The Clinical Courses of Retinoblastoma Patients Who Underwent Bilateral Enucleation

Sang Beom Han; Jeong Hun Kim; Ho Kyung Choung; Seong Joon Kim; Sang In Khwarg; Young Suk Yu

doi:10.3341/jkos.2007.48.9.1234

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Han, Kim, Choung, Kim, Khwarg, and Yu: The Clinical Courses of Retinoblastoma Patients Who Underwent Bilateral Enucleation

Original Article

J Korean Ophthalmol Soc 2007;48(9):1234-1241.

Published online: 30 August 2007

DOI: https://doi.org/10.3341/jkos.2007.48.9.1234

The Clinical Courses of Retinoblastoma Patients Who Underwent Bilateral Enucleation

Sang Beom Han, M.D.¹, Jeong Hun Kim, M.D.¹, Ho Kyung Choung, M.D.^1,^2,³, Seong Joon Kim, M.D.^1,², Sang In Khwarg, M.D.^1,², Young Suk Yu, M.D.^1,²

¹Department of Ophthalmology, Seoul National University College of Medicine, Seoul, Korea

²Seoul Artificial Eye Center, Seoul National University Hospital Clinical Research Institute, Seoul, Korea

³Department of Ophthalmology, Seoul Metropolitanl Boramae Hospital, Seoul, Korea

Address Reprint requests to Young Suk Yu, M.D., Department of Ophthalmology, Seoul National University, College of Medicine, #28 Yeongeon-dong, Jongno-gu, Seoul 110-744, Korea, Tel: 82-2-2072-3492, Fax: 82-2-747-5130, E-mail: ysyu@snu.ac.kr

Received 20 March 2007 Accepted 19 July 2007

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Purpose

To investigate the courses leading to bilateral enucleation in bilateral retinoblastoma patients.

Methods

Medical records of 5 bilateral retinoblastoma patients who underwent bilateral enucleation were reviewed for patient information, history, change of tumor state during the treatment and the cause of bilateral enucleation.

Results

Out of 48 bilateral retinoblastoma patients, both eyes were saved in 6 children, 1 eye was saved in 37 children, and the remaining 5 children lost both eyes. All patients who underwent bilateral enucleation were female and had no family history of retinoblastoma. At diagnosis, 3 children were 4 months old and the remaining 2 patients were 1 year and 1.5 years old each. Out of 10 eyes, 8 eyes were in Reese Ellsworth group V and the remaining 2 eyes were in group III. The initial treatment was enucleation of 1 eye followed by chemotherapy in 3 patients, and chemotherapy alone in 2 patients. Additional treatment included laser photocoagulation, cryotherapy, external beam radiation therapy and proton beam irradiation. Vitreous seeding, development of new tumors, and increase in tumor size despite of intensive, conservative treatment resulted in second enucleation.

Conclusion

Most patients who underwent bilateral enucleation were 1 year old or younger at diagnosis of retinoblastoma. All patients had a progressed disease status at presentation and showed poor response to intensive, conservative treatment.

Keywords: Retinoblastoma, Enucleation, Bilateral retinoblastoma, Bilateral enucleation

References

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Figure 1.

Orbital CT of case 1 shows calcification in both eyes.

Figure 2.

Fundus photographs of the right eye of case 1. (A): multiple vitreous seedings on the posterior pole and regressed mass at 3 o'clock, (B) large new tumor mass at 2∼3 o'clock, and (C): multiple vitreous seedings in the inferonasal area.

Table 1.

Characteristics of patients at diagnosis

Case	Sex/Age	FHx^∗	R/L	Chief Complaint	Time to Dx^†
1	F/18 m	no	R	Found on examination	2 w ^‡
			L	Conjunctival injection and lid edema
2	F/4 m	no	R	Leucocoria	3 m ^§
			L	Leucocoria
3	F/4 m	no	R	Found on examination	1 w
			L	Leucocoria
4	F/4 m^∏	no	R	Esodeviation	2 w
			L	Esodeviation
5	F/12 m	no	R	Poor fixation	3 mo
			L	Poor fixation

^∗ FHx=family history,

^† Dx=Diagnosis,

^‡ w=week,

^§ m=month,

^∏ =Diagnosed at 4 months, but began treatment at 1 year of age.

Table 2.

Clinical characteristics at the diagnosis

Case	R/L	Mass on funduscopy	R-E^∗	Calcification	Brain MRI	Bone scan	Prim Tx^†
1	R	2 (4∼6DD^‡, 1.5DD) Vit. seeding (+)	Vb	(+)	(−)	(−)	Chemo^§
	L	Full vol. of vit. cavity	Va	(+)			En^∏
2	R	Full vol. of vit. cavity	Va	(+)			En
	L	4 (1∼2DD x 2 (anterior to equator, 4∼5DD, 8DD)	IIIa	(−)	(−)	(−)	Chemo
3	R	Larger than 10DD	IIIb	(+)			Chemo, L^#
	L	1/2 vol. of vit cavity	Va	(+)	(−)	(−)	En
4	R	Full vol. of vit cavity	Va	Not done			Chemo
	L	Full vol. of vit cavity	Va	Not done	(−)	(−)	Chemo
5	R	1/2 vol. of vit cavity	Va	(+)			Chemo
	L	1/2 vol. of vit cavity	Va	(+)	(−)	(−)	Chemo

^∗ R-E=R-E classification,

^† Prim Tx=Primary treatment,

^‡ DD=disc diameter,

^§ Chemo=chemotherapy,

^∏ En=enucleation,

^# L=laser.

Table 3.

Treatment course 1

Case	Chemotherapy (cycles)			Local Tx	RadioTx	Tx response	Outcome
1/R	A^Π	A	B	C^∗ x2	EBR^§ x22	Vit seeding ↑	Enucleation
	12	7	1	L^† x1		New, multiple
				T^‡ x1		tumors
2/L	A			L x1		Vit seeding ↑	Enucleation
	6					New tumors
						Size ↑
3/R	A	B^#	A	C x1		Size ↑	Enucleation
	14	10	4	L x5
4/R	A	A		C x1	Proton	Size ↑	Enucleation
	13	2			beam
4/L	A	A				Size ↑	Enucleation
	13	2				New tumors
5/R	A	B	A		EBR x25	Size ↑	Enucleation
	8	1	10
5/L	A	B	A	C x1	EBR x25	New, multiple	Enucleation
	8	1	10	L x1		tumors

^∗ C=cryotherapy,

^† L=laser,

^‡ T=thermotherapy,

^§ EBR=external beam radiation, Chemotherapy regimen,

^Π A=basic regimen,

^# B=additional regimen.

Table 4.

Treatment course 2

Case	Interval btn^∗ enucleations	Reason of 2nd eye enucleation	F/U^† brain MRI & bone scan	F/U after 2nd enucleation
1	22 m	Vit seeding ↑	(−)^‡	16 m
		Multiple new tumors (+)
2	8 m	Multiple new tumors (+)	(−)	19 m
		Vit seeding ↑
		Size ↑
3	55 m	Size ↑	(−)	29 m
4	14 m	Size ↑	(−)	30 m
5	8 m	Size ↑	(−)	22 m

^∗ btn=between,

^† F/U=follow up,

^‡ (-) : no evidence of metastasis.

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