Isolated Optic Nerve Gliomas

IONG

Ben Shofty, Liat Ben-Sira, Anat Kesler, George Jallo, Mary L. Groves, Alvaro Lassaletta, Uri Tavori, & Shlomi Constantini

Department of Pediatric Neurosurgery, Dana Children’s Hospital, Tel-Aviv Medical Center

The Gilbert Israeli Neurofibromatosis Center

Methods

Multi-center, historical cohort study

• Most common neoplasm of the optic system

• Represent 1% of all brain tumors

(5% in children)

• Amongst NF+ patients present

in 15%-20% of pts.

OPG - Mainly indolent course,

especially in NF+ children

OPG’s: Introduction

OPG - A Heterogeneous Disease

Do we have a good classification?

• Basic classification by Dodge (1958)

• Modified Dodge Classification (2008)

• New staging (Suggestion: un-published)

What is IONG?

Isolated Optic Nerve Glioma

• Tumors restricted to the ON

• >4mm width

• Enhancing +/-

From IONA to IONG

Optic Nerve

Enhancement

Optic Nerve

Tortuosity

T2 signal changes

True IONG

IONA -IONG International Study

• Inclusion criteria:

– Clear cut IONG only

– NF and non-NF

– Follow-up > 1 year

• Exclusion Criteria:

– Chiasmatic involvement

– Other brain tumors

– Hydrocephalus

Results (n=34)

• 34 patients, 16 males, 22 NF

– 10 Tel-Aviv

– 7 Baltimore

– 17 Toronto

• Average age at diagnosis - ~6y (±4y)

• Average follow-up time – 5.6y (±4.25y)

Results - Radiology• Intraorbital 13 pts (38%)

• Intracranial 8 pts (23%)

• Both 13 pts (39%)

• 8 pts (23%) bilateral disease (7/8 NF1)

Radiological outcome

• 15/34 pts (44%) radiological progression

– 4/8 pts (50%) with bilateral disease

– 7/22 pts (31%) with NF1

• 6/34 (17%) spontaneous regression – all NF1!

IONG (n=34; 100%)

Normal Vision (n=19; 56%)

Deterioration (n=8; 42%)

Stable (n=11; 58%)

Visual Impairment (n=15; 44%)

Deterioration (n=13; 86%)

Stable (n=2; 14%)

Visual outcome

IONG (n=34; 100%)

Normal Vision (n=19; 56%)

Deterioration (n=8; 42%)

Stable (n=11; 58%)

Visual Impairment (n=15; 44%)

Deterioration (n=13; 86%)

Stable (n=2; 14%)

Visual outcome

Patients with decreased vision at presentation

– high risk for visual deterioration

Results - Treatment• 16 pts (47%) required treatment (6/22 NF1, 10/12 spor)

• 2 pts – 2ed line of chemo

• 8/34 pts (23%) chemo

– 5/8 (62%) benefit visually

– 4/8 (50%) benefit radiologically

Pre-Treatment Post-Treatment

Treatment Cont.

• 1 pt radiation – no benefit

• 8/34 pts (23%) – resection d/t severe proptosis

– All for progressive tumors

– All with severe visual deterioration

– After resection no additional tumors

* After

resection no

additional

tumors

Conclusion - IONG

• Active tumors, >50% will progress (5.6 years of

follow-up)

• Progressive visual impairment is common

• May respond better to chemo then general OPG

• Spontaneous regression is possible in NF1

Thank you

Dr. George Jallow

Dr. Mary Groves

Prof. Shlomi Constantini

Dr. Liat Ben-Sira

Prof. Anat Kesler

Dr. Felix Bokstein

Dr. Li-Tal Pratt

Dr. Michal Mauda-

Havakuk

Edna Fadida

Dr. Ben Shofty

The Gilbert Israeli NF Center

Dr. Uri Tavori

Dr. Alvaro Lassaleta

Dr. Eric Bouffet

Jhons Hopkins Hospital For Sick Kids Toronto

Ramon y Cajal 1891

• Is it the same disease ?

• Does it need the same treatment ?

• From the literature – following chemo -

– ~45% radiological stabilization

– ~15-30% visual improvement

• Mixed groups – different tumors

• Short-term FU

• Often multiple treatment lines

Surgical Dilemma

• Indications for surgery –

– Severe proptosis

– Blind eye

– Intractable pain

• Resection prevents progression ?

– In our series - Yes

Visual outcome

• 41% (add numbers) - impairment at diagnosis

– 22% in NF1 group

• 61% visual deterioration (length of follow-up)

– 50% had visual impairment

– 60% in the NF1 group!

• 0% spontaneous visual improvement

• OPG - Mainly indolent, especially in NF+

children

• 1/3-1/2 will cause clinical symptoms

• >1/3 will require oncological treatment

From OPG to IONG Tel Aviv

• Local OPG Database – 100 patients

• True IONG – 12 patients – 12%

– 2 excluded – insufficient data