ArticlePDF Available

Surgical outcome and prognostic factors in spinal cord ependymoma: a single-center, long-term follow-up study

Authors:

Abstract and Figures

Objective Spinal cord ependymomas account for 3–6% of all central nervous system tumors and around 60% of all intramedullary tumors. The aim of this study was to analyze the neurological outcome after surgery and to determine prognostic factors for functional outcome. Patients and Methods Patients treated surgically due to a spinal cord ependymoma between 1990 and 2018 were retrospectively included. Demographics, neurological symptoms, radiological parameters, histopathology, and neurological outcome (using McCormick Score [MCS]) were analyzed. Possible prognostic factors for neurological outcome were evaluated. Results In total, 148 patients were included (76 males, 51.4%). The mean age was 46.7 ± 15.3 years. The median follow-up period was 6.8 ± 5.4 years. The prevalence was mostly in the lumbar spine (45.9%), followed by the thoracic spine (28.4%) and cervical spine (25.7%). Gross-total resection was achieved in 129 patients (87.2%). The recurrence rate was 8.1% and depended on the extent of tumor resection ( p = 0.001). Postoperative temporary neurological deterioration was observed in 63.2% of patients with ependymomas of the cervical spine, 50.0% of patients with ependymomas of the thoracic spine, and 7.4% of patients with ependymomas of the lumbosacral region. MCS 1–2 was detected in nearly two-thirds of patients with cervical and thoracic spinal cord ependymoma 36 months after surgery. Neurological recovery was superior in thoracic spine ependymomas compared with cervical spine ependymomas. Poor preoperative functional condition (MCS >2), cervical and thoracic spine location, and tumor extension >2 vertebrae were independent predictors of poor neurological outcome. Conclusion Neurological deterioration was seen in the majority of cervical and thoracic spine ependymomas. Postoperative improvement was less in thoracic cervical spine ependymomas compared with thoracic spine ependymomas. Poor preoperative status and especially tumor extension >2 vertebrae are predictors of poor neurological outcome (MCS >2).
Content may be subject to copyright.
https://doi.org/10.1177/17562864211055694
https://doi.org/10.1177/17562864211055694
Therapeutic Advances in Neurological Disorders
journals.sagepub.com/home/tan 1
Ther Adv Neurol Disord
2021, Vol. 14: 1–14
DOI: 10.1177/
17562864211055694
© The Author(s), 2021.
Article reuse guidelines:
sagepub.com/journals-
permissions
Creative Commons CC BY: This article is distributed under the terms of the Creative Commons Attribution 4.0 License (https://creativecommons.org/licenses/by/4.0/)
which permits any use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open
Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage).
Introduction
Spinal cord ependymomas are usually slow-grow-
ing tumors arising from ependymal cells of the
central canal of the spinal cord.1 Ependymomas
account for 3–6% of all central nervous system
tumors and are the most frequent spinal cord
neoplasm in adults, presenting 60% of all
intramedullary tumors.2–5
Surgical outcome and prognostic factors in
spinal cord ependymoma: a single-center,
long-term follow-up study
Oliver Gembruch , Mehdi Chihi, Merle Haarmann, Ahmet Parlak,
Marvin Darkwah Oppong , Laurèl Rauschenbach, Anna Michel, Ramazan Jabbarli,
Yahya Ahmadipour, Ulrich Sure, Philipp Dammann and Neriman Özkan
Abstract
Objective: Spinal cord ependymomas account for 3–6% of all central nervous system
tumors and around 60% of all intramedullary tumors. The aim of this study was to analyze
the neurological outcome after surgery and to determine prognostic factors for functional
outcome.
Patients and Methods: Patients treated surgically due to a spinal cord ependymoma between
1990 and 2018 were retrospectively included. Demographics, neurological symptoms,
radiological parameters, histopathology, and neurological outcome (using McCormick Score
[MCS]) were analyzed. Possible prognostic factors for neurological outcome were evaluated.
Results: In total, 148 patients were included (76 males, 51.4%). The mean age was 46.7 ± 15.3
years. The median follow-up period was 6.8 ± 5.4 years. The prevalence was mostly in the
lumbar spine (45.9%), followed by the thoracic spine (28.4%) and cervical spine (25.7%).
Gross-total resection was achieved in 129 patients (87.2%). The recurrence rate was 8.1%
and depended on the extent of tumor resection (p = 0.001). Postoperative temporary
neurological deterioration was observed in 63.2% of patients with ependymomas of the
cervical spine, 50.0% of patients with ependymomas of the thoracic spine, and 7.4% of patients
with ependymomas of the lumbosacral region. MCS 1–2 was detected in nearly two-thirds
of patients with cervical and thoracic spinal cord ependymoma 36 months after surgery.
Neurological recovery was superior in thoracic spine ependymomas compared with cervical
spine ependymomas. Poor preoperative functional condition (MCS >2), cervical and thoracic
spine location, and tumor extension >2 vertebrae were independent predictors of poor
neurological outcome.
Conclusion: Neurological deterioration was seen in the majority of cervical and thoracic spine
ependymomas. Postoperative improvement was less in thoracic cervical spine ependymomas
compared with thoracic spine ependymomas. Poor preoperative status and especially tumor
extension >2 vertebrae are predictors of poor neurological outcome (MCS >2).
Keywords: neurological outcome, outcome prediction, predictors, spinal ependymoma,
surgery
Received: 19 April 2021; revised manuscript accepted: 8 October 2021.
Correspondence to:
Oliver Gembruch
Department of
Neurosurgery, University
Hospital Essen, University
of Duisburg-Essen,
Hufelandstrasse 55, 45122
Essen, Germany.
oliver.gembruch@uk-
essen.de
Mehdi Chihi
Merle Haarmann
Ahmet Parlak
Marvin Darkwah Oppong
Laurèl Rauschenbach
Anna Michel
Ramazan Jabbarli
Yahya Ahmadipour
Ulrich Sure
Philipp Dammann
Neriman Özkan
Department of
Neurosurgery, University
Hospital Essen, University
of Duisburg-Essen, Essen,
Germany
1055694TAN0010.1177/17562864211055694Therapeutic Advances in Neurological Disorders X(X)O Gembruch, M Chihi
research-article20212021
Original Research
Therapeutic Advances in Neurological Disorders 14
2 journals.sagepub.com/home/tan
Symptom presentation is related to the tumor
location and can include radicular or local pain,
motor weakness of the extremities, hypoesthesia,
gait disturbance, and sphincter or sexual dysfunc-
tion.6–8 Non-specificity of symptoms can lead to
adaptation to symptoms and late diagnosis.
Cervical tumors can present symptoms of upper
or lower extremities, due to the corticospinal tract
or dorsal column being affected.9 Symptom dura-
tion depends on tumor location and symptom
characteristics, with back pain being the most
common symptom. The average symptom dura-
tion described in the literature is around 2
years.10–12 In rare cases, an acute deterioration of
the symptoms can be provoked by intratumoral
hemorrhage.13–15
The World Health Organization (WHO) grading
system includes three ependymoma subtypes:
WHO grade I: the myxopapillary ependymoma
and the subependymoma; WHO grade II: ‘clas-
sic’ ependymoma including papillary, clear cell,
and tanycytic subtypes; and WHO grade III: ana-
plastic ependymoma.16
The ‘classic’ ependymoma is the most common
in the spinal cord with a frequency of 55–75%.9,13
Benign ependymomas (WHO grade I) and semi-
benign ependymomas (WHO grade II) have
well-defined margins that allow microsurgical
tumor removal without damaging the spinal cord
tissue. In contrast, anaplastic ependymomas
(WHO III) are infiltrative and only subtotal
resection is possible.13,17,18 The prognosis based
on WHO grading alone is difficult due to the het-
erogeneity of ependymomas and its tumor
characteristics.1,6,13
The long-term survival and prognostic factors for
tumor-free survival in spinal cord ependymomas
have been thoroughly investigated.
According to current gold standard, surgical
resection remains the therapy of choice in spinal
cord ependymomas, especially for patients pre-
senting with neurological impairments.
Postoperative neurological deterioration remains
a major problem that might be reduced further as
surgical techniques continue to advance.19
Despite the well-known neurological deteriora-
tion after surgery, studies focusing on predictors
of neurological outcome are scarce because of the
small sample size.6,9,20,21
This study is one of the largest single-center stud-
ies of a European neurosurgical center and aims
to describe the tumor entity and the surgical
course. In addition, we focused on factors causing
postoperative functional deterioration and tried
to identify predictive factors with impact on the
postoperative neurological outcome.
Patients and methods
Study population
A retrospective analysis of the electronic database
‘spinal neoplasm’ evaluating the clinical and radi-
ological data and operative reports of patients suf-
fering from a spinal ependymoma who attended
to our department between 1990 and 2018 was
performed. Only patients suffering from primary
spinal cord ependymoma were included in the
analysis. Patients with primary cerebral epend-
ymoma and secondary spinal cord metastasis or
leptomeningeal metastasis were excluded.
Evaluated parameters
The demographics, symptom duration until sur-
gery, neurological symptoms such as pain, sen-
sory deficits, motor deficits (monoparesis/
hemiparesis and paraparesis), gait disturbance,
and bladder dysfunction for each case were noted.
Radiological parameters including tumor location
(intramedullary and extramedullary, cervical,
thoracic, and lumbar), tumor size in terms of size
expanding over the number of vertebrae, and vol-
ume (cm3) according to preoperative magnetic
resonance imaging (MRI) were evaluated. Gross-
total resection (GTR) was defined as complete
tumor removal, showing no tumor remnants in
the early postoperative MRI with contrast.
Subtotal tumor resection (STR) was present if
the early postoperative MRI showed tumor
remnants.
Patients’ neurological status was evaluated using
the McCormick Score (MCS I: neurologically
normal; mild focal deficit not significantly affect-
ing the function of the involved limb; mild spas-
ticity or reflex abnormality; normal gait; MCS II:
presence of sensorimotor deficit affecting the
function of the involved limb; mild to moderate
gait difficulty; severe pain or dysesthetic syn-
drome impairing patient’s quality of life; still
functions and ambulates independently; MCS
III: more severe neurological deficit; requires
O Gembruch, M Chihi et al.
journals.sagepub.com/home/tan 3
cane/brace for ambulation or significant bilateral
upper extremity impairment; may or may not
function independently; MCS IV: severe deficit;
requires wheelchair or cane/brace with bilateral
upper extremity impairment; usually not inde-
pendent).22 The MCS was retrospectively derived
from clinical data at the beginning of the observa-
tional study. Later, MCS was routinely used in
clinical practice. The MCS was modified accord-
ing to the current literature20,23 to allow a more
valuable discrimination in postoperative neuro-
logical outcome: ‘good’ was defined as MCS
I + II and ‘poor’ was defined as MCS III + IV.
A cut-off value of MCS >2 was chosen because
patients suffer from moderate neurological defi-
cits with limitations in function. Furthermore,
external aid may be needed. Patients with MCS
2 do not show severe neurological deficits and
do not need external help.
According to our clinical standard of care, neuro-
logical examination was performed routinely pre-
operatively, postoperatively on the last day at the
hospital, and 3 months, 6 months, 12 months, 24
months, and 36 months after surgery. Furthermore,
postoperative status including MCS was assessed
3, 5, and 10 years after surgery. Postoperative MRI
was performed within 72 h after surgery. Further
MRI controls were performed every 3–6 months.
Surgical treatment
The surgery was performed in microsurgical fash-
ion using a standard dorsal approach in prone posi-
tion for lesions located at the thoracal and lumbar
spine. Semi-sitting position was favored in cervical
spine tumors. Laminoplasty was routinely per-
formed, whereas laminectomy was performed in
cases where refixation of the laminae was deemed
unfavorable (distinct osteoporosis or vertebral
deformity). Hemilaminectomy was indicated in lat-
eral located tumors and usually in lumbar filum ter-
minal ependymomas. Surgical removal of the
ependymoma was performed with the aid of intra-
operative monitoring (somatosensory evoked
potentials and motor evoked potentials). The
tumor was removed piecemeal-like, beginning from
the center to the well-defined margins and the sur-
rounding spinal cord tissue (Figures 1–3). Patients
were mobilized after a bed rest of 3 days to avoid
postoperative cerebrospinal fluid fistula. Tumor
analysis was performed at the Department of
Neuropathology of the University Hospital Essen.
Statistics
Data were analyzed using SPSS 25.0 (Statistical
Package for the Social Sciences, SPSS Inc.,
Chicago, IL, USA) for Windows. Metric data
were described by mean and standard deviation
and nominal data by frequency and valid percent-
age. P values <0.05 in two-sided testing were
considered significant.
Demographic, clinical, and radiographic param-
eters were analyzed in a univariate way regarding
their association or correlation with preoperative
and postoperative McCormick Score. Therefore,
Pearson’s χ2 statistics or Fisher’s exact test was
used for dichotomous variables. For non-nor-
mally distributed data, the Kendall’s tau-b was
assessed for continuous and ordinal, Spearman’s
rho for continuous and dichotomous, and Mann–
Whitney U test for ordinal and continuous varia-
bles. Significant parameters selected through
univariate analysis and parameters with p values
<0.1 were subsequently evaluated using multi-
variate analysis.
The neurological outcome was analyzed based on
the tumor location: cervical, thoracic, and lumbar.
Patients who were lost to follow-up were not
included in statistical analysis at those time points.
Results
Clinical characteristics
Over a period of 28 years, 148 patients [72
females (48.6%) and 76 males (51.4%)] suffering
from spinal cord ependymoma underwent sur-
gery in our institute. The mean age was
46.7 ± 15.3 years, ranging from 9 to 83 years.
Four patients were 16 years and younger. The
mean follow-up was 6.8 ± 5.4 years (up to 27
years). However, 13 patients (8.8%) were lost to
follow-up 12 months after surgery and 23.0% (34
patients) 36 months after surgery.
The most frequently involved localization was the
lumbar-sacral region (45.9%), followed by the
thoracic (28.4%) and cervical region (25.7%).
The tumor was located intramedullary in 59.5%
(Table 1).
The average duration of symptoms until surgical
treatment was 29.4 ± 57.3 months. The present-
ing symptom was pain in 67.6%. Radiating pain
Therapeutic Advances in Neurological Disorders 14
4 journals.sagepub.com/home/tan
Figure 1. Preoperative T2-weighted MRI showing the intramedullary ependymoma (*) at Th 5 with
intertumoral hemorrhage and edema of the spinal cord () from C7-Th7 (a-c). T1-weighted MRI with contrast
showing the contrast enhancement of the ependymoma (d–g).
Figure 2. The spinal cord is exposed after dura opening and bulged due to the intramedullary tumor (a).
Myelotomy performed medially (b). The cranial and caudal boundary (see tidal flats) of the tumor is prepared
(c). The margins () of the grayish tumor (*) are well defined (d). Debulking of the tumor and piecemeal
removal using a CUSA with preservation of the surrounding spinal cord tissue (e–i). Spinal cord after complete
tumor removal (j).
O Gembruch, M Chihi et al.
journals.sagepub.com/home/tan 5
was presented in the majority of the cases (53.5%),
while back pain was described in 34.3%. In
12.1%, the symptom could not be specified by
the patients. Sensory deficits and motor deficits
were described in 14.2% and 9.5%. Gait distur-
bances were complained in 7.4%. Only two
patients (1.4%) reported sphincter and bladder
dysfunction. The main presenting symptom
depended on tumor location. Pain was more
often seen in patients with ependymomas located
in the lumbar spine (83.8%). Sensory deficits
(26.3%) and motor deficits (18.4%) are more
common in cervical spine ependymomas. Gait
disturbance was detected in cervical and thoracic
spine ependymomas, whereas sphincter and blad-
der dysfunction were observed in thoracic and
lumbar spine tumors (Graph 1).
The preoperative functional status according to
MCS was generally good (MCS I: 58.8% and
MCS II: 20.9%). Only a few patients presented
with severe neurological impairments (MCS III:
17.6% and MCS grade IV: 2.7%) prior to sur-
gery. Worse preoperative MCS (III + IV) was
seen more often in patients with cervical and tho-
racic ependymomas (Graph 2).
Surgery
Laminectomy was chosen in 47.3% of the cases
and mainly performed in the 1990s and the
early 2000s. Laminoplasty was the preferred
approach in the later phase of the observation
period.24 The GTR was achieved in 129 of cases
(87.2%), and the STR was performed in 19
cases (12.8%). Lumbar spine ependymomas
were most commonly resected via GTR (92.6%)
followed by cervical spine ependymomas
(86.8%) and thoracic spine ependymomas
(78.6%) (Table 1).
Surgical complications
A cerebrospinal fluid fistula prolonged the wound
healing in 9 (6.1%) of 148 patients. There was
no relation to the operative approach.
Laminectomy was used in five cases and lamino-
plasty in four cases. The tumor was located in the
cervical spine in two cases, in the thoracic spine
in one case, in the thoracic-lumbar region in
three cases, and in the lumbar spine in three
cases. However, none of these patients required
surgery and the fistula healed out completely
with conservative treatment.
Figure 3. Early postoperative T2-weighted MRI without contrast (a + d) and T1-weighted MRI with contrast
(b + c) 24 h after surgery with completely removed ependymoma. T1-weighted MRI with contrast (e + f) 6
months after surgery showing no tumor recurrence (*) despite the normal contrast enhancement at the dorsal
approach (#). T2-weighted MRI without contrast showing the postoperative changes of the spinal cord (*) and
the smaller edema of the spinal cord () (g).
Therapeutic Advances in Neurological Disorders 14
6 journals.sagepub.com/home/tan
Histopathology
Ependymomas (WHO grade I) were diagnosed in
47.3% and ependymomas (WHO grade II) in
50.0%. Anaplastic ependymoma was detected in
2.7%. Ependymomas (WHO grade I) were more
commonly located in the lumbar region, whereas
tumors (WHO grade II) were more often diag-
nosed in cervical spine (Table 1).
Recurrence
Tumor recurrence was detected in 12 patients
(8.1%) after a mean follow-up of 21.8 months
(range, 1 month–4.6 years). One patient developed
a spinal ependymoma 20 years after surgery at a
completely different spinal location. A tumor recur-
rence occurred in 3.9% (5/129 cases) after GTR
and in 36.8% after STR (7/119 cases), showing sig-
nificantly higher rates of recurrence than after GTR
(p = 0.0001). Histological examination confirmed
a benign tumor (WHO grade I) in five cases, a
semi-benign tumor (WHO grade II) in two cases
and anaplastic ependymoma (WHO grade III) in
five patients. All patients with recurrent tumor,
except one, who were treated using radiotherapy
after biopsy underwent a second surgery (Table 1).
Adjuvant therapy
Each case was discussed at the interdisciplinary
tumor board. Postoperative radiotherapy was
routinely offered to all patients with ependymo-
mas WHO grades II and III. Of these patients,
15.6% of the patients with a grade II epend-
ymoma (10/64 patients) and 100% of the patients
with a grade III ependymoma underwent postop-
erative radiotherapy (Table 1).
Neurological outcome according to the spine
level
Cervical spine. Preoperative MCS was ‘good’ in
71.1% and ‘poor’ in 28.91% (Table 2). Postoper-
ative neurological deterioration was seen in the
majority of the patients (63.2%). Of these, 40%
did not reach preoperative neurological status
(Graph 3). However, almost all patients (63.3%)
recovered to the previous ‘good’ preoperative sta-
tus. The ‘poor’ MCS consecutively decreased
from 63.2% postoperatively to 48.6% 6 months
and to 36.7% 36 months after surgery (Table 2).
Thoracic spine. Patients with thoracic spine epen-
dymoma presented with ‘good’ preoperative MCS
Table 1. Demographic, surgical, and tumor characteristics.
Patients’ characteristic P value
Number of patients 148
Age (years) 46.7 ± 15.3
Sex (female) 72 (48.6%)
Duration of symptoms (months) 29.4 ± 57.3
Tumor characteristic P value
Tumor location
Intramedullary 88 (59.5%)
Extramedullary 60 (40.5%)
Tumor location
Cervical 38 (25.7%)
Thoracic 42 (28.4%)
Lumbar 68 (45.9%)
WHO grade I 70 (47.3%)
WHO grade II 74 (50.0%)
WHO grade III 4 (2.7%)
Cervical: WHO grade (I/II/III) (7/30/1)/38 p = 0.0001
Thoracic: WHO grade (I/II/III) (17/24/1)/42
Lumbar: WHO grade (I/II/III) (46/20/2)/68
Tumor recurrence P value
Total tumor recurrence 12 (8.1%) p = 0.024
Cervical spine 0/38 (0%)
Thoracic spine 7/42 (16.7%)
Lumbar spine 5/68 (7.4%)
Tumor recurrence after GTR 5/129 (6.0%) p = 0.0001
Tumor recurrence after STR 7/19 (36.8%)
Surgical characteristics P value
Surgical approach
Laminoplasty 76 (51.4%)
Laminectomy 70 (47.3%)
Hemilaminectomy 2 (1.4%)
GTR 129/148 (87.2%)
Cervical Spine 33/38 (86.8%) p = 0.100
Thoracic Spine 33/42 (78.6%)
Lumbar Spine 63/68 (92.6%)
GTR, gross-total resection; STR, subtotal tumor resection; WHO, World Health
Organization.
O Gembruch, M Chihi et al.
journals.sagepub.com/home/tan 7
in 69.0%. The ‘good’ MCS decreased to 54.7%
postoperatively and increased to 61.8% 36 months
after surgery (Table 2). Neurological deterioration
was seen in 50.0% postoperatively, while 38.1%
remained stable and 11.9% reported direct post-
operative improvement in neurological deficits.
However, 36 months after surgery neurological
deterioration was still present in 29.4%. Preopera-
tive status was reached in 53.0%, while
neurological improvement compared with the pre-
operative status was detected in 17.6% (Graph 3).
Lumbar spine. In contrast to patients suffering
from cervical spine and thoracic spine ependymo-
mas, preoperative MCS was ‘good’ in general
(91.29%). A ‘poor’ preoperative MCS was only
detected in 8.8%. Postoperative MCS remained
‘good’ in the majority of the cases (92.6%). A
Graph 1. Main presenting symptoms according to tumor location.
Graph 2. Preoperative McCormick Score (I–IV) in relation to the affected spine level.
Therapeutic Advances in Neurological Disorders 14
8 journals.sagepub.com/home/tan
‘good’ MCS was seen in 92.0% 36 months after
surgery (Table 2).
Postoperative deterioration was only seen in
7.4%, while 85.2% showed stable neurological
status or improvement in status after surgery
Graph 3. Postoperative neurological status according to different spine levels.
Table 2. Postoperative functional outcome according to the MCS.
MCS Preoperative
n = 148
Postoperative
n = 148
3 months
postoperatively
n = 142
6 months
postoperatively
n = 141
12 months
postoperatively
n = 135
24 months
postoperatively
n = 131
36 months
postoperatively
n = 114
Cervical MCS I + II 27 (71.1%) 14 (36.8%) 17 (47.2%) 18 (51.4%) 21 (61.7%) 20 (60.6%) 19 (63.3%)
MCS III + IV 11 (28.9%) 24 (63.2%) 19 (52.8%) 17 (48.6%) 13 (38.3%) 13 (39.4%) 11 (36.7%)
Thoracic MCS I + II 29 (69.0%) 23 (54.7%) 24 (58.5%) 24 (58.5%) 25 (62.5%) 24 (61.5%) 21 (61.8%)
MCS III + IV 13 (31.0%) 19 (45.3%) 17 (41.5%) 17 (41.5%) 15 (37.5%) 15 (38.5%) 13 (38.2%)
Lumbar MCS I + II 62 (91.2%) 63 (92.6%) 60 (92.3%) 60 (92.3%) 57 (93.4%) 55 (93.2%) 46 (92.0%)
MCS III + IV 6 (8.8%) 5 (7.4%) 5 (7.7%) 5 (7.7%) 4 (6.6%) 4 (6.8%) 4 (8.0%)
MCS, McCormick Score.
O Gembruch, M Chihi et al.
journals.sagepub.com/home/tan 9
(7.4%). Neurological deterioration was observed
in only 6% 36 months after surgery, while 86.0%
remained unchanged or improved their neuro-
logical status (8.0%) (Graph 3).
Analysis of possible predictors of neurological
outcome
Complete cohort. Univariate analysis of the total
cohort revealed that poor preoperative functional
condition (MCS > 2), WHO grades II and III,
tumor volume (cm3), spine segment (cervical and
thoracic spine), tumor extension >2 vertebras,
and STR had a high significant impact on poor
neurological outcome (p < 0.05).
In addition, neurological symptoms (pain, pare-
sis, and ataxia) at onset of the disease were nega-
tively associated with postoperative outcome
(p < 0.05), whereas sensory disorders had no
effect (p > 0.05) on patients’ outcome. Symptom
duration also showed no significant correlation
with poor neurological outcome (p > 0.05)
(Supplementary Table 3).
Multivariate analysis confirmed that preoperative
status (MSC >2) was a negative predictor of neu-
rological outcome at all analyzed time points.
Tumor extension >2 vertebrae was a negative pre-
dictor until 24 months after surgery. In addition,
STR was also a negative predictor 36 months after
surgery (p < 0.05) (Supplementary Table 3).
Cervical and thoracal spine ependymomas. The
univariate analysis demonstrated that preopera-
tive MCS >2 and tumor extension (>2 verte-
brae) had significant association with poor
neurological outcome at every evaluated time
points (Supplementary Table 4).
The multivariate analysis showed an important
impact of tumor extension >2 vertebrae on a
poor functional outcome (postoperatively: 24
months after surgery (Supplementary Table 4).
Lumbar ependymomas. The univariate analysis
of the lumbar ependymomas revealed preopera-
tive MCS >2, pain, tumor extension (>2 verte-
brae), and ataxia as potential predictive factors of
poor neurological outcome (p < 0.05) (Supple-
mentary Table 5).
Multivariate analysis revealed that preoperative
MCS was a predictive factor postoperatively
(p < 0.05). However, quality of this subgroup
was limited due to the low number of evaluated
patients and lost to follow-up 24 and 36 months
after surgery (Supplementary Table 5).
Discussion
The surgical treatment of spinal ependymomas
remains challenging, as postoperative neurologi-
cal deterioration plays a key role in prognosis. Up
to now, several authors presented their surgical
experience and their recommendation for surgical
tumor removal or mass reduction.2,4,7,25,26
The known postoperative neurological deteriora-
tion of the majority of patients makes it necessary
to define possible prognostic factors for functional
outcome. Therefore, we tried to present our sin-
gle-center experience on the surgical treatment of
spinal cord ependymomas and evaluated possible
prognostic factors for neurological deterioration.
This single cohort outnumbers cohorts published
in literature.
A mild predominance of males (52.4%) was detected
in our study. The frequency of males ranged from
62% to 82% in other series.20,22,25 Ependymomas
were mostly located in the lumbar region (45.9%).
Lumbar ependymomas were described in 2.9–
29.1% of the cases in the literature.27,28
Symptoms caused by the spinal ependymoma are
unspecific. The most common symptom was
pain in 67.6% of the cases, followed by sensory
deficits in 14.2% and motor weakness in 9.5%.
These results are similar to previously pub-
lished reports.6,18,29 Symptom duration until
surgery was, on average, 29.4 months. Late
diagnosis is caused by the non-specificity of
symptoms.29,30 Furthermore, authors described
a misinterpretation of symptoms such as back
pain or slow deterioration of other neurological
symptoms with resulting lack of differential diag-
nosis, including a slow-growing spinal tumor.31
In addition, patients can adopt the slow worsen-
ing of neurological deficits at the early stage or
comorbidities can cover these symptoms.
Nevertheless, at the time of diagnosis, a consid-
erable number of patients showed severe neuro-
logical deficits (MCS III = 17.6%) or were not
able to walk (MCS IV = 2.7%). Boström etal.18
reported about 2% and Klekamp6 reported about
11.2% of patients who were unable to walk pre-
operatively. Li et al.25 reported about 26.2% of
Therapeutic Advances in Neurological Disorders 14
10 journals.sagepub.com/home/tan
patients with MCS III and 6.2% of patients with
MCS IV in a series of 210 patients suffering from
a spinal cord ependymoma.
Surgical treatment and tumor recurrence
In our series, complete resection of spinal cord
ependymomas was seen in the early postoperative
MRI in 87.2%, showing comparable results to the
literature.2,5,20,25,32 However, GTR was most
commonly achieved after tumor removal within
the lumbar spine followed by the cervical spine.
Rate of GTR was lowest in thoracic spine epend-
ymomas. This might be caused by the anatomical
differences between the spine segments. In the
current literature, GTR, encapsulated tumors,
and postoperative radiotherapy are reported as
the most important prognostic factors for pro-
gression-free survival.2,5–7,15,29,33 Tumor recur-
rence was detected in 8.1% of the patients after a
median of 21.8 months, leading to consecutive
impairment of the spinal cord. This was also
reported by Samii and Klekamp, who described
higher rates of neurological deterioration in STR.
The regrowth of tumor remnants was proposed as
a possible explanation.34 However, the only sig-
nificant predictor of recurrence-free survival is
the degree of resection.18 In addition, it has been
well established that an early start of adjuvant
treatment after non-total resection prolongs pro-
gression-free survival.35
Functional outcome
Change in surgical approach (laminectomy at the
beginning of the period versus laminoplasty24 from
the early 2000s onward) during the treatment
period had no impact on neurological outcome.5
Neurological deterioration after surgery was
most common in cervical and thoracic spine
ependymomas due to the anatomical differences
between the lumbar spine. Neurological deteriora-
tion after surgery was present in 63.2% of the cer-
vical spine ependymomas and in 50.0% of the
thoracic spine ependymomas compared with a
worsening of 7.4% of the lumbar spine ependymo-
mas. However, direct postoperative improvement
of the neurological function was detected in 5.2%,
11.9%, and 7.4%, respectively. Neurological
improvement after rehabilitation 36 months after
surgery was seen especially in thoracic spine epend-
ymomas. Of those, 53% reached the preoperative
status, while 17.6% were better than preoperative
status. Thoracic spine ependymomas showed poor
MCS after 36 months in 61.8%. Some authors
propose that the anatomy of the spinal cord
(smaller volume of the thoracic spinal cord, com-
pared with the cervical spinal cord) is reasonable
for the worse recovery.6 However, neurological
improvement might be also influenced by tumor
recurrence at the time of follow-up. In our analy-
sis, tumor recurrence was observed after 21.8
months. Nevertheless, good MCS was observed in
the majority of lumbar spine ependymomas and in
63.3% of the cervical and 61.8% of the thoracic
spine ependymomas 36 months after surgery.
Cervical spine ependymomas presented less
improvement than thoracic spine ependymomas.
This is comparable with the findings of Klekamp,6
who described neurological deterioration of 67.5%
of the patients with intramedullary ependymomas
and of 16.6% of the patients with filum terminale
ependymomas.27 Transient neurological deteriora-
tion was evaluated in 40% and 8.3%, respec-
tively.6,27 Similar to our study, Klekamp6 found a
higher rate of postoperative permanent morbidity
in patients with tumors of thoracic spine.
Predictors of poor neurological outcome
However, there are some factors with potential risk
for permanent neurological deficits. In our series
as well as in others, the preoperative neurological
status bears an increased risk of poor neurological
outcome.6,7,18,20,36,37 Preoperative neurological def-
icit reflects the damage of the spinal cord caused
by the tumor, and the operative procedure
increases this damage. In addition, regeneration of
the neurological function is limited, especially if
the preoperative poor neurological status persists
over a longer time. Early diagnosis and referral to
specialized surgical centers might also improve the
postoperative outcome. Epstein etal. also call for
early surgery based on their findings.
Another predictive factor for poor neurological
outcome until follow-up of 24 months is tumor
extension >2 vertebrae. The extent of tumor
resection and the consecutive injury of the spinal
cord may be reasonable. Prokopienko et al.20
showed in their study that neurological outcome
is worse in tumors extending over three spinal lev-
els. Wang et al.38 evaluated that tumor size is a
predictive factor for worse neurological outcome
if the tumor is larger than 4 cm.
Tumor location was also a predictor of poor neu-
rological outcome. Tumors located in the cervical
O Gembruch, M Chihi et al.
journals.sagepub.com/home/tan 11
and thoracic spine were less likely to achieve good
neurological outcome compared with lumbar
spine ependymomas. These findings are similar
to the current literature.39 For example, Samuel
etal.19 showed similar results analyzing treatment
of 63 patients with intramedullary spinal cord
tumors. Interestingly, Wostrack etal.21 evaluated
that cervically located ependymomas causing
transient deficits were more frequent but failed to
demonstrate that cervical tumor location is a pre-
dictor of permanent neurological deficits.
Finally, STR showed a tendency to be a predictor
of neurological outcome 12 and 24 months after
surgery. It was detected as a negative predictor of
neurological outcome 36 months after surgery.
The majority of tumor recurrence occurred in
patients with STR after a mean follow-up of 21.8
months. In those cases, second surgery was
offered to the patients and a second neurological
deterioration was detected.
Study limitations
Various limitations must be addressed. First, this
is a retrospective, non-randomized study with its
associated inherent bias. Second, data were ana-
lyzed from our retrospective electronic database
‘spinal neoplasm’, in which the patient’s elec-
tronic data, surgical reports, and radiological data
were collected. Nevertheless, incomplete data
bear an additional limitation and risk of selection
bias. Univariant and multivariant analysis was not
useful due to the ongoing lost to follow-up 36
months after surgery, caused by the retrospective
character of the study. In addition, the study
encompassed a long epoch in time, in which
patients were treated by different neurosurgeons
creating another confounding factor.
Furthermore, some surgical techniques have
changed over these years in terms of minimally
invasive approaches or the use of intraoperative
electrophysiology. In addition, lower image qual-
ity of MRI during the beginning of the observa-
tional study has to be acknowledged and might
have influenced the quality of the data.
Conclusion
Spinal cord ependymomas present different clini-
cal features according to their location. The surgi-
cal treatment of these tumors is associated with a
considerable risk of postoperative neurological
deterioration, which is most common in cervical
and thoracal spine ependymomas. However,
postoperative improvement is likely in half of
these patients.
The preoperative neurological status, tumor loca-
tion at the cervical spine, and STR are negative
predictors of the postoperative MCS. Therefore,
the surgical treatment of spinal cord ependymo-
mas before further neurological deterioration is
recommended.
Acknowledgements
We acknowledge support by the Open Access
Publication Fund of the University of
Duisburg-Essen.
Author contributions
Oliver Gembruch was involved in conception and
design of the study; drafting, analysis, and inter-
pretation of data; drafting the article; and revision
and final approval of the version to be submitted.
Mehdi Chihi was involved in analysis and interpre-
tation of data; statistics; and revision and final
approval of the version to be submitted. Merle
Haarmann was involved in drafting, analysis, and
interpretation of data; and revision and final
approval of the version to be submitted. Ahmet
Parlak was involved in analysis and interpretation
of data, and revision and final approval of the ver-
sion to be submitted. Marvin Darkwah Oppong
was involved in analysis and interpretation of data,
and revision and final approval of the version to be
submitted. Laurèl Rauschenbach was involved in
analysis and interpretation of data, and revision
and final approval of the version to be submitted.
Anna Michel was involved in analysis and interpre-
tation of data, and revision and final approval of
the version to be submitted. Ramazan Jabbarli was
involved in revising it critically for important intel-
lectual content and final approval of the version to
be submitted. Yahya Ahmadipour was involved in
revising it critically for important intellectual con-
tent and final approval of the version to be submit-
ted. Ulrich Sure was involved in revising it critically
for important intellectual content and final
approval of the version to be submitted. Philipp
Dammann was involved in revising it critically for
important intellectual content and final approval
of the version to be submitted. Neriman Özkan
was involved in conception and design of the study;
analysis and interpretation of data; and revision
and final approval of the version to be submitted.
Therapeutic Advances in Neurological Disorders 14
12 journals.sagepub.com/home/tan
Conflict of interest statement
The authors declared no potential conflicts of
interest with respect to the research, authorship,
and/or publication of this article.
Funding
The authors received no financial support for the
research, authorship, and/or publication of this
article.
Ethics statement
The study was conducted in accordance with the
Strengthening the Reporting of Observational
Studies in Epidemiology (STROBE) guidelines
after the approval by the Institutional Review
Board (Medical Faculty, University of Duisburg-
Essen, Registration Number: 16-7178-BO), and
followed The Code of Ethics of the World
Medical Association (Declaration of Helsinki).
Written informed consent was not needed due to
the retrospective character of the study and the
approval of Institutional Review Board.
ORCID iDs
Oliver Gembruch https://orcid.org/0000-
0002-0054-1611
Marvin Darkwah Oppong https://orcid.org/
0000-0003-1021-5024
Supplemental material
Supplemental material for this article is available
online.
References
1. Benesch M, Frappaz D and Massimino M. Spinal
cord ependymomas in children and adolescents.
Childs Nerv Syst 2012; 28: 2017–2028. DOI:
10.1007/s00381-012-1908-4.
2. Brotchi J and Fischer G. Spinal cord
ependymomas. Neurosurg Focus 1998; 4: e2. DOI:
10.3171/foc.1998.4.5.5.
3. Eroes CA, Zausinger S, Kreth FW, etal.
Intramedullary low grade astrocytoma and
ependymoma. Surgical results and predicting
factors for clinical outcome. Acta Neurochir
(Wien) 2010; 152: 611–618. DOI: 10.1007/
s00701-009-0577-x.
4. Ruda R, Gilbert M and Soffietti R.
Ependymomas of the adult: molecular biology
and treatment. Curr Opin Neurol 2008; 21: 754–
761. DOI: 10.1097/WCO.0b013e328317efe8.
5. Sandalcioglu IE, Gasser T, Asgari S, etal.
Functional outcome after surgical treatment of
intramedullary spinal cord tumors: experience
with 78 patients. Spinal Cord 2005; 43: 34–41.
DOI: 10.1038/sj.sc.3101668.
6. Klekamp J. Spinal ependymomas.
Part 1: intramedullary ependymomas.
Neurosurg Focus 2015; 39: E6. DOI:
10.3171/2015.5.FOCUS15161.
7. Lee SH, Chung CK, Kim CH, etal. Long-term
outcomes of surgical resection with or without
adjuvant radiation therapy for treatment of spinal
ependymoma: a retrospective multicenter study
by the Korea Spinal Oncology Research Group.
Neuro Oncol 2013; 15: 921–929. DOI: 10.1093/
neuonc/not038.
8. Oh MC, Tarapore PE, Kim JM, etal. Spinal
ependymomas: benefits of extent of resection
for different histological grades. J Clin Neurosci
2013; 20: 1390–1397. DOI: 10.1016/j.
jocn.2012.12.010.
9. Celano E, Salehani A, Malcolm JG, etal. Spinal
cord ependymoma: a review of the literature
and case series of ten patients. J Neurooncol
2016; 128: 377–386. DOI: 10.1007/s11060-
016-2135-8.
10. Chang UK, Choe WJ, Chung SK, etal.
Surgical outcome and prognostic factors
of spinal intramedullary ependymomas in
adults. J Neurooncol 2002; 57: 133–139. DOI:
10.1023/a:1015789009058.
11. Al-Habib A, Al-Radi OO, Shannon P, etal.
Myxopapillary ependymoma: correlation of
clinical and imaging features with surgical
resectability in a series with long-term follow-up.
Spinal Cord 2011; 49: 1073–1078. DOI: 10.1038/
sc.2011.67.
12. Patel P, Mehendiratta D, Bhambhu V, etal.
Clinical outcome of intradural extramedullary
spinal cord tumors: a single-center retrospective
analytical study. Surg Neurol Int 2021; 12: 145.
DOI: 10.25259/SNI_839_2020.
13. Hubner JM, Kool M, Pfister SM, etal.
Epidemiology, molecular classification and
WHO grading of ependymoma. J Neurosurg
Sci 2018; 62: 46–50. DOI: 10.23736/S0390-
5616.17.04152-2.
14. Payne NS II and McDonald JV. Rupture
of spinal cord ependymoma. Case report. J
Neurosurg 1973; 39: 662–665. DOI: 10.3171/
jns.1973.39.5.0662.
15. Schwartz TH and McCormick PC.
Intramedullary ependymomas: clinical
O Gembruch, M Chihi et al.
journals.sagepub.com/home/tan 13
presentation, surgical treatment strategies and
prognosis. J Neurooncol 2000; 47: 211–218. DOI:
10.1023/a:1006414405305.
16. Louis DN, Perry A, Reifenberger G, etal. The
2016 World Health Organization classification of
tumors of the central nervous system: a summary.
Acta Neuropathol 2016; 131: 803–820. DOI:
10.1007/s00401-016-1545-1.
17. Bostrom A, Kanther NC, Grote A, etal.
Management and outcome in adult
intramedullary spinal cord tumours: a 20-year
single institution experience. BMC Res Notes
2014; 7: 908. DOI: 10.1186/1756-0500-7-908.
18. Bostrom A, von Lehe M, Hartmann W, etal.
Surgery for spinal cord ependymomas: outcome
and prognostic factors. Neurosurgery 2011;
68: 302–308; discussion 309. DOI: 10.1227/
NEU.0b013e3182004c1e.
19. Samuel N, Tetreault L, Santaguida C, etal.
Clinical and pathological outcomes after resection
of intramedullary spinal cord tumors: a single-
institution case series. Neurosurg Focus 2016; 41:
E8. DOI: 10.3171/2016.5.FOCUS16147.
20. Prokopienko M, Kunert P, Podgorska A,
etal. Surgical treatment of intramedullary
ependymomas. Neurol Neurochir Pol 2017; 51:
439–445. DOI: 10.1016/j.pjnns.2017.06.008.
21. Wostrack M, Ringel F, Eicker SO,
etal. Spinal ependymoma in adults:
a multicenter investigation of surgical
outcome and progression-free survival. J
Neurosurg Spine 2018; 28: 654–662. DOI:
10.3171/2017.9.SPINE17494.
22. McCormick PC, Torres R, Post KD, etal.
Intramedullary ependymoma of the spinal cord.
J Neurosurg 1990; 72: 523–532. DOI: 10.3171/
jns.1990.72.4.0523.
23. Svoboda N, Bradac O, de Lacy P, etal.
Intramedullary ependymoma: long-term outcome
after surgery. Acta Neurochir (Wien) 2018; 160:
439–447. DOI: 10.1007/s00701-017-3430-7.
24. Wiedemayer H, Sandalcioglu IE, Aalders M,
etal. Reconstruction of the laminar roof with
miniplates for a posterior approach in intraspinal
surgery: technical considerations and critical
evaluation of follow-up results. Spine (Phila Pa
1976) 2004; 29: E333–342. DOI: 10.1097/
01.brs.0000134592.07941.5e.
25. Li TY, Chu JS, Xu YL, etal. Surgical strategies
and outcomes of spinal ependymomas of different
lengths: analysis of 210 patients: clinical article.
J Neurosurg Spine 2014; 21: 249–259. DOI:
10.3171/2014.3.SPINE13481.
26. Sun XY, Wang W, Zhang TT, etal. Factors
associated with postoperative outcomes
in patients with intramedullary Grade II
ependymomas: a systematic review and meta-
analysis. Medicine (Baltimore) 2019; 98: e16185.
DOI: 10.1097/MD.0000000000016185.
27. Klekamp J. Spinal ependymomas. Part
2: ependymomas of the filum terminale.
Neurosurg Focus 2015; 39: E7. DOI:
10.3171/2015.5.FOCUS15151.
28. Sun XY, Kong C, Lu SB, etal. Survival
outcomes and prognostic factors of patients
with intramedullary Grade II ependymomas
after surgical treatments. J Clin Neurosci 2018;
57: 136–142. DOI: 10.1016/j.jocn.2018
.08.001.
29. Engelhard HH, Villano JL, Porter KR, etal.
Clinical presentation, histology, and treatment
in 430 patients with primary tumors of the
spinal cord, spinal meninges, or cauda equina.
J Neurosurg Spine 2010; 13: 67–77. DOI:
10.3171/2010.3.SPINE09430.
30. Tarapore PE, Modera P, Naujokas A,
etal. Pathology of spinal ependymomas:
an institutional experience over 25 years
in 134 patients. Neurosurgery 2013; 73:
247–255; discussion 255. DOI: 10.1227/01.
neu.0000430764.02973.78.
31. Pena M, Galasko CS and Barrie JL. Delay in
diagnosis of intradural spinal tumors. Spine
(Phila Pa 1976) 1992; 17: 1110–1116. DOI:
10.1097/00007632-199209000-00017.
32. Kobayashi K, Ando K, Kato F, etal. Surgical
outcomes of spinal cord and cauda equina
ependymoma: postoperative motor status and
recurrence for each WHO grade in a multicenter
study. J Orthop Sci 2018; 23: 614–621. DOI:
10.1016/j.jos.2018.03.004.
33. Halvorsen CM, Kolstad F, Hald J, etal. Long-
term outcome after resection of intraspinal
ependymomas: report of 86 consecutive cases.
Neurosurgery 2010; 67: 1622–1631; discussion
1631. DOI: 10.1227/NEU.0b013e3181f96d41.
34. Samii M and Klekamp J. Surgical results
of 100 intramedullary tumors in relation to
accompanying syringomyelia. Neurosurgery
1994; 35: 865–873; discussion 873. DOI:
10.1227/00006123-199411000-00010.
35. Lin YH, Huang CI, Wong TT, etal. Treatment
of spinal cord ependymomas by surgery with or
without postoperative radiotherapy. J Neurooncol
2005; 71: 205–210. DOI: 10.1007/s11060-004-
1386-y.
Therapeutic Advances in Neurological Disorders 14
14 journals.sagepub.com/home/tan
36. McCormick PC and Stein BM. Intramedullary
tumors in adults. Neurosurg Clin N Am 1990; 1:
609–630.
37. Yang C, Sun J, Xie J, etal. Multisegmental
versus monosegmental intramedullary spinal
cord ependymomas: perioperative neurological
functions and surgical outcomes. Neurosurg Rev.
Epub ahead of print 1 November 2021. DOI:
10.1007/s10143-021-01567-5.
38. Wang X, Gao J, Wang T, etal. The long-term
outcome after resection of upper cervical spinal cord
tumors: report of 51 consecutive cases. Sci Rep 2018;
8: 14831. DOI: 10.1038/s41598-018-33263-8.
39. Aghakhani N, Messerer M, David P, etal.
[Intramedullary ependymomas: a French
retrospective multicenter study of 221 cases].
Neurochirurgie 2017; 63: 391–397. DOI:
10.1016/j.neuchi.2016.07.002.
Visit SAGE journals online
journals.sagepub.com/
home/tan
SAGE journals
... The patient's pre-operative neurologic and functional status are important for optimal postoperative outcomes in the case of spinal ependymomas (1). The tumor location has been shown to contribute to surgical morbidity and weaker results were reported for thoracic ependymomas as compared to other location (5,21). In this case the patient benefited from a gross total resection (GTR) of the tumor mass. ...
Article
Full-text available
Spinal ependymomas are a group of mostly slow-growing tumors that can cause non-traumatic spinal cord injury with insidious clinical symptomatology ranging from neck or back pain to as-sociated sensory-motor impairment. Due to their neurological and systemic frailty, patients with spinal cord injury might be especially vulnerable to the effects of SARS-CoV-2 infection and the resulting respiratory impairment. CASE PRESENTATION: We present the case of a 66 year old women admitted in our Neuromuscular Rehabilitation Clinic Division for severe in-complete paraplegia and neurogenic bladder. She was previously diagnosed in the Neurosurgi-cal Ward with a thoracic grade II (classic) ependymoma and underwent a gross tumor resection. During the neurorehabilitation program the patient was diagnosed with COVID-19. The multi-drug related treatments were associated with supportive oxygen therapy and neuromuscular and respiratory rehabilitation techniques. RESULTS: Despite of the patient’s favorable rehabili-tation, consisting of walking abilities with support in a walking frame on short distances, her prognosis may be worsened by resting neurogenic bladder symptoms. COVID-19 has led to im-portant acute respiratory morbidity in our patient, regardless of the mild course of the disease, and might further cause a post-infectious respiratory impairment. ONCLUSION: Spinal cord in-jury remains a life-long condition and emphasizes the necessity of supporting the affected pati-ents on the long run. Keywords: students, balance, postural stability, physical exercise, progress, motor skills
Article
Full-text available
Multiple factors, such as tumor size, lateralization, tumor location, accompanying syringomyelia, and regional spinal cord atrophy, may affect the resectability and clinical prognosis of intramedullary spinal cord ependymomas. However, whether long-segmental involvement of the spinal cord may impair functional outcomes remains unclear. This study was aimed to compare perioperative neurological functions and long-term surgical outcomes between multisegmental ependymomas and their monosegmental counterparts. A total of 62 patients with intramedullary spinal cord ependymoma (WHO grade II) were enrolled, and all of them underwent surgical resection. The patients were classified into the multisegmental group (n = 43) and the monosegmental group (n = 19). Perioperative and long-term (average follow-up period, 47.3 ± 21.4 months) neurological functions were evaluated using the modified McCormick (mMC) scale and the modified Japanese Orthopaedic Association (mJOA) scoring system. Preoperative neurological functions in the multisegmental group were significantly worse than those in the monosegmental group (P < 0.05). However, postoperative short-term neurological functions, as well as long-term functional outcomes, were similar between the two groups (P > 0.05). Logistic regression analysis showed that preoperative mMC and mJOA scores were significantly correlated with neurological improvement during the follow-up period (P < 0.05). Multisegmental involvement of the spinal cord is associated with worse neurological functions in patients with intramedullary spinal cord ependymoma, while the long-term prognosis is not affected. The preoperative neurological status of the patient is the only predictor of long-term functional improvement.
Article
Full-text available
Background Intradural extramedullary spinal cord tumors (IESCT) account for approximately two-thirds of largely benign intraspinal neoplasms. They occasionally present with acute neurological deterioration warranting emergent surgical intervention. Methods Here, we reviewed a series of 31 patients with intradural extramedullary spinal tumors who underwent surgery from 2012 to 2019. Patients averaged 50.8 years of age, and there were 16 males and 15 females. Patients were followed for a minimum of 1 year. Multiple clinical outcome variables were studied (e.g., Karnofsky Performance Score [KPS], visual analog scale (VAS), and Frankel grade). Results The majority of IESCT tumors were found in the thoracic spine 18 (58.06%) followed by the lumbar 8 (25.80%), cervical 1 (3.22%), and combined junctional tumors 4 (12.90%) (cervicothoracic-02 and thoracolumbar-02). Histopathological diagnoses included schwannomas-16 (51.61%), meningiomas-11 (35.48%), lipomas-2 (6.45%), hemangiomas-1 (3.22), and ependymomas-01 (03.22%). The VAS score was reduced in all cases, while KPS and Frankel grades were significantly improved. Complications included cerebrospinal fluid leakage, new/residual paresthesias, and tumor recurrence (12.50%). Conclusion Most intradural extramedullary tumors are benign and are readily diagnosed utilizing MRI scans. Notably, good functional outcomes follow surgical intervention.
Article
Full-text available
Background: Most of the previous studies combined all types of intramedullary ependymomas without providing accurate pathological subtypes. In addition, it was very difficult to evaluate the factors associated with postoperative outcomes of patients with different pathological subtypes of intramedullary Grade II ependymomas by traditional meta-analysis. This study evaluated the factors related with postoperative outcomes of patients with intramedullary Grade II ependymomas. Methods: Individual patient data analysis was performed using PubMed, Embase, and the Cochrane Central Register of Controlled Trials. The search included articles published up to April 2018 with no lower date limit on the search results. The topics were intramedullary Grade II ependymomas. Progression-free survival (PFS) and overall survival (OS) were analyzed by Kaplan-Meier survival analysis (log-rank test). The level of significance was set at P < .05. Results: A total of 21 studies with 70 patients were included in this article. PFS of patients who underwent total resection was much longer than the PFS of those who received subtotal resection (P < .001). Patients who received adjuvant therapy (P = .005) or radiotherapy and chemotherapy (P < .001) seemed to have shorter PFS than others; PFS of patients who had cerebrospinal fluid disease dissemination (P = .022) or scoliosis (P = .001) were significantly shorter than others. OS of cellular ependymoma patients was less than giant cell ependymoma patients (P < .001). Conclusions: PFS of patients who received total resection was much longer than those who received subtotal resection. Patients treated with adjuvant therapy or radiotherapy and chemotherapy appeared to have shorter PFS than others; PFS of patients with cerebrospinal fluid disease dissemination or scoliosis were significantly shorter than others. Cellular ependymomas would have better OS than giant cell ependymoma. However, giant cell ependymoma patients might have the worst OS.
Article
Full-text available
The literature discussing the long-term outcome after resection of upper cervical spinal cord tumors is limited. The purpose of this study was to review the progression-free survival (PFS), overall survival (OS), and long-term outcomes in a consecutive series of 51 patients with upper cervical spinal cord tumors who underwent surgery at our institution between 2005 and 2010. Patient outcome were evaluated using the Japanese Orthopaedic Association score (JOA) and the McCormick functional schema. Follow-up data was collected completely and the median follow-up time was 6.1 years. Gross total resection (GTR) was performed in 27 patients (52.94%) and subtotal resection (STR) in 24 patients (47.06%). Progression-free survival and overall survival at 5 years was 88.23% and 92.16%, respectively. Good prognosis was defined as 74.51% based on JOA scoring. The univariate analysis showed that patients over 60y, tumors located higher than C2, tumor size greater than 4 cm as well as malignant tumors and subtotal resection were factors indicating a poor prognosis. However, the multivariate regression analyses showed only the level of tumor and tumor size were independent risk factors for a poor prognosis. The gold standard treatment for intraspinal tumors is gross total resection and follow-up should be focused on patients with a high risk of poor prognosis.
Article
Full-text available
Background: Overall (OS) and progression-free survival (PFS) of patients undergoing spinal ependymoma resection has been frequently reported. Contrarily, OS and PFS of purely intramedullary ependymomas have not been clearly determined yet. Methods: The data of 37 patients undergoing resection of an intramedullary ependymoma (IE) from January 2000 to December 2016 were analysed retrospectively. Results: The mean age was 46 years. The male:female ratio was 24:13. The median duration of symptoms was 12 months. Sixty-two per cent of ependymomas were in the cervical, 24% in the thoracic, and 14% in the conus region in our series. The median volume was 1.3 ml. A syrinx was found in 49% and a cyst in 32%. GTR was achieved in 89%, STR in three (8%), and PR in one patient (3%). Median follow-up was 114 months. PFS was 87%, 82%, and 82% at 5, 10, and 15 years, respectively. OS was 97%, 88%, and 63% at 5, 10, and 15 years, respectively. There was a significant difference in PFS depending on the extent of resection and in OS depending on the pre-operative clinical status. There was no significant difference in OS and PFS regarding the other examined influencing factors. Conclusion: GTR resection was the most important factor influencing PFS. According to our results OS of IEs is much worse than that of spinal ependymomas. Our analysis confirms that patients with good pre-operative (McCormick grade 1 and 2) clinical status have significantly better OS than patients with McCormick grade 3 and higher.
Article
This study evaluated survival outcomes of patients with intramedullary Grade II ependymomas and identify prognostic factors. Electronic searches of PubMed, EMBASE, OVID, the Cochrane Central Register of Controlled Trials were performed to identify trials according to the Cochrane Collaboration guidelines. The objects were intramedullary Grade II ependymoma according to 2007 WHO classification. Kaplan-Meier survival analysis with log-rank test was used to analyze progressive free survival (PFS) and overall survival (OS). Cox proportional hazard model was utilized for multivariate analysis with hazard ratio (HR) and 95% confidence interval (CI) calculated. P values <0.05 were considered statistically significant. A total of 28 studies including 138 cases of intramedullary Grade II ependymomas were retrieved. Patients who were classified as cellular ependymomas or papillary ependymomas had higher risks of progression than those who possessed typical Grade II ependymomas. Patients who were treated with adjuvant therapy had a higher risk of progression than those without adjuvant therapy. OS of patients with giant cell ependymoma was significantly shorter than those with typical Grade II ependymoma. Patients who had cellular or papillary subtype, adjuvant therapy would have a shorter estimated value of progression-free time and a higher risk of progression than typical Grade II ependymomas. Giant cell ependymoma patients would have a higher risk of fatality than those with typical Grade II ependymomas. Definite pathology type and appropriate treatments were foundations of intramedullary Grade II ependymomas' managements.
Article
Background The goals of the study are to analyze postoperative outcomes and recurrence in cases of spinal cord and cauda equina ependymoma in each World Health Organization (WHO) Grade, and to examine the influence of extent of surgical removal on prognosis. Spinal ependymoma has a relatively high frequency among intramedullary spinal cord tumors. The tumor is classified in WHO guidelines as grades I, II, and III, but few studies have examined postoperative prognosis based on these grades. Methods The records of 80 patients undergoing surgery for spinal cord and cauda equina ependymoma were examined in a multicenter study using a retrospective database. Neurological motor status, pathological type, extent of resection, and tumor recurrence were evaluated. Results The histopathological types were grade I in 23 cases (myxopapillary: 21, subependymoma: 2), grade II in 52 cases, and grade III in 5 cases (including all anaplastic cases). Total resection was performed in 60 cases (83%), and eight cases had recurrence, including 2 in WHO grade I, 2 in grade II, and 4 in grade III. The 5-year recurrence-free survival rates were 90%, 91%, and 20% in grades I, II and III, respectively. Adjuvant radiotherapy for the local site was performed in 8 cases, including 3 in grade I and 5 in grade III; however, 4 of the 5 grade III cases (80%) had recurrence after radiotherapy. Among 59 patients with normal ambulation or independence without external assistance (McCormick Grade I or II), 53 (90%) maintained the same mobility after surgery. In cases that underwent total resection, the recurrence rate was significantly lower (p < 0.01). A good preoperative motor status also resulted in significantly better postoperative recovery of motor status (p < 0.05). Conclusions Total resection of spinal cord and cauda equina ependymoma leads to postoperative motor recovery and may reduce tumor recurrence. Therefore, early surgery for this tumor is recommended before aggravation of paralysis.
Article
OBJECTIVE Spinal ependymomas are rare glial neoplasms. Because their incidence is low, only a few larger studies have investigated this condition. There are no clear data concerning prognosis and therapy. The aim of the study was to describe the natural history, perioperative clinical course, and local tumor control of adult patients with spinal ependymomas who were surgically treated under modern treatment standards. METHODS The authors performed a multicenter retrospective study. They identified 158 adult patients with spinal ependymomas who had received surgical treatment between January 2006 and June 2013. The authors analyzed the clinical and histological aspects of these cases to identify the predictive factors for postoperative morbidity, tumor resectability, and recurrence. RESULTS Gross-total resection (GTR) was achieved in 80% of cases. At discharge, 37% of the patients showed a neurological decline. During follow-up the majority recovered, whereas 76% showed at least preoperative status. Permanent functional deterioration remained in 2% of the patients. Transient deficits were more frequent in patients with cervically located ependymomas (p = 0.004) and in older patients (p = 0.002). Permanent deficits were independently predicted only by older age (p = 0.026). Tumor progression was observed in 15 cases. The 5-year progression-free survival (PFS) rate was 80%, and GTR (p = 0.037), WHO grade II (p = 0.009), and low Ki-67 index (p = 0.005) were independent prognostic factors for PFS. Adjuvant radiation therapy was performed in 15 cases. No statistically relevant effects of radiation therapy were observed among patients with incompletely resected ependymomas (p = 0.079). CONCLUSIONS Due to its beneficial value for PFS, GTR is important in the treatment of spinal ependymoma. Gross-total resection is feasible in the majority of cases, with acceptable rates of permanent deficits. Also, Ki-67 appears to be an important prognostic factor and should be included in a grading scheme for spinal ependymomas.
Article
Ependymoma can arise throughout all compartments of the central nervous system with prevalence for intracranial and spinal location in children and adults, respectively. The current histopathology based WHO grading system distinguishes grade I, II 'classic', and III 'anaplastic' ependymoma. However, analysis of multiple cohorts of intracranial ependymoma demonstrate a wide variance in the utility of the grade II versus grade III distinction as a prognostic marker that may additionally be confounded by the anatomic compartment. Recent (epi)genomic profiling efforts have identified molecularly distinct groups of ependymoma arising from all three anatomic compartments of the central nervous system that outperform the current histopathological classification regarding clinical associations. These advances have led to the cognition that molecular classification should be part of all future clinical trials in ependymoma patients. Clinical management of intracranial ependymomas (WHO Grade II/III) is challenging and molecular classification based risk stratification may help to intensify treatment and surveillance in high-risk patients but to de-escalate therapy in certain patient groups at low risk for recurrence. Finally, experience of neurosurgeons, and other disciplines, as well as intensified co-operation between all stakeholders involved hold promise to finally improve outcome of patients affected with ependymoma.
Article
Object: The aims of this study were to present the results of surgery for intramedullary ependymomas (IEs), analyze complication and recurrence rates, and analyze factors that might influence outcome. Methods: The charts of 29 consecutive patients (women - 8, men - 21; mean age - 38 years; range: 18-72) operated for IE were retrospectively analyzed. Mean follow-up was 9 years. Eighteen tumors (62%) were located in the cervical or cervicothoracic spine, and average tumor length was four spinal levels. Twenty patients (69%) presented with neurological deficit. Results: Gross total resections (GTRs) comprised 87% of cases, subtotal resections (STRs) 10%, and partial resections 3%. The neurological outcome on postoperative day 1 was as follows: modified McCormick scale (mMS) grade I - 6%, grade II - 21%, grade III - 21%, grade IV - 31%, and grade V - 21%; at follow-up, outcomes were mMS grade I - 42%, grade II - 34%, grade III - 10%, and grade V - 14% of patients. Compared to the preoperative period, 69% of patients deteriorated postoperatively; however, 62% improved or remained without deficit in follow-up, and deterioration persisted in 24%. The functional results were significantly worse when the intraoperative monitoring potentials dropped below 50% (p=0.005) and if the tumor involved >3 spinal levels (p=0.039). Fourteen postoperative complications in 10 patients (34%) included respiratory failure (14%), pneumonia (7%), urinary infection (10%), bed sores (10%), and CSF leak (7%). Two tumors progressed after STR, with progression-free survival times of 5 and 14 years. No recurrence was observed after GTR. Conclusions: Total tumor resection is the treatment of choice in cases of IEs: no tumor re-growth occurred after total resection, 86% of patients were independent at follow-up, and the 10-year survival rate was 79%.