Objective: Medulloblastoma (MB) is a heterogenous tumor, and the prognosis is influenced by various clinical, histological, and molecular factors. The aim of the study is to determine the clinical profile and radiologic characteristics among the histo-molecular subgroups, the predictors of surgical outcome, and the pattern of relapse in pediatric and adult MB. Method: An analysis of 118 patients of MB who underwent surgical treatment at National Institute of Mental Health and Neurosciences, India, over a 7-year period (2005–2011) is presented. The clinical profile, radiologic characteristics, surgical nuances, and survival patterns are discussed. The relevant statistical analysis was done using SPSS software, version 22.0. Results: The mean age of the cohort was 12 years (12.3 ± 8.7). The primary manifestation was raised intracranial tension headache in 53 patients (44.9%), which was the predominant symptom in large cell/anaplastic (LCA)- and WNT-activated subgroups. The median preoperative Karnofsky performance score was 60 (60.6 ± 12.9). Vermian and hemispheric location of tumor was most commonly observed in non-WNT/non-SHH (groups 3 and 4; 91.7%) and SHH-activated (42.9%) subgroups, respectively. Ninety-two patients (78%) underwent preoperative ventriculoperitoneal shunts (VPS) for obstructive hydrocephalus (HCP) and 14 patients (11.8%) underwent VPS in the postoperative period. The median overall survival (OS) for the whole group was 82.1 ± 5.7 months and the median recurrence-free survival was 51.0 ± 4.8 months. While radiotherapy had a significant influence on OS, progression-free survival was influenced by radiotherapy as well as chemotherapy in both pediatric and adult cohort. Desmoplastic/nodular subtype and WNT-activated subgroup had the best prognosis; LCA and non-WNT/non-SHH had the worst prognosis. Conclusions: Majority of the patients were pediatric in the study. Age, hemispheric location of tumor, extent of resection, and adjuvant treatment status were the important clinical prognostic factors for survival. Surgery for MB is formidable, and VPS can be considered in persistent symptomatic and progressive HCP. Our study on pediatric and adult MB validates the prognostic significance of various clinical, radiologic, and histo-molecular parameters of MB.

1.
Perreault
S
,
Ramaswamy
V
,
Achrol
AS
,
Chao
K
,
Liu
TT
,
Shih
D
, et al
MRI surrogates for molecular subgroups of medulloblastoma
.
AJNR Am J Neuroradiol
.
2014
Jul
;
35
(
7
):
1263
9
.
[PubMed]
0195-6108
2.
Kaur
K
,
Kakkar
A
,
Kumar
A
,
Mallick
S
,
Julka
PK
,
Gupta
D
, et al
Integrating Molecular Subclassification of Medulloblastomas into Routine Clinical Practice: A Simplified Approach
.
Brain Pathol
.
2016
May
;
26
(
3
):
334
43
.
[PubMed]
1015-6305
3.
Ellison
DW
,
Kocak
M
,
Dalton
J
,
Megahed
H
,
Lusher
ME
,
Ryan
SL
, et al
Definition of disease-risk stratification groups in childhood medulloblastoma using combined clinical, pathologic, and molecular variables
.
J Clin Oncol
.
2011
Apr
;
29
(
11
):
1400
7
.
[PubMed]
0732-183X
4.
Louis
DN
,
Perry
A
,
Reifenberger
G
,
von Deimling
A
,
Figarella-Branger
D
,
Cavenee
WK
, et al
The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary
.
Acta Neuropathol
.
2016
Jun
;
131
(
6
):
803
20
.
[PubMed]
0001-6322
5.
Kool
M
,
Korshunov
A
,
Remke
M
,
Jones
DT
,
Schlanstein
M
,
Northcott
PA
, et al
Molecular subgroups of medulloblastoma: an international meta-analysis of transcriptome, genetic aberrations, and clinical data of WNT, SHH, Group 3, and Group 4 medulloblastomas
.
Acta Neuropathol
.
2012
Apr
;
123
(
4
):
473
84
.
[PubMed]
0001-6322
6.
Min
HS
,
Lee
JY
,
Kim
SK
,
Park
SH
.
Genetic grouping of medulloblastomas by representative markers in pathologic diagnosis
.
Transl Oncol
.
2013
Jun
;
6
(
3
):
265
72
.
[PubMed]
1936-5233
7.
Yachnis
AT
,
Perry
A
. Embryonal Tumors of the Central Nervous System. In:
Perry
A
,
Brat
DJ
, editors
.
Practical Surgical neuropathology-A diagnostic approach
. 2nd ed.
ELSEVIER
;
2018
. p.
244
.
8.
Gupta
T
,
Sarkar
C
,
Rajshekhar
V
,
Chatterjee
S
,
Shirsat
N
,
Muzumdar
D
, et al
Indian Society of Neuro-Oncology consensus guidelines for the contemporary management of medulloblastoma
.
Neurol India
.
2017
Mar-Apr
;
65
(
2
):
315
32
.
[PubMed]
0028-3886
9.
Polednak
AP
,
Flannery
JT
.
Brain, other central nervous system, and eye cancer
.
Cancer
.
1995
Jan
;
75
(
1
Suppl
):
330
7
.
[PubMed]
0008-543X
10.
Muzumdar
D
,
Deshpande
A
,
Kumar
R
,
Sharma
A
,
Goel
N
,
Dange
N
, et al
Medulloblastoma in childhood-King Edward Memorial hospital surgical experience and review: comparative analysis of the case series of 365 patients
.
J Pediatr Neurosci
.
2011
Oct
;
6
(
3
Suppl 1
):
S78
85
.
[PubMed]
1817-1745
11.
Wang
C
,
Yuan
XJ
,
Jiang
MW
,
Wang
LF
.
Clinical characteristics and abandonment and outcome of treatment in 67 Chinese children with medulloblastoma
.
J Neurosurg Pediatr
.
2016
Jan
;
17
(
1
):
49
56
.
[PubMed]
1933-0707
12.
Blaser
SI
,
Harwood-Nash
DC
.
Neuroradiology of pediatric posterior fossa medulloblastoma
.
J Neurooncol
.
1996
Jul
;
29
(
1
):
23
34
.
[PubMed]
0167-594X
13.
Meyers
SP
,
Kemp
SS
,
Tarr
RW
.
MR imaging features of medulloblastomas
.
AJR Am J Roentgenol
.
1992
Apr
;
158
(
4
):
859
65
.
[PubMed]
0361-803X
14.
Vézina
LG
,
Packer
RJ
.
Infratentorial brain tumors of childhood
.
Neuroimaging Clin N Am
.
1994
May
;
4
(
2
):
423
36
.
[PubMed]
1052-5149
15.
Bühring
U
,
Strayle-Batra
M
,
Freudenstein
D
,
Scheel-Walter
HG
,
Küker
W
.
MRI features of primary, secondary and metastatic medulloblastoma
.
Eur Radiol
.
2002
Jun
;
12
(
6
):
1342
8
.
[PubMed]
0938-7994
16.
Rippe
DJ
,
Boyko
OB
,
Friedman
HS
,
Oakes
WJ
,
Schold
SC
 Jr
,
DeLong
GR
, et al
Gd-DTPA-enhanced MR imaging of leptomeningeal spread of primary intracranial CNS tumor in children
.
AJNR Am J Neuroradiol
.
1990
Mar-Apr
;
11
(
2
):
329
32
.
[PubMed]
0195-6108
17.
Salunke
P
,
Gupta
K
,
Kovai
P
,
Sura
S
,
Aggarwal
A
.
Preoperative diffuse leptomeningeal spread in a medulloblastoma: paraplegia following surgery for posterior fossa and call for newer management protocols
.
J Pediatr Neurosci
.
2011
Jul
;
6
(
2
):
152
4
.
[PubMed]
1998-3948
18.
Dasgupta
A
,
Gupta
T
,
Pungavkar
S
,
Shirsat
N
,
Epari
S
,
Chinnaswamy
G
, et al
Nomograms based on preoperative multiparametric magnetic resonance imaging for prediction of molecular subgrouping in medulloblastoma: results from a radiogenomics study of 111 patients
.
Neuro-oncol
.
2019
Jan
;
21
(
1
):
115
24
.
[PubMed]
1522-8517
19.
Gibson
P
,
Tong
Y
,
Robinson
G
,
Thompson
MC
,
Currle
DS
,
Eden
C
, et al
Subtypes of medulloblastoma have distinct developmental origins
.
Nature
.
2010
Dec
;
468
(
7327
):
1095
9
.
[PubMed]
0028-0836
20.
Zhao
F
,
Li
C
,
Zhou
Q
,
Qu
P
,
Wang
B
,
Wang
X
, et al
Distinctive localization and MRI features correlate of molecular subgroups in adult medulloblastoma
.
J Neurooncol
.
2017
Nov
;
135
(
2
):
353
60
.
[PubMed]
0167-594X
21.
Muzumdar
D
,
Ventureyra
EC
.
Treatment of posterior fossa tumors in children
.
Expert Rev Neurother
.
2010
Apr
;
10
(
4
):
525
46
.
[PubMed]
1473-7175
22.
Schneider
C
,
Ramaswamy
V
,
Kulkarni
AV
,
Rutka
JT
,
Remke
M
,
Tabori
U
, et al
Clinical implications of medulloblastoma subgroups: incidence of CSF diversion surgery
.
J Neurosurg Pediatr
.
2015
Mar
;
15
(
3
):
236
42
.
[PubMed]
1933-0707
23.
Dias
MS
,
Aronyk
KE
.
A modified lateral decubitus position for surgical approaches to midline posterior fossa lesions: technical note
.
Pediatr Neurosurg
.
1996
Oct
;
25
(
4
):
210
2
.
[PubMed]
1016-2291
24.
Lal
P
,
Nagar
YS
,
Kumar
S
,
Singh
S
,
Maria Das
KJ
,
Narayan
SL
, et al
Medulloblastomas: clinical profile, treatment techniques and outcome - an institutional experience
.
Indian J Cancer
.
2002
Jul-Sep
;
39
(
3
):
97
105
.
[PubMed]
0019-509X
25.
Packer
RJ
,
Cogen
P
,
Vezina
G
,
Rorke
LB
.
Medulloblastoma: clinical and biologic aspects
.
Neuro-oncol
.
1999
Jul
;
1
(
3
):
232
50
.
[PubMed]
1522-8517
26.
Packer
RJ
,
Rood
BR
,
MacDonald
TJ
.
Medulloblastoma: present concepts of stratification into risk groups
.
Pediatr Neurosurg
.
2003
Jul
;
39
(
2
):
60
7
.
[PubMed]
1016-2291
27.
Zeltzer
PM
,
Boyett
JM
,
Finlay
JL
,
Albright
AL
,
Rorke
LB
,
Milstein
JM
, et al
Metastasis stage, adjuvant treatment, and residual tumor are prognostic factors for medulloblastoma in children: conclusions from the Children’s Cancer Group 921 randomized phase III study
.
J Clin Oncol
.
1999
Mar
;
17
(
3
):
832
45
.
[PubMed]
0732-183X
28.
Griffin
CA
,
Hawkins
AL
,
Packer
RJ
,
Rorke
LB
,
Emanuel
BS
.
Chromosome abnormalities in pediatric brain tumors
.
Cancer Res
.
1988
Jan
;
48
(
1
):
175
80
.
[PubMed]
0008-5472
29.
Gajjar
AJ
,
Robinson
GW
.
Medulloblastoma-translating discoveries from the bench to the bedside
.
Nat Rev Clin Oncol
.
2014
Dec
;
11
(
12
):
714
22
.
[PubMed]
1759-4774
30.
Srikantha
U
,
Balasubramaniam
A
,
Santosh
V
,
Somanna
S
,
Bhagavatula
ID
,
Ashwathnarayana
CB
.
Recurrence in medulloblastoma - influence of clinical, histological and immunohistochemical factors
.
Br J Neurosurg
.
2010
Jun
;
24
(
3
):
280
8
.
[PubMed]
0268-8697
31.
David
KM
,
Casey
AT
,
Hayward
RD
,
Harkness
WF
,
Phipps
K
,
Wade
AM
.
Medulloblastoma: is the 5-year survival rate improving? A review of 80 cases from a single institution
.
J Neurosurg
.
1997
Jan
;
86
(
1
):
13
21
.
[PubMed]
0022-3085
32.
Albright
AL
,
Wisoff
JH
,
Zeltzer
PM
,
Boyett
JM
,
Rorke
LB
,
Stanley
P
.
Effects of medulloblastoma resections on outcome in children: a report from the Children’s Cancer Group
.
Neurosurgery
.
1996
Feb
;
38
(
2
):
265
71
.
[PubMed]
0148-396X
33.
Tait
DM
,
Thornton-Jones
H
,
Bloom
HJ
,
Lemerle
J
,
Morris-Jones
P
.
Adjuvant chemotherapy for medulloblastoma: the first multi-centre control trial of the International Society of Paediatric Oncology (SIOP I)
.
Eur J Cancer
.
1990
Apr
;
26
(
4
):
464
9
.
[PubMed]
0959-8049
34.
Taylor
MD
,
Northcott
PA
,
Korshunov
A
,
Remke
M
,
Cho
YJ
,
Clifford
SC
, et al
Molecular subgroups of medulloblastoma: the current consensus
.
Acta Neuropathol
.
2012
Apr
;
123
(
4
):
465
72
.
[PubMed]
0001-6322
35.
Ellison
DW
,
Dalton
J
,
Kocak
M
,
Nicholson
SL
,
Fraga
C
,
Neale
G
, et al
Medulloblastoma: clinicopathological correlates of SHH, WNT, and non-SHH/WNT molecular subgroups
.
Acta Neuropathol
.
2011
Mar
;
121
(
3
):
381
96
.
[PubMed]
0001-6322
36.
Cho
YJ
,
Tsherniak
A
,
Tamayo
P
,
Santagata
S
,
Ligon
A
,
Greulich
H
, et al
Integrative genomic analysis of medulloblastoma identifies a molecular subgroup that drives poor clinical outcome
.
J Clin Oncol
.
2011
Apr
;
29
(
11
):
1424
30
.
[PubMed]
0732-183X
37.
Northcott
PA
,
Korshunov
A
,
Pfister
SM
,
Taylor
MD
.
The clinical implications of medulloblastoma subgroups
.
Nat Rev Neurol
.
2012
May
;
8
(
6
):
340
51
.
[PubMed]
1759-4758
38.
Clifford
SC
,
Lusher
ME
,
Lindsey
JC
,
Langdon
JA
,
Gilbertson
RJ
,
Straughton
D
, et al
Wnt/Wingless pathway activation and chromosome 6 loss characterize a distinct molecular sub-group of medulloblastomas associated with a favorable prognosis
.
Cell Cycle
.
2006
Nov
;
5
(
22
):
2666
70
.
[PubMed]
1538-4101
39.
Northcott
PA
,
Korshunov
A
,
Witt
H
,
Hielscher
T
,
Eberhart
CG
,
Mack
S
, et al
Medulloblastoma comprises four distinct molecular variants
.
J Clin Oncol
.
2011
Apr
;
29
(
11
):
1408
14
.
[PubMed]
0732-183X
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.