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
• 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
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 +/-
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
• 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