Acta Clin Croat 2020; 59:329-337Review

doi: 10.20471/acc.2020.59.02.17

MYXOPAPILLARY EPENDYMOMA
OF THE SPINAL CORD IN ADULTS:
A REPORT OF PERSONAL SERIES
AND REVIEW OF LITERATURE
Ibrahim Omerhodžić1, Mirza Pojskić2,3, Krešimir Rotim3-5, Bruno Splavski3-6,
Lukas Rasulić7 and Kenan I. Arnautovic8,9

1
Department of Neurosurgery, Sarajevo University Clinical Center, Sarajevo, Bosnia and Herzegovina;
2
Department of Neurosurgery, University of Marburg, Marburg, Germany;
3
Josip Juraj Strossmayer University of Osijek, Faculty of Medicine, Osijek, Croatia;
4
University of Applied Health Sciences, Zagreb, Croatia;
5
Department of Neurosurgery, Sestre milosrdnice University Hospital Centre, Zagreb, Croatia;
6
Josip Juraj Strossmayer University of Osijek, School of Dental Medicine and Health, Osijek, Croatia;
7
Department of Neurosurgery, Clinical Center of Serbia, Belgrade, Serbia;
8
Semmes Murphey Neurologic & Spine Institute, Memphis, TN, United States;
9
Department of Neurosurgery, University of Tennessee Health Science Center, Memphis, TN, United States

SUMMARY – Myxopapillary ependymomas (MPE) of the spinal cord are slow-growing benign
tumors most frequently found in adults between 30 and 50 years of age. They arise from the ependyma
of the filum terminale and are located in the area of the medullary conus and cauda. The recom-
mended treatment option is gross total resection, while patients undergoing subtotal resection usually
require radiotherapy. Complete resection without capsular violation can be curative and is often ac-
complished by simple resection of the filum above and below the tumor mass. Nevertheless, dissemi-
nation and distant treatment failure may occur in approximately 30% of the cases. In this paper, we
propose an original MPE classification, which is based upon our personal series report concerned with
tumor location and its correlation with the extent of resection. We also provide literature review, dis-
cussing surgical technique, tumor recurrence rate and dissemination, and adjuvant treatment. In con-
clusion, our findings suggest that MPE management based on the proposed 5-type tumor classifica-
tion is favorable when total surgical resection is performed in carefully selected patients. Yet, further
studies on a much broader model is obligatory to confirm this.
Key words: Myxopapillary ependymoma; Gross total resection; Surgical technique; Tumor classification

Introduction counts for nearly 13% of all ependymomas2-4. Accord-

ing to the World Health Organization (WHO) clas-
Spinal cord myxopapillary ependymoma (MPE) sification5,6, MPE is a slow-growing grade I tumor,
derives from the filum terminale1, representing the which is most often found in adults aged 30-50 years7.
most common tumor of the medullary conus and ac- Although such a tumor is mainly situated in the lum-
bar spine just below the medullary conus, it may also
Correspondence to: Kenan Arnautovic, MD, PhD, 6325 Hum-
phreys Blvd, Memphis, TN 38120, USA
spread to the thoracic spine, and sacrum8. Some of
E-mail: karnautovic@semmes-murphey.com these tumors may become aggressive in spite of their
Received April 24, 2020, accepted May 26, 2020 benign histopathology, and they may metastasize

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I. Omerhodžić et al. Adult spinal cord myxopapillary ependymoma

Fig. 1. Preoperative sagittal post-contrast T1-weighted MRI of the spine showing

a cystic contrast-enhancing tumor at medullary conus (L1-2) level (arrow)
with syringomyelia at T10-12 level (a) and sagittal T2-weighted MRI visualizing
the cyst and syringomyelia (arrow) better (b). Postoperative sagittal T2-weighted
MRI revealing complete tumor resection with syringomyelia resolution (c).

Fig. 2. Preoperative sagittal T2-weighted spinal MRI revealing spinal cord cystic tumor with
intratumoral hemorrhage at L1-L3 level (arrow), and subdural hematoma at S1 level (asterisk) (a).
Axial T2-weighted MRI demonstrating the tumor cyst of the vertebral canal ventral part at L1 level
(arrow) (b) and subdural hematoma of the dorsal part of the canal at S1 level (asterisk) (c).
Postoperative sagittal T2-weighted MRI confirming complete tumor resection, hematoma
evacuation, and spinal T12-L3 stabilization (d,e).

along the neural axis to distant cranial and spinal loca- partial (subtotal) resection typically require adjuvant
tions, or to local spots after surgery1,9. Therefore, gross high-dose radiotherapy12,13. Complete tumor removal
total tumor resection, accompanied by intraoperative without iatrogenic capsular damage followed by sim-
neuromonitoring, remains the best surgical manage- ple resection of the filum above and below the tumor
ment option available9-11, while patients undergoing mass is always beneficial, but it may be difficult to ac-

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I. Omerhodžić et al. Adult spinal cord myxopapillary ependymoma

Fig. 3. Preoperative sagittal contrast-enhanced T1-weighted spinal MRI demonstrating an intradural-extramedullary

tumor mass at L4 level (a) confined to filum terminale and adherent to the nerve roots (a). Preoperative T2-weighted
sagittal (arrow) (b) and axial (arrow) MRI scans (c) visualizing the tumor confined to the filum terminale and
adherent to the nerve roots. Postoperative sagittal T2-weighted MRI showing gross total tumor resection (d).

complish. When rupture of the tumor capsule occurs medullary conus with cystic compartment and signs of
during surgery, brain and whole spine adjuvant radio- syringomyelia in the upper part of the cord). Postop-
therapy is suggested to prevent local recurrence and erative sagittal T2-weighted MRI of the spine revealed
cerebrospinal fluid (CSF) dissemination14. Preserva- complete tumor resection with syringomyelia resolu-
tion of sacrococcygeal nerve roots is vital to diminish tion (Fig. 1c) resulting in full neurological recovery.
the incidence of postoperative urinary dysfunction15.
Nevertheless, neural axis dissemination, distant treat- Case 2
ment failure, and tumor relapse may occur in one-third A 43-year-old female presented with acute urinary
of patients13. retention and initial paraparesis. A spinal cord cystic
Herein, we present a 7-case personal series report tumor with intratumoral hemorrhage at L1-L3 level
and propose an original anatomical and surgical MPE (arrow), and subdural hematoma at S1 level (asterisk)
classification grounded on tumor location and its cor- were visualized on sagittal T2-weighted MRI of the
relation with the extent of resection16. We also provide lumbar spine (Fig. 2a). Axial T2-weighted MRI dem-
a literature review, discussing surgical technique, tu- onstrated tumor cyst of the vertebral canal ventral part
mor recurrence rate and dissemination, and adjuvant at L1 level (arrow) (Fig. 2b), and subdural hematoma
treatment. of the dorsal part of the canal at S1 level (asterisk)
(Fig. 2c). The tumor was also classified as type IV B
Case Series Report MPE (involving the entire medullary conus with cys-
tic compartment hemorrhage). Complete tumor resec-
Case 1 tion, hematoma evacuation, and spinal T12-L3 stabili-
A 45-year-old female was admitted to the hospital zation with transpedicular screws and rods were ac-
due to minor lower-limb motor weakness and urinary complished successfully, which was confirmed by post-
retention. A cystic contrast-enhancing tumor at L1-2 operative sagittal T2-weighted MRI (Fig. 2d,e) and
level (arrow) with syringomyelia at T10-12 level was resulted in full patient recovery.
identified on preoperative sagittal post-contrast T1-
Case 3
weighted spinal magnetic resonance imaging (MRI)
(Fig. 1a). The cyst and syringomyelia (arrow) were well A 72-year-old female complained of low back and
visualized on sagittal T2-weighted MRI (Fig. 1b). Ac- bilateral leg pain without any neurological deficit. Pre-
cording to our classification, the tumor was deter- operative sagittal contrast-enhanced T1-weighted spi-
mined to be type IV B MPE (involving the entire nal MRI demonstrated an intradural-extramedullary

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I. Omerhodžić et al. Adult spinal cord myxopapillary ependymoma

Fig. 4. Preoperative sagittal post-contrast T1-weighted MRI of the lumbar spine showing
an enhancing solid tumor at L3 level with engrossment of filum terminale and cauda equina
(a), also shown on post-contrast axial T1-weighted (b) and sagittal T2-weighted MRI (c)
(arrows). Postoperative post-contrast T1-weighted sagittal (d), axial (e) and sagittal
T2-weighted MRI (f ) confirming total tumor resection.

tumor mass at L4 level (Fig. 3a). The tumor, which was 4c) MRI scans (arrows). The tumor was classified as
confined to the filum terminale and adherent to the type I B MPE (extramedullary conus with lumbar
nerve roots, was also visible on preoperative T2- nerve root and filum involvement). Postoperative post-
weighted sagittal (arrow) (Fig. 3b) and axial (arrow) contrast T1-weighted sagittal (Fig. 4d), axial (Fig. 4e),
(Fig. 3c) MRI scans. The tumor was classified as type I as well as sagittal T2-weighted MRI (Fig. 4f ) demon-
B MPE (extramedullary conus with lumbar nerve root strated total tumor resection resulting in complete
and filum involvement). Postoperative sagittal T2- neurological recovery.
weighted MRI demonstrated gross total tumor resec-
tion (Fig. 3d). The patient reported no low back and Case 5
leg pain following surgery. A 47-year-old female presented with paraparesis,
L2 level sensory loss, and bowel incontinence. Preop-
Case 4
erative post-contrast sagittal T2-weighted (Fig. 5a)
A 56-year-old female presented with low back and T1-weighted (Fig. 5b) lumbar spine MRI demon-
pain, lower limb numbness, and voiding difficulties. strated a solid tumor mass at L3 level (arrows). The
Preoperative sagittal post-contrast T1-weighted MRI tumor was classified as type I B MPE (extramedullary
of the lumbar spine showed an enhancing solid tumor conus with lumbar nerve root and filum involvement).
at L3 level affecting filum terminale and cauda equina Postoperative sagittal T2-weighted MRI confirmed
(Fig. 4a), which was also shown on post-contrast axial complete tumor resection (Fig. 5c). The patient re-
T1-weighted (Fig. 4b) and sagittal T2-weighted (Fig. mained neurologically intact following surgery.

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I. Omerhodžić et al. Adult spinal cord myxopapillary ependymoma

Fig. 5. Preoperative post-contrast sagittal T2-weighted (a) and T1-weighted (b)

lumbar spine MRI demonstrating a solid tumor mass at L3 level (arrows).
Postoperative sagittal T2-weighted MRI confirming complete tumor resection (c).

Fig. 6. Preoperative post-contrast sagittal T2-weigted (a) and T1-weighted (b) lumbar MRI
revealing a large cystic tumor at L2 level with hydromyelia and involvement of medullary conus.
Postoperative sagittal T2-weighted MRI confirming complete tumor resection with cyst drainage
and hydromyelia resolution (c).

Case 6 MPE (involving the entire medullary conus with cys-

A 35-year-old male presented with severe low back tic compartment and signs of hydromyelia in the up-
pain and urinary retention. Preoperative post-contrast per part of the cord). Postoperative sagittal T2-weight-
sagittal T2-weigted (Fig. 6a) and T1-weighted (Fig. ed MRI showed complete tumor resection with cyst
6b) lumbar MRI demonstrated a large cystic tumor at drainage and hydromyelia resolution (Fig. 6c). Follow-
L2 level with hydromyelia and involvement of the ing surgery, the patient reported no urinary retention
medullary conus. The tumor was classified as type IV B or other neurological deficit.

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I. Omerhodžić et al. Adult spinal cord myxopapillary ependymoma

Fig. 7. Preoperative post-contrast sagittal T2-weighted MRI of the lumbar spine demonstrating a large cystic tumor at
L1-L2 level with involvement of medullary conus and filum terminale (asterisk) (a). Sagittal T1-weighted MRI
revealing two additional lesions at L3 level, and S1 segment as the site of primary seeding (arrows) (b). Axial post-
contrast T1-weighted MRI depicting separate tumors at L1 (c) and L3 levels (d) (arrows). Postoperative sagittal T2-
weighted (e) and post-contrast T1-weighted MRI (f ) confirming total resection of all the three tumors.

Case 7 roots together with filum terminale, which are surgi-

cally more challenging. Type II represents intramedul-
A 33-year-old male presented with sudden urinary
lary MPEs where conus and filum terminale are in-
retention and no other symptoms or neurological im-
volved that could be easily treated by gross total resec-
pairment. Preoperative post-contrast sagittal T2-
tion. Type III encompasses intramedullary MPEs in-
weighted MRI of the lumbar spine showed a large
volving the lower spinal cord, medullary conus and
cystic tumor at L1-L2 level with involvement of the
filum, causing the upper lumbar cord enlargement.
medullary conus and filum terminale (asterisk) (Fig.
Type IV A stands for solid or cystic MPEs involving
7a), while T1-weighted MRI revealed two additional
the lower spinal cord, while type IV B represents the
lesions at L3 level, and S1 segment, which was the site
entire medullary conus involvement with MPE having
of primary seeding (arrows) (Fig. 7b). Axial post-con-
a cystic component, and signs of hydromyelia/syringo-
trast T1-weighted MRI confirmed the separate tu-
myelia upon the tumor site, where gross or subtotal
mors at L1 (Fig. 7c) and L3 levels (Fig. 7d) (arrows).
resection are management options. Tumors located
The tumor was classified as type IV B MPE (involving
the entire medullary conus with cystic compartment).
Table 1. Classification of spinal cord myxopapillary
Postoperative sagittal T2-weighted (Fig. 7e) and post-
ependymoma
contrast T1-weighted MRI (Fig. 7f ) confirmed total
resection of all the three tumors. Following surgery, Type I A Filum terminale only
urinary retention resolved entirely, and the patient re- Type I B Extramedullary conus with lumbar nerve
covered completely. Further MRI screenings excluded root and filum
secondary seeding. Type II Intramedullary conus and filum terminale
Type III Upper lumbar cord enlargement
Discussion Type IV A Lower part of spinal cord and medullary
conus with solid and cystic component
We have proposed an original 5-type MPE classi- Type IV B Entire medullary conus with cystic
fication based on tumor location and its correlation compartment and signs of hydromyelia/
with the extent of resection16 (Table 1, Fig. 8). Accord- syringomyelia in the cord upper part
ingly, extramedullary intradural MPEs incorporating Type V A Outside the lumbar cord
filum terminale only represent type I A, where gross (cervical/thoracic spine)
total resection is achievable easily. Type I B stands for Type V B Outside the vertebral canal
extramedullary conus tumors involving lumbar nerve (intracranial, sacrococcygeal)

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Fig. 8. Schematic drawing of 5-type spinal cord MPE classification.

outside the lumbar cord, but still within the vertebral geon and other medical staff7,23. The same happened
canal along the thoracic and/or cervical spine repre- with the patients from this series where no surgical
sent type V A MPE, whereas those located outside the morbidity and no tumor relapse or dissemination was
vertebral canal epitomize type V B MPEs that may be observed. Accordingly, Klekamp et al. report that gross
situated at intracranial or sacrococcygeal regions. total tumor resection was achieved in 77.7% of their
Reviewing the literature, we found that the mean patients7, while several studies suggest that it must be
patient age at the time of tumor symptomatic occur- combined with high-dose radiotherapy in order to im-
rence was 36.44 years, and that medullary conus was prove the outcome24-26, and to avoid recurrence, as well
involved in 28.4% of cases17, which was less consistent as primary and secondary seeding, which remains un-
with our results where the mean patient age was 47.28 der-recognized in adults1,27. Although tumor benign
years and the entire medullary conus was involved in histologic features are commonly preserved in case of
four out of seven (57.1%) patients. dissemination28, rostral neuro-axial spread mostly af-
Younger age, preoperative functional capacity, less- fects the thoracic and cervical spine29,30. Then, addi-
er initial neurological deficit, tumor location, size and tional resection or irradiation would be endorsed if
the extent of resection, as well as adjuvant radiotherapy metastases become symptomatic30. There are single
were identified as significant prognostic factors influ- case reports describing the benefits of second- and
encing better outcome8,13,18,19. All the patients from our third-line chemotherapy concomitant with radiother-
series had minor or lesser degree neurological deficit apy after multiple surgeries of recurrent MPE with
on admission, and total surgical tumor resection was disseminated metastases31,32. However, this was not the
possible in all of them. case in patients from our series, since all of them re-
Long-term outcome is also correlated to the extent covered well after total tumor resection. Therefore, the
of tumor resection, as well as to the integrity of the need for repeat surgery or adjuvant therapy was not
tumor capsule7,20, since larger tumors may perforate established and no tumor relapse or dissemination was
the capsule14, leading to CSF seeding and dissemina- recorded. Nonetheless, one should still remember the
tion21. Hence, an association between capsular damage possibility of tumor relapse, primary seeding, and drop
and tumor relapse was found22. Still, surgical morbidity metastases; one should also consider the entire cranio-
is generally low and postoperative clinical improve- spinal neuroimaging as part of both the preoperative
ment is pretty good, while recurrence rate remains work-up and postoperative follow-up1. Therefore,
scarce when treatment recommendations and proto- long-term or lifelong MRI surveillance should be ad-
cols are strictly followed by an experienced neurosur- vised1,16, which is also part of our patient protocol.

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I. Omerhodžić et al. Adult spinal cord myxopapillary ependymoma

In conclusion, having in mind the small number of surgery. Childs Nerv Syst. 2011;27(9):1445-52, http://dx.doi.
patients in this personal case series, as well as its retro- org/10.1007/s00381-011-1471-4.
spective design, our findings suggest that MPE man- 11. Tobin MK, Geraghty JR, Engelhard HH, et al. Intramedullary
spinal cord tumors: a review of current and future treatment
agement based on the proposed 5-type tumor classifi-
strategies. Neurosurg Focus. 2015;39(2):E14, http://dx.doi.
cation is favorable when total surgical resection is per- org/10.3171/2015.5.FOCUS15158.
formed in carefully selected patients, which is well-
12. Fassett DR, Pingree J, Kestle JR. The high incidence of tumor
supported by the literature data. Yet, additional studies dissemination in myxopapillary ependymoma in pediatric pa-
on a much broader model are obligatory to confirm tients. Report of five cases and review of the literature. J Neu-
these results. rosurg. 2005;102(1 Suppl):59-64, http://dx.doi.org/10.3171/
ped.2005.102.1.0059.
Acknowledgment 13. Weber DC, Wang Y, Miller R, et al. Long-term outcome of
patients with spinal myxopapillary ependymoma: treatment re-
We thank Mr. Andrew Gienapp for editing this sults from the MD Anderson Cancer Center and institutions
manuscript. from the Rare Cancer Network. Neuro Oncol. 2015;17(4):588-
95, http://dx.doi.org/10.1093/neuonc/nou293.
14. Nakamura M, Ishii K, Watanabe K, et al. Long-term surgical
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Sažetak

MIKSOPAPILARNI EPENDIMOM KRALJEŽNIČNE MOŽDINE U ODRASLIH:

PRIKAZ OSOBNE SERIJE I PREGLED LITERATURE

I. Omerhodžić, M. Pojskić, K Rotim, B. Splavski, L Rasulić i K. Arnautovic

Miksopapilarni ependimomi (MPE) kralježnične moždine sporo su rastući, dobroćudni tumori najčešće zastupljeni u
odraslih u dobi između 30 i 50 godina života. Nastaju iz ependima filuma terminale, a pretežito su smješteni u području
medularnoga konusa i kaude. Kirurško uklanjanje tumora u cijelosti preporučena je metoda liječenja, dok u bolesnika u kojih
to nije moguće učiniti u obzir dolazi subtotalna resekcija nakon koje je potrebno zračenje. Potpuno uklanjanje tumora uz
očuvanje cjelovitosti tumorske kapsule postiže se jednostavnom resekcijom filuma terminale iznad i ispod tumorske mase, što
može dovesti do izlječenja. Unatoč tomu, tumorska diseminacija uzduž neuralne osi može se javiti u oko 30% slučajeva. U
ovom radu predlažemo originalnu klasifikaciju MPE koja prosuđuje smještaj tumora i obujam tumorske resekcije, a temelje-
na je na osobnoj seriji operiranih bolesnika. Također raspravljamo o kirurškoj tehnici, o mogućnostima recidiva i širenja
ovakvih tumora, kao i o oblicima pomoćnog liječenja, koristeći se pregledom literature. Zaključujemo kako naši rezultati
zagovaraju kirurško liječenje temeljeno na predloženoj originalnoj tumorskoj klasifikaciji, koje može biti uspješno u pažljivo
odabranih bolesnika u kojih je tumor uklonjen u cijelosti. Naknadna istraživanja na znatno većem uzorku potrebna su za
potvrdu naših rezultata.
Ključne riječi: Miksopapilarni ependimom; Potpuna resekcija tumora; Kirurška tehnika; Klasifikacija tumora

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