The clinical characteristics and survival rate of the patients with extraocular retinoblastoma_Chinese Journal of Ocular Fundus Diseases

Authors：

ChengYong , YangFei , ZhaoMin ,  梁建宏 , 黎晓新

Department of Ophthalmology, Peking University People's Hospital, Beijing 100044, China;

Corresponding author：

梁建宏, Email: jianhongliang@hotmail.com

Keywords：

Retinoblastoma/diagnosis; Retinoblastoma/mortality; Neoplasm metastasis; Survival rate

DOI：

10.3760/cma.j.issn.1005-1015.2015.05.009

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Objective To observe the clinical characteristics and survival rate of the patients with extraocular retinoblastoma (RB). Methods This is a retrospective case analysis. From November 2003 to May 2015, 38 eyes of 31 patients with RB in the extra-ocular stage from 213 RB patients were enrolled in this study. There were 18 males and 13 females. Bilateral lesions were observed in 7 patients and unilateral lesions were observed in 24 patients.19 patients were diagnosed at less than 2 years old, 10 patients at 2 to 5 years old, and 2 patients at age over 5 years old. First visit time was less than 1 month in 12 patients, from 1 to 3 months in 15 patients, over 3 months to 6 months in 4 patients. Medical history and family history were record at the first visit. All patients underwent orbital CT, MRI, double color Doppler imaging and wide angle digital retinal imaging system. CT and (or) MRI examination detected tumor extraocular invasion. Histopathological examination showed that there were tumor cells invasion of the scleral, optic nerve root and optic nerve. Chemotherapy was done after surgery. In the extra-ocular stage, 3 to 6 rounds of intensive chemotherapy combined with orbital radiotherapy were done. The average follow-up period was (25.5±4.5) months after treatment. The cumulative survival rate was observed after 6 months, 1 and 5 years after treatment, and the relationship between the initial age, time, sex, single eye, tumor and survival time of the patients was analyzed. Results The extraocular RB accounted 14.55% of all RB patients in this study. There is no family history of RB, no special history. There were 15 patients with leukocoria and yellow-white reflection in the pupil; 5 patients with lacrimation, swelling, photophobia and exophthalmos; 11 patients with strabismus. The cumulative survival rate at 6 months, 1, 5 years after treatment was (78.0±9.0)%, (62.0±11.0)%, (57.0±11.0)% respectively. The average survival time was (53.9±7.8) months; the cumulative survival rate was (59.3±11.3)%. When the age of first visit was less than 1 month, 1-3 months, 3-6 months, the median survival time was 78, 15 and 18 months respectively, the cumulative survival rate was 100.0%, (40.0±21.9)% and (25.0±21.7)%, respectively. The survival time of the newly diagnosed patients at 1 month was more than at 1 to 6 months, and the difference was statistically significant (t=9.20, P < 0.05). Conclusions 14.55% of all RB patients was extraocular RB in this study. One of the most common clinical manifestations is leukocoria at the first visit. The cumulative survival rate of extraocular RB is lower, while the survival rate of patients with the age of first visit time was less than 1 month is higher.

Citation： ChengYong, YangFei, ZhaoMin. The clinical characteristics and survival rate of the patients with extraocular retinoblastoma. Chinese Journal of Ocular Fundus Diseases, 2015, 31(5): 447-450. doi: 10.3760/cma.j.issn.1005-1015.2015.05.009 Copy

1.	Bosaleh A, Sampor C, Solernou V, et al. Outcome of children with retinoblastoma and isolated choroidal invasion[J].Arch Ophthalmol, 2012, 130(6):724-729.
2.	Chantada GL, Qaddoumi I, Canturk S, et al. Strategies to manage retinoblastoma in developing countries[J]. Pediatr Blood Cancer, 2011, 56(3):341-348.
3.	Gobin YP, Dunkel IJ, Marr BP, et al. Combined, sequential intravenous and intra-arterial chemotherapy (bridge chemotherapy) for young infants with retinoblastoma.PLoS One, 2012, 7(9):44322[2012-09-18].http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0044322.
4.	Kumar A, Moulik NR, Mishra RK, et al. Causes, outcome and prevention of abandonment in retinoblastoma in India[J]. Pediatr Blood Cancer, 2013, 60(5):771-775.
5.	Schaiquevich P, Ceciliano A, Millan N, et al. Intra-arterial chemotherapy is more effective than sequential periocular and intravenous chemotherapy as salvage treatment for relapsed retinoblastoma[J]. Pediatr Blood Cancer, 2013, 60(5):766-770.
6.	Temming P, Lohmann D, Bornfeld N, et al. Current concepts for diagnosis and treatment of retinoblastoma in Germany: aiming for safe tumor control and vision preservation[J].Klin Padiatr, 2012, 224(6):339-347.
7.	Araki Y, Matsuyama Y, Kobayashi Y, et al. Secondary neoplasms after retinoblastoma treatment: retrospective cohort study of 754 patients in Japan[J]. Jpn J Clin Oncol, 2011, 41(3):373-379.
8.	Shields CL, Ramasubramanian A, Thangappan A, et al. Chemoreduction for group E retinoblastoma: comparison of chemoreduction alone versus chemoreduction plus low-dose external radiotherapy in 76 eyes[J]. Ophthalmology, 2009, 116(3):544-551.
9.	Shields CL, Manjandavida FP, Arepalli S, et al. Intravitreal melphalan for persistent or recurrent retinoblastoma vitreous seeds: preliminary results[J]. JAMA Ophthalmol, 2014, 132(3):319-325.
10.	Shields CL, Mashayekhi A, Au AK, et al.The International Classification of Retinoblastoma predicts chemoreduction success[J].Ophthalmology, 2006, 113(12): 2276-2280.
11.	Kaneko A, Suzuki S. Eye-preservation treatment of retinoblastoma with vitreous seeding[J]. Jpn J Clin Oncol, 2003, 33(12):601-607.
12.	Sethi S, Pushker N, Kashyap S, et al. Extraocular retinoblastoma in Indian children: clinical, imaging and histopathological features[J]. Int J Ophthalmol, 2013, 6(4):481-486.
13.	Bai S, Ren R, Shi J, et al. Retinoblastoma in the Beijing Tongren Hospital from 1957 to 2006: clinicopathological findings[J]. Br J Ophthalmol, 2011, 95(8):1072-1076.
14.	Lumbroso-Le Rouic L, Aerts I, Lévy-Gabriel C, et al. Conservative treatments of intraocular retinoblastoma[J].Ophthalmology, 2008, 115(8):1405-1410.
15.	Chawla B, Hasan F, Azad R, et al. Clinical presentation and survival of retinoblastoma in Indian children. Br J Ophthalmol, 2015.http://bjo.bmj.com/content/early/2015/06/30/bjophthalmol-2015-306672.long.[published online ahead of print].
16.	Abramson DH. Retinoblastoma in the 20th century: past success and future challenges the Weisenfeld lecture[J]. Invest Ophthalmol Vis Sci, 2005, 46(8):2683-2691.
17.	Gobin YP, Dunkel IJ, Marr BP, et al. Intra-arterial chemotherapy for the management of retinoblastoma: four-year experience[J]. Arch Ophthalmol, 2011, 129(6): 732-737.
18.	Palioura S, Gobin YP, Brodie SE, et al. Ophthalmic artery chemosurgery for the management of retinoblastoma in eyes with extensive (>50%) retinal detachment[J]. Pediatr Blood Cancer, 2012, 59(5):859-864.
19.	Bakhshi S, Meel R, Mohanti BK, et al. Treatment and outcome of nonmetastatic extraocular retinoblastoma with a uniform chemotherapy protocol[J]. J Pediatr Hematol Oncol, 2010, 32(2):42-45.

1. Bosaleh A, Sampor C, Solernou V, et al. Outcome of children with retinoblastoma and isolated choroidal invasion[J].Arch Ophthalmol, 2012, 130(6):724-729.
2. Chantada GL, Qaddoumi I, Canturk S, et al. Strategies to manage retinoblastoma in developing countries[J]. Pediatr Blood Cancer, 2011, 56(3):341-348.
3. Gobin YP, Dunkel IJ, Marr BP, et al. Combined, sequential intravenous and intra-arterial chemotherapy (bridge chemotherapy) for young infants with retinoblastoma.PLoS One, 2012, 7(9):44322[2012-09-18].http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0044322.
4. Kumar A, Moulik NR, Mishra RK, et al. Causes, outcome and prevention of abandonment in retinoblastoma in India[J]. Pediatr Blood Cancer, 2013, 60(5):771-775.
5. Schaiquevich P, Ceciliano A, Millan N, et al. Intra-arterial chemotherapy is more effective than sequential periocular and intravenous chemotherapy as salvage treatment for relapsed retinoblastoma[J]. Pediatr Blood Cancer, 2013, 60(5):766-770.
6. Temming P, Lohmann D, Bornfeld N, et al. Current concepts for diagnosis and treatment of retinoblastoma in Germany: aiming for safe tumor control and vision preservation[J].Klin Padiatr, 2012, 224(6):339-347.
7. Araki Y, Matsuyama Y, Kobayashi Y, et al. Secondary neoplasms after retinoblastoma treatment: retrospective cohort study of 754 patients in Japan[J]. Jpn J Clin Oncol, 2011, 41(3):373-379.
8. Shields CL, Ramasubramanian A, Thangappan A, et al. Chemoreduction for group E retinoblastoma: comparison of chemoreduction alone versus chemoreduction plus low-dose external radiotherapy in 76 eyes[J]. Ophthalmology, 2009, 116(3):544-551.
9. Shields CL, Manjandavida FP, Arepalli S, et al. Intravitreal melphalan for persistent or recurrent retinoblastoma vitreous seeds: preliminary results[J]. JAMA Ophthalmol, 2014, 132(3):319-325.
10. Shields CL, Mashayekhi A, Au AK, et al.The International Classification of Retinoblastoma predicts chemoreduction success[J].Ophthalmology, 2006, 113(12): 2276-2280.
11. Kaneko A, Suzuki S. Eye-preservation treatment of retinoblastoma with vitreous seeding[J]. Jpn J Clin Oncol, 2003, 33(12):601-607.
12. Sethi S, Pushker N, Kashyap S, et al. Extraocular retinoblastoma in Indian children: clinical, imaging and histopathological features[J]. Int J Ophthalmol, 2013, 6(4):481-486.
13. Bai S, Ren R, Shi J, et al. Retinoblastoma in the Beijing Tongren Hospital from 1957 to 2006: clinicopathological findings[J]. Br J Ophthalmol, 2011, 95(8):1072-1076.
14. Lumbroso-Le Rouic L, Aerts I, Lévy-Gabriel C, et al. Conservative treatments of intraocular retinoblastoma[J].Ophthalmology, 2008, 115(8):1405-1410.
15. Chawla B, Hasan F, Azad R, et al. Clinical presentation and survival of retinoblastoma in Indian children. Br J Ophthalmol, 2015.http://bjo.bmj.com/content/early/2015/06/30/bjophthalmol-2015-306672.long.[published online ahead of print].
16. Abramson DH. Retinoblastoma in the 20th century: past success and future challenges the Weisenfeld lecture[J]. Invest Ophthalmol Vis Sci, 2005, 46(8):2683-2691.
17. Gobin YP, Dunkel IJ, Marr BP, et al. Intra-arterial chemotherapy for the management of retinoblastoma: four-year experience[J]. Arch Ophthalmol, 2011, 129(6): 732-737.
18. Palioura S, Gobin YP, Brodie SE, et al. Ophthalmic artery chemosurgery for the management of retinoblastoma in eyes with extensive (>50%) retinal detachment[J]. Pediatr Blood Cancer, 2012, 59(5):859-864.
19. Bakhshi S, Meel R, Mohanti BK, et al. Treatment and outcome of nonmetastatic extraocular retinoblastoma with a uniform chemotherapy protocol[J]. J Pediatr Hematol Oncol, 2010, 32(2):42-45.

Chinese Journal of Ocular Fundus Diseases

The clinical characteristics and survival rate of the patients with extraocular retinoblastoma

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