Background: Optic pathway/hypothalamic gliomas are rare pediatric brain tumors. The management paradigm for these challenging tumors includes chemotherapy, radiotherapy, or surgical resection, but the optimal management strategy remains elusive. Gamma knife radiosurgery (GKRS) has emerged as a promising treatment for such lesions as documented by a small number of cases in the literature. Case Presentation: We present a rare case of hypothalamic glioma in a 13-year-old girl who was referred to our service due to growth of an incidentally diagnosed hypothalamic lesion following head injury at the age of 8 years. The lesion demonstrated hypointensity on T1- and hyperintensity on T2-weighted imaging without contrast enhancement. Given the growth of the lesion on serial imaging, a stereotactic biopsy was performed demonstrating low-grade glioma. The patient underwent GKRS treatment with a marginal dose of 15 Gy at 50% isodose line for a tumor volume of 2.2 mL. Annual radiological surveillance over the next 17 years demonstrated a gradual shrinkage of the lesion until it completely disappeared. The patient is currently a healthy 31-year-old female without any visual, endocrine, or neurocognitive deficits. Conclusion: The outcome obtained after extended follow-up in our patient highlights the safety and efficacy of GKRS in the management of hypothalamic gliomas in pediatrics, which in turn can avoid potentially serious complications of surgery in this vulnerable patient population, especially in this sensitive location.

1.
Rutka
JT
,
Becker
LE
,
Hoffman
HJ
.
Optic chiasmatic‐hypothalamic glioma
.
Brain Pathol
.
1997
;
7
(
2
):
799
806
.
2.
Barkovich
AJ
.
Pediatric neuroimaging
.
Lippincott Williams & Wilkins
;
2005
.
3.
Hoffman
HJ
,
Humphreys
RP
,
Drake
JM
,
Rutka
JT
,
Becker
LE
,
Jenkin
D
,
Optic pathway/hypothaiamic gliomas: a dilemma in management
.
Pediatr Neurosurg
.
1993
;
19
(
4
):
186
95
.
4.
Jaing
TH
,
Lin
KL
,
Tsay
PK
,
Hsueh
C
,
Hung
PC
,
Wu
CT
,
Treatment of optic pathway hypothalamic gliomas in childhood: experience with 18 consecutive cases
.
J Pediatr Hematol Oncol
.
2008
;
30
(
3
):
222
4
. .
5.
Silva
MM
,
Goldman
S
,
Keating
G
,
Marymont
MA
,
Kalapurakal
J
,
Tomita
T
.
Optic pathway hypothalamic gliomas in children under three years of age: the role of chemotherapy
.
Pediatr Neurosurg
.
2000
;
33
(
3
):
151
8
. .
6.
Lacaze
E
,
Kieffer
V
,
Streri
A
,
Lorenzi
C
,
Gentaz
E
,
Habrand
JL
,
Neuropsychological outcome in children with optic pathway tumours when first-line treatment is chemotherapy
.
Br J Cancer
.
2003
;
89
(
11
):
2038
44
. .
7.
Diaz
RJ
,
Laughlin
S
,
Nicolin
G
,
Buncic
JR
,
Bouffet
E
,
Bartels
U
.
Assessment of chemotherapeutic response in children with proptosis due to optic nerve glioma
.
Childs Nerv Syst
.
2008
;
24
(
6
):
707
12
. .
8.
Tao
ML
,
Barnes
PD
,
Billett
AL
,
Leong
T
,
Shrieve
DC
,
Scott
RM
,
Childhood optic chiasm gliomas: radiographic response following radiotherapy and long-term clinical outcome
.
Int J Radiat Oncol Biol Phys
.
1997
;
39
(
3
):
579
87
. .
9.
Saran
FH
,
Baumert
BG
,
Khoo
VS
,
Adams
EJ
,
Garré
ML
,
Warrington
AP
,
Stereotactically guided conformal radiotherapy for progressive low-grade gliomas of childhood
.
Int J Radiat Oncol Biol Phys
.
2002
;
53
(
1
):
43
51
. .
10.
Pepin
SM
,
Lessell
S
.
Anterior visual pathway gliomas: the last 30 years
.
Sem Ophthalmol
.
2006
;
21
(
3
):
117
24
. .
11.
Ju
D
,
Kocag̈onc̈u
KO
,
Ḧoss
A
,
Wenz
F
,
Wannenmacher
M
.
Fractionated stereotactic radiotherapy (FSRT) for optic glioma
.
Int J Radiat Oncol Biol Phys
.
1999
;
44
(
2
):
243
8
.
12.
Combs
SE
,
Schulz-Ertner
D
,
Moschos
D
,
Thilmann
C
,
Huber
PE
,
Debus
J
.
Fractionated stereotactic radiotherapy of optic pathway gliomas: tolerance and long-term outcome
.
Int J Radiat Oncol Biol Phys
.
2005
;
62
(
3
):
814
9
. .
13.
Astrup
J
.
Natural history and clinical management of optic pathway glioma
.
Br J Neurosurg
.
2003
;
17
(
4
):
327
35
. .
14.
Grabenbauer
GG
,
Schuchardt
U
,
Buchfelder
M
,
Rödel
CM
,
Gusek
G
,
Marx
M
,
Radiation therapy of optico–hypothalamic gliomas (OHG)–radiographic response, vision and late toxicity
.
Radiother Oncol
.
2000
;
54
(
3
):
239
45
.
15.
Collett‐Solberg
PF
,
Sernyak
H
,
Satin‐Smith
M
,
Katz
LL
,
Sutton
L
,
Molloy
P
,
Endocrine outcome in long‐term survivors of low‐grade hypothalamic/chiasmatic glioma
.
Clin Endocrinol
.
1997
;
47
(
1
):
79
85
.
16.
Alvord
EC
,
Lofton
S
.
Gliomas of the optic nerve or chiasm: outcome by patientsʼ age, tumor site, and treatment
.
J Neurosurg
.
1988
;
68
(
1
):
85
98
.
17.
El-Shehaby
AM
,
Reda
WA
,
Karim
KMA
,
Eldin
RME
,
Nabeel
AM
.
Single-session gamma knife radiosurgery for optic pathway/hypothalamic gliomas
.
J Neurosurg
.
2016
;
125
(
Suppl 1
):
50
7
.
18.
Han
SR
,
Yoon
SW
,
Yee
GT
,
Choi
CY
,
Sohn
MJ
,
Lee
DJ
,
Novalis radiosurgery of optic gliomas in children: preliminary report
.
Pediatr Neurosurg
.
2007
;
43
(
4
):
251
7
. .
19.
Liang
CL
,
Lu
K
,
Liliang
PC
,
Chen
HJ
.
Gamma knife surgery for optic glioma: report of 2 cases
.
J Neurosurg
.
2010
;
113
:
44
7
.
20.
Lim
YJ
,
Leem
W
.
Two cases of gamma knife radiosurgery for low-grade optic chiasm glioma
.
Stereotact Funct Neurosurg
.
1996
;
66
(
Suppl 1
):
174
83
. .
21.
Coffey
RJ
,
Lunsford
LD
,
Taylor
FH
.
Survival after stereotactic biopsy of malignant gliomas
.
Neurosurgery
.
1988
;
22
(
3
):
465
73
. .
22.
Jumah
F
,
Rallo
MS
,
Quinoa
T
,
Raju
B
,
Narayan
V
,
Gupta
G
,
Incidental brain tumors in the pediatric population: a systematic review and reappraisal of literature
.
World Neurosurg
.
2020
;
139
:
121
31
. .
23.
Zaazoue
MA
,
Manley
PE
,
Kapur
K
,
Ullrich
NJ
,
Silvera
VM
,
Goumnerova
LC
.
Natural history and management of incidentally discovered focal brain lesions indeterminate for tumor in children
.
Neurosurgery
.
2020
;
86
(
3
):
357
65
. .
24.
Soleman
J
,
Kozyrev
DA
,
Ben-Sira
L
,
Constantini
S
,
Roth
J
.
Management of incidental brain tumors in children: a systematic review
.
Childs Nerv Syst
.
2020
;
36
(
8
):
1607
19
. .
25.
Horwich
A
,
Bloom
HJ
.
Optic gliomas: radiation therapy and prognosis
.
Int J Radiat Oncol Biol Phys
.
1985
;
11
(
6
):
1067
79
. .
26.
Jenkin
D
,
Angyalfi
S
,
Becker
L
,
Berry
M
,
Buncic
R
,
Chan
H
,
Optic glioma in children: surveillance, resection, or irradiation?
Int J Radiat Oncol Biol Phys
.
1993
;
25
(
2
):
215
25
. .
27.
Weiss
L
,
Sagerman
RH
,
King
GA
,
Chung
CT
,
Dubowy
RL
.
Controversy in the management of optic nerve glioma
.
Cancer
.
1987
;
59
(
5
):
1000
4
. .
28.
Jahraus
CD
,
Tarbell
NJ
.
Optic pathway gliomas
.
Pediatr Blood Cancer
.
2006
;
46
(
5
):
586
96
. .
29.
Goodden
J
,
Pizer
B
,
Pettorini
B
,
Williams
D
,
Blair
J
,
Didi
M
,
The role of surgery in optic pathway/hypothalamic gliomas in children
.
J Neurosurg Pediatr
.
2014
;
13
(
1
):
1
12
. .
30.
Tsang
DS
,
Murphy
ES
,
Merchant
TE
.
Radiation therapy for optic pathway and hypothalamic low-grade gliomas in children
.
Int J Radiat Oncol Biol Phys
.
2017
;
99
(
3
):
642
51
. .
31.
Merchant
TE
,
Kun
LE
,
Wu
S
,
Xiong
X
,
Sanford
RA
,
Boop
FA
.
Phase II trial of conformal radiation therapy for pediatric low-grade glioma
.
J Clin Oncol
.
2009
;
27
(
22
):
3598
. .
32.
Fouladi
M
,
Wallace
D
,
Langston
JW
,
Mulhern
R
,
Rose
SR
,
Gajjar
A
,
Survival and functional outcome of children with hypothalamic/chiasmatic tumors
.
Cancer
.
2003
;
97
(
4
):
1084
92
. .
33.
Kwon
Y
,
Bae
JS
,
Kim
JM
,
Kim
SY
,
Ahn
JS
,
Kim
JH
,
Visual changes after gamma knife surgery for optic nerve tumors: report of three cases
.
J Neurosurg
.
2005
;
102
:
143
6
.
34.
Palma
L
,
Celli
P
,
Mariottini
A
.
Long-term follow-up of childhood cerebellar astrocytomas after incomplete resection with particular reference to arrested growth or spontaneous tumour regression
.
Acta Neurochir
.
2004
;
146
(
6
):
581
8
. .
35.
Gunny
RS
,
Hayward
RD
,
Phipps
KP
,
Harding
BN
,
Saunders
DE
.
Spontaneous regression of residual low-grade cerebellar pilocytic astrocytomas in children
.
Pediatr Radiol
.
2005
;
35
(
11
):
1086
91
. .
36.
Rozen
WM
,
Joseph
S
,
Lo
PA
.
Spontaneous regression of low-grade gliomas in pediatric patients without neurofibromatosis
.
Pediatr Neurosurg
.
2008
;
44
(
4
):
324
8
. .
37.
Kano
H
,
Niranjan
A
,
Kondziolka
D
,
Flickinger
JC
,
Pollack
IF
,
Jakacki
RI
,
Stereotactic radiosurgery for pilocytic astrocytomas part 2: outcomes in pediatric patients
.
J Neurooncol
.
2009
;
95
(
2
):
219
29
. .
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.