmerman LE. Uveal melanoma in children and adolescents. Arch Ophthalmol 1981;99:2133-2136.

            (4)       Greven CM, Stanton C, Yeatts RP, Shields CL. Diffuse iris melanoma in a young patient. Arch Ophthalmol 1997;115:682-683.

            (5)       Jensen OA. Malignant melanomas of the human uvea: 25-year follow-up of cases in Denmark, 1943--1952. Acta Ophthalmol (Copenh) 1982;60:161-182.

            (6)       Leonard BC, Shields JA, McDonald PR. Malignant melanomas of the uveal tract in children and young adults. Can J Ophthalmol 1975;10:441-449.

            (7)       Cury D, Lucic H, Irvine AR, Jr. Prepubertal intraocular malignant melanoma. Am J Ophthalmol 1959;47:202-206.

            (8)       Ellsworth RM. Juvenile melanoma of the uvea. Trans Am Acad Ophthalmol Otolaryngol 1960;64:148-149.

            (9)       Verdaguer J, Jr. Prepuberal and puberal melanomas in ophthalmology. Am J Ophthalmol 1965;60:1002-1011.

            (10)     Gailloud C, Zografos L, Bercher L, Uffer S, Egger E. [Uveal melanomas in patients less than 20 years of age]. Klin Monbl Augenheilkd 1992;200:428-430.

            (11)     Leonard BC, Shields JA, McDonald PR. Malignant melanomas of the uveal tract in children and young adults. Can J Ophthalmol 1975;10:441-449.

            (12)     Pogrzebielski A, Orlowska-Heitzman J, Romanowska-Dixon B. Uveal melanoma in young patients. Graefes Arch Clin Exp Ophthalmol 2006;244:1646-1649.

            (13)     Vavvas D, Kim I, Lane AM, Chaglassian A, Mukai S, Gragoudas E. Posterior uveal melanoma in young patients treated with proton beam therapy. Retina 2010;30:1267-1271.