Clinical results of chemotherapy based treatment in retinoblastoma patients: a single center experience.
OBJECTIVE
Retinoblastoma is the most common intraocular malignancy in children. Since the 1990s, chemotherapy was indicated for intraocular disease to reduce the frequency of enucleation and spare the complications associated with external beam radiation. In this study, we analyzed treatment results of retinoblastoma in our institute.


METHODS
Datas from children diagnosed with retinoblastoma and treated at Seoul National University Children's Hospital between 1986 and 2008 were analyzed retrospectively. We utilized cyclophosphamide, vincristine, adriamycin, and methotrexate (CVAM) for OPD-based adjuvant chemotherapy. From 1990, primary chemotherapy was administered to patients with intraocular disease for eyeball-saving and patients received a combination of etoposide, vincristine, cisplatin (or ifosfamide) as a moderately intensive regimen, or a combination of cisplatin, doxorubicin, etoposide, and cycophosphamide (CDEC) as a highly intensive regimen.


RESULTS
One hundred eighteen children were analyzed. There were 68 unilateral and 50 bilateral diseases. The median age at diagnosis was 1 year and Reese-Ellsworth stage V was the most common stage at the time of diagnosis. All patients were treated by chemotherapy-based multimodality methods, and primary chemotherapy was administered to 80 patients. The 10-year overall and event-free survival rate of all patients were 93.9% and 91.6%, respectively. Two patients who died were in the CDEC regimen group, but there was no significant statistical difference in survival rates by chemotherapy regimens. Fifty-six of 114 eyeballs were saved after primary chemotherapy-based treatment, and the eyeball-saving rate was 49.1%. Six patients relapsed after enucleation and 2 patients were treated successfully after autologous PBSCT. Osteosarcoma occurred in 2 patients as a secondary malignancy, and facial asymmetry after radiotherapy was the most common long-term sequelae.


CONCLUSIONS
In this study, the overall and event-free survival rates of retinoblastoma were satisfactory and eye-saving was possible with primary chemotherapy. Development of new chemotherapeutic regimens and a team approach are necessary to improve the eyeball-saving rate.
Kim
Hyery
H
Division of Hematology/Oncology, Department of Pediatrics, Cancer Research Institute, Seoul National University College of Medicine, Seoul, Korea.
Lee
Ji Won
JW
Kang
Hyoung Jin
HJ
Park
Hyeon Jin
HJ
Kim
Yoon Yi
YY
Shin
Hee Young
HY
Yu
Young Suk
YS
Kim
Il Han
IH
Ahn
Hyo Seop
HS
Oncologist. 2007 Oct;12(10):1237-46
17962617
Cancer Res Treat. 2001 Jun;33(3):191-8
26680785
Bone Marrow Transplant. 2005 Apr;35(8):763-6
15750608
Curr Oncol Rep. 2007 Nov;9(6):453-8
17991352
Pediatr Blood Cancer. 2008 Mar;50(3):567-72
17729249
Ophthalmic Paediatr Genet. 1989 Jun;10(2):111-6
2779981
Bull Soc Ophtalmol Fr. 1976 Jan;76(1):17-22
1000747
Br J Ophthalmol. 1983 Sep;67(9):592-5
6882716
Int J Clin Oncol. 2004 Feb;9(1):1-6
15162819
Br J Ophthalmol. 2003 Nov;87(11):1374-80
14609838
Bone Marrow Transplant. 2008 Sep;42(6):385-91
18574441
Trans Am Acad Ophthalmol Otolaryngol. 1963 Mar-Apr;67:164-72
13973597
Eur J Cancer. 1995;31A(4):572-5
7576971
Australas Radiol. 2003 Sep;47(3):226-30
12890239
Trans Am Ophthalmol Soc. 1969;67:462-534
5381307
Korean J Ophthalmol. 2003 Jun;17(1):35-43
12882506
Curr Opin Ophthalmol. 2002 Oct;13(5):331-6
12218465
J Pediatr Ophthalmol Strabismus. 1994 Jan-Feb;31(1):32-7
8195960
Curr Opin Ophthalmol. 1996 Jun;7(3):20-6
10163455
Br J Ophthalmol. 1988 Aug;72(8):576-83
3415951