Clinical Features and Management Outcome of Optic Nerve Sheath Meningioma in Korea

Eun Kyoung Lee; Seong-Joon Kim; Sun Ha Paek; Dong Gyu Kim; Il Han Kim; Sang In Khwarg

doi:10.3341/jkos.2011.52.1.74

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Lee, Kim, Paek, Kim, Kim, and Khwarg: Clinical Features and Management Outcome of Optic Nerve Sheath Meningioma in Korea

Original Article

J Korean Ophthalmol Soc 2011;52(1):74-85.

Published online: 7 September 2011

DOI: https://doi.org/10.3341/jkos.2011.52.1.74

Clinical Features and Management Outcome of Optic Nerve Sheath Meningioma in Korea

Eun Kyoung Lee, MD^1,², Seong-Joon Kim, MD, PhD^1,², Sun Ha Paek, MD, PhD³, Dong Gyu Kim, MD, PhD³, Il Han Kim, MD, PhD⁴, Sang In Khwarg, MD, PhD^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 Neurosurgery, Seoul National University College of Medicine, Seoul, Korea

⁴Department of Radiation Oncology, Seoul National University College of Medicine, Seoul, Korea

Address reprint requests to Seong-Joon Kim, MD, PhD Department of Ophthalmology, Seoul National University Hospital #28 Yongon-dong, Chongno-gu, Seoul 110-744, Korea Tel: 82-2-2072-2438, Fax: 82-2-741-3187, E-mail: ophjun@snu.ac.kr

Received 12 June 2010 Revised 15 September 2010 Accepted 12 November 2010

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 evaluate the clinical features and treatment results of optic nerve sheath meningioma (ONSM) in Korean patients.

Methods

The present retrospective noncomparative case series was comprised of 10 eyes of 10 patients with a diagnosis of ONSM who were treated between 1997 and 2010 at the Seoul National University Hospital.

Results

The mean age at presentation was 47.9 years. Two males and 8 females participated in the study. Decreased vision and proptosis were the most common presenting symptoms. On imaging, the most common pattern was tubular. The most common histopathologic pattern was a meningothelial type. Two patients who were observed maintained good vision during the follow-up period and showed a slow progression of the tumor. Two patients out of 3 who underwent surgical management presented significant visual loss and complications. Three patients in the gamma-knife surgery (GKS) group showed no significant changes in visual acuity after treatment; however, tumor growth was halted. Two patients who underwent 3-dimensional conformal radiotherapy (3D-CRT) presented improvement in their visual acuity or visual field, and tumor growth was halted.

Conclusions

Management should be conservative in most cases because of the slow and indolent growth pattern of ONSM. However, 3D-CRT can be considered as an initial treatment in patients with progressive visual deterioration and having still higher possibility of vision maintenance. GKS can be indicated in patients with progressive visual deterioration and a rare possibility of visual recovery. Surgical indications for ONSM are limited and must be carefully considered for each patient.

Keywords: Meningioma, Optic nerve sheath meningioma, Orbital meningioma

References

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Figure 1.

Distribution of patients with optic nerve sheath meningioma (ONSM) by age and gender.

Figure 2.

Eyelid photos of two patients showing proptosis resulting from ONSM. One patient (case 1) presented with proptosis of the left eye (A, white arrow), and the other patient (case 10) showed proptosis of the right eye (B, white arrow).

Figure 3.

Fundus photographs of patients with ONSM. (A) Fundus photograph of case 1 with globular ONSM demonstrates blurring of optic nerve disc margin (white arrow) and choroidal folds (black arrow). (B) Fundus photograph of case 9 with tubular-diffuse pattern ONSM also showed optic disc swelling (white arrow). (C) Fundus photograph of case 10 with globular ONSM also demonstrates optic disc swelling (white arrow) and choroidal folds (black arrow) radiating superonasally and inferonasally from the disc.

Figure 4.

(A) The tubular-apical expansion ONSM (white arrow) is seen in a 57-year-old woman. Her tumor showed intracranial involvement. (B) The globular pattern ONSM (white arrow) is seen in a 50-year-old woman with a history of decreased vision on the left. (C) The fusiform pattern ONSM (white arrow) occurred in a 52-year-old man who presented with proptosis of his right eye. Note tram tracking in this meningioma.

Figure 5.

(A) Histologic findings of case 3 showed a meningothelial ONMS, in which polygonal cells are arranged in sheets separated by vascular trabeculae. (B) Histologic findings of transitional pattern of ONSM in a 19-year-old man, case 5, in which spindle cells are arranged in whorls and result in deposition of calcium salts (psammomma bodies, white arrow).

Figure 6.

(A) This 50-year-old woman (Case 1) presented with decreased vision and proptosis of the left eye. Under diagnosis of globular ONSM of the left eye, her visual field examinations were shown to have an unchanged enlarged blind spot and inferonasal field defect for 2 years and 7 months. (B) This 52-year-old man (Case 2) presented with proptosis of the right eye. He was diagnosed with tubular-diffuse ONSM of the right eye. His visual field examination was normal and unchanged for 1 year and 1 month.

Figure 7.

T1-weighted transverse MRI imagings of Case 1 patient with globular ONSM (white arrow) of the left eye. (A) MRI imaging taken January 12 ^th, 2005. (B) MRI imaging taken December 28 ^th, 2007. These MRI scans of the same patient 2 years and 11 months later demonstrates only a slight change in tumor size and shows slowly progressive pattern of the ONSM.

Figure 8.

(A) This 46-year-old woman (Case 6) presented with decreased vision of the left eye. She was diagnosed with ONSM (white arrow) of the left eye. (B) This MRI scan of the same patient 5 years and 1 month later demonstrates a dramatically increase in size of tumor with marked proptosis. (C) Gamma-knife surgery was performed. (Volume 15.7 cc, Dose 12 Gy at 50% isodose line, Shot 16x) (D) After 5 years' follow-up, tumor decreased in volume slightly and she remains with decreased exophthalmos.

Figure 9.

(A) This tubular-diffuse ONSM (white arrow) was seen in a 64-year-old woman (Case 9). Optic disc swelling was found accidentallyon regular medical checkup. On physical examination, her vision was 0.9, with evidence of a temporal constriction (C). After 2 years' follow-up, her visual field examinations were shown to have an enlarged blind spot (D). Over a 1-year period, her tumor showed an increase in size and thickness (B). Her vision deteriorated to 0.4–1 and her visual field revealed central island of vision only (E). She underwent a fractionated stereotactic radiotherapy and maintained stable vision with a recovery of visual field (F∼H). She has no more increase in tumor size on MRI (I∼J).

Table 1.

Demographics of patients

Case	Age (yr)	Sex	Underlying disease	Laterality	Treatment modality	Follow-up period (yr)
1	50	F	−	L	Observation	4
2	52	M	−	R	Observation	5
3	57	F	HTN	L	OP: Craniotomy & tumor excisio	n 5
4	52	F	−	R	OP: Kronlein op & tumor excisio	on 4
5	19	M	NF-II	R	OP: Kronlein op & tumor excisio	on 5
6	46	F	−	L	GKS	9
7	57	F	−	L	GKS	1
8	44	F	−	R	GKS	11
9	64	F	−	R	RTx	4
10	38	F	−	R	Incisional biopsy of tumor & RTx	3

HTN = hypertension; NF = neurofibromatosis; OP = operation; GKS = gamma knife surgery; RTx = radiotherapy.

Table 2.

Presenting symptoms and signs

Initial symptoms	No. of patients (%)	Initial signs	No. of patients (%)
Decreased vision	5 (41.7%)	Proptosis	8 (42.1%)
Proptosis	5 (41.7%)	Optic disc change	7 (36.8%)
Transient visual obscurations	1 (8.3%)	Choroidal fold	2 (10.5%)
Eye pain	1 (8.3%)	Limitation of EOM	2 (10.5%)

EOM = extraocular movement.

Table 3.

Configuration of tumor on imaging

Configuration	No. (%)	Intracranial involvement	Tram tracking	Calcification	Irregular margins
Tubular					
Diffuse	3 (33%)	0	2	0	3
Apical expansion	3 (33%)	2	2	0	1
Anterior expansion	0 (0%)	0	0	0	0
Globular	3 (33%)	0	2	0	1
Fusiform	1 (10%)	0	1	0	0
Focal enlargement of optic nerve	0 (0%)	0	0	0	0
Total	10	2	7	0	5

Table 4.

Treatment results

Case	Treatment modality	Best corrected visual acuity		Visual fields
Case	Treatment modality	Pre-treatment	Post-treatment	Pre-treatment	Post-treatment
1	Observation	0.9–1	Last F/U: 0.8–1	1 Enlarged blind spot, IN field defect	Unchanged
2	Observation	1.0	Last F/U: 1.5	WNL	Unchanged
3	OP: Craniotomy & tumor excision	FC	NLP	Cecocentral scotoma	−
4	OP: Kronlein op & tumor excision	0.2	0.02	−	−
5	OP: Kronlein op & tumor excision	1.0–1	1.2	−	−
6	GKS	NLP	NLP	−	−
7	GKS	HM	FC	Temporal island	Unchanged
8	GKS	NLP	NLP	−	−
9	RTx	0.4–1	0.4	Central island	Improved
10	Incisional biopsy of tumor & RTx	0.2	0.5	Enlarged blind spot	−

F/U = follow-up; IN = Inferonasal; WNL = within normal limit; OP = operation; GKS = gamma-knife surgery; RTx = radiotherapy; NLP = no light perception; FC = finger count; HM = hand movements.

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