Myxopapillary Ependymoma

Earn CME/CE in your profession:

Continuing Education Activity

Myxopapillary ependymoma is a distinct subtype of spinal cord ependymomas that has a predilection for the lumbosacral region. To achieve satisfactory outcomes, the basic and clinical aspects of myxopapillary ependymomas must be clearly defined. This activity focuses on the etiology, evaluation, management, and complications of myxopapillary ependymomas and highlights the role of the interprofessional team in improving healthcare outcomes.


  • Identify the etiology of myxopapillary ependymoma.
  • Review the appropriate evaluation of myxopapillary ependymoma.
  • Outline the management options available for myxopapillary ependymoma.
  • Summarize interprofessional team strategies for improving care coordination and communication to advance the care of myxopapillary ependymoma and improve outcomes.


Initially described by Kernohan in 1932, myxopapillary ependymoma is a distinct subtype of spinal cord ependymomas that has a predilection for the lumbosacral region.[1] It represents 13% of all spinal ependymomas and accounts for 90% of all tumors in the conus medullaris.[2][3] Myxopapillary ependymoma is a benign and slow-growing neoplasm that has been histologically classified as grade 2, according to the World Health Organization (WHO) classification.


Myxopapillary ependymoma arises from the ependymal glia of the filum terminale. No risk factors for the development of this tumor have been described. Extradural myxopapillary ependymomas are thought to arise from the coccygeal medullary vestige or the extradural remnants of the filum terminale.[4]


Myxopapillary ependymomas account for 1% to 5% of all spinal neoplasms and approximately 13% of all spinal ependymomas. In the American population, their incidence was 1.00 per million person-years. The age at onset varies between 30 and 50 years (mean age, 35 years). The most common location of myxopapillary ependymoma is the lumbosacral spine segment, mainly in the conus medullaris and cauda equina regions. Other rare locations include the cerebral ventricles and the brain.[5]



Grossly, myxopapillary ependymomas are sausage-shaped and often encapsulated. They are reddish to purplish in color and soft in consistency.[6] Mucinous degeneration or hemorrhage may be sometimes seen in these tumors.[7]


Histologically, myxopapillary ependymomas are characterized by a papillary architecture. In the center of the papillae, there are blood vessels that are surrounded by cuboidal to spindled glial cells. The stroma is myxoid and alcian blue–positive.[8] Vascular hyalinization and other degenerative changes are often prominent. Some tumors may show a more compact fascicular pattern with occasional perivascular pseudorosettes with mucin-rich microcysts and occasional perivascular pseudorosettes. In some cases, the tumor may exhibit rounded to spiculated collagen “balloons.” In these tumors, mitotic figures are usually scarce. Endothelial proliferation and necrosis are absent. Some authors described in isolated case reports anaplastic and giant cell variants of myxopapillary ependymomas.[9]


An immunohistochemical study is frequently required to distinguish myxopapillary ependymoma from chondrosarcoma or chordoma. The tumor cells are often positive for S-100, GFAP, CD99, COX-2. However, they are negative for endomysial antibody (EMA).[8]

History and Physical

The clinical presentation of myxopapillary ependymomas is not specific and is similar to that of other intradural tumors located below the spinal cord in the region of the filum terminale.[1]

The symptoms related to this tumor are influenced by the size, the site of presentation, and the local extent of the tumor. Myxopapillary ependymomas are characterized by an indolent and slow clinical course. The majority of the patients experience symptoms for months to years before diagnosis and have a nonspecific presentation.[10]

Nonspecific Symptoms 

Patients with myxopapillary ependymomas usually complain of radicular and back pain worsening during the night and in the lounging position.[1]

Other Symptoms of Myxopapillary Ependymoma

Patients with myxopapillary ependymoma may complain of motor, sensory, urinary, and gait abnormalities.[1] Among the other symptoms of myxopapillary ependymoma, enlargement of the neural foramina scoliosis and scalloping of the vertebral body can be distinguished. Sudden exacerbation of the symptoms with leg weakness and sphincter disturbances can occur secondary to hemorrhage.[1]


The best imaging modality for the diagnosis of myxopapillary ependymoma is magnetic resonance imaging (MRI) because of its superior soft-tissue contrast. Other imaging studies include plain radiograph and computed tomography (CT) scan.

Plain radiograph and CT scan can reveal scalloping of the vertebral bodies secondary to the expansion of the spinal canal caused by the presence of a large myxopapillary ependymoma.

Magnetic resonance imaging is helpful in identifying the extent of the tumor and its relationship to intraspinal structures. It also allows for visualization of the cauda equina both above and below the tumor and will identify drop metastases in the subarachnoid space. Such information is useful in preoperative surgical planning. Magnetic resonance imaging findings in myxopapillary ependymomas are nonspecific.[4][11]

On magnetic resonance imaging, the lesion is iso- to hypointense on T1 and hyperintense on T2. Owing to their mucin content, the signal is hyperintense in myxopapillary ependymoma. Moreover, the tumor is heterogeneous after gadolinium injection. Hemorrhage and cyst formations are common features that contribute to signal inhomogeneity.[11]

However, these tumors do contain certain features that help suggest the diagnosis. These features include:

  1. An intradural extramedullary thoracolumbar mass
  2. The tumor extends for several vertebral levels in the lumbar and sacral canal.
  3. It is hypointense to isointense on T1-weighted images.
  4. It is hyperintense on T2 weighted images.
  5. There will be an intense, homogeneous enhancement after the administration of contrast.
  6. There will usually be a region of slightly lower intensity at tumor margin on T2-weighted sequences.
  7. Cystic rostral or caudal degeneration exists in 50% of cases.

Treatment / Management

Owing to the rarity of myxopapillary ependymomas, there are no definitive treatment guidelines. The consensus is that surgical excision is the preferred initial treatment modality.[12]

Early detection and treatment of patients affected by myxopapillary ependymomas are crucial to achieving optimal patient outcomes. The best treatment modality for myxopapillary ependymoma is complete surgical resection, which is associated with a complete resolution for the majority of patients.[13][14] Surgery should be performed early so as to guarantee better postoperative outcomes.[15] Complete resection of this tumor may be hazardous, principally if the tumor involves the conus medullaris or is interlaced with the nerve roots of the cauda equina. Surgeons must be very careful since nerve roots may also penetrate directly through the tumor.[16]

In the case of subtotal resection, adjuvant radiotherapy is recommended. However, chemotherapy is used in young children who are more prone to negative side effects from radiation therapy.[15] The efficacy of chemotherapy has not been established in myxopapillary ependymomas, although it has been suggested as a potential treatment to prevent a recurrence.[17]

Radical surgery for myxopapillary ependymoma is associated with a favorable outcome in both children and adults as with other spinal cord ependymomas.[18]

Long-term survival is better in the case of gross total removal of myxopapillary ependymoma rather than subtotal resection.[1][16]

Differential Diagnosis

The differential diagnoses of filum terminale and small conus myxopapillary ependymomas include:

  • Fibrous meningioma: The fibrillary variant of myxopapillary ependymomas may be confused with fibrous meningioma
  • Paraganglioma: However, myxopapillary ependymomas are chromogranin-A negative and GFAP positive
  • Schwannoma: The fibrillary variant of myxopapillary ependymomas may be confused with schwannoma

The epithelial and papillary variants of myxopapillary meningioma may mimic carcinoma or meningioma.

The differential diagnosis of a large myxopapillary ependymoma that is responsible for sacral destruction includes:

  • Aneurysmal bone cyst
  • Chondrosarcoma
  • Chordoma
  • Giant cell tumor
  • Metastatic carcinoma: However, myxopapillary ependymomas are devoid of cytological atypia, have a lower Ki-67 LI, and are focally fibrillar

Radiation Oncology

The therapeutic benefits of radiotherapy for myxopapillary ependymomas are still debated owing to the benign and slow-growing nature of the tumor. In one study, the authors reported no difference in irradiated versus nonirradiated patients after total or subtotal resection.[16]

According to other recent studies, postoperative radiotherapy is beneficial for patients who had subtotal resection of myxopapillary ependymoma and was considered as the best treatment option for these patients because of the high risk of long-term recurrence in case of subtotal resection of the tumor.[19][20][21]

Other studies demonstrated that radiotherapy is significantly more beneficial in the case of subtotally resected myxopapillary ependymomas smaller than 6 cm compared to larger tumors (58% versus 92% 5- and 10-year progression-free survival).[22]


World Health Organization classification:

Recently, the classification of central nervous system tumors is moving toward a more molecularly oriented classification scheme.

In the 2021 WHO classification of central nervous system tumors, ependymal tumors are classified as follows:

  • Myxopapillary ependymoma (grade 2)
  • Subependymoma (grade 1)
  • Classic ependymoma (grade 2)
  • RELA fusion-protein positive ependymoma (grade 2 or 3, this is the only molecularly classified ependymoma)
  • Anaplastic ependymoma (grade 3)[23]


Currently, neither the histopathology nor the MIB-1 index appears to predict the natural history, likelihood of recurrence, or metastases.

Patients should be followed postoperatively with serial magnetic resonance imaging to monitor for tumor recurrence, even if a gross total resection was accomplished because even then, recurrence rates of 10% to 19% have been reported.[1][16]

Despite the good results with surgery, tumor recurrence and dissemination may occur. Local recurrence and metastases have been related to the clinical history of less than 1 year, the extent of resection, atypical histology, and lesions extending into the substance of the spinal cord.[21][24]

Myxopapillary ependymomas are considered benign tumors of the central nervous system with long-term survival rates and a tendency for local recurrence. However, an aggressive course has occasionally been described, leading to cerebrospinal fluid dissemination and even systemic metastases.

Despite its well-differentiated appearance, it occasionally shows aggressive behavior. Local progression is common and systemic metastases have also been described with the lungs being the organs most commonly affected. Systemic dissemination is more common in cases of a primary extraspinal location.[1]

The estimated 10-year overall survival has been reported to be 92.4% with a 10-year progression-free survival of 61.2%.[25] Age (<36 vs. ≥36 years), treatment modality (surgery alone vs. surgery and radiotherapy), and extent of surgery are prognostic factors for local control and progression-free survival.

The 5-year overall survival rate of patients with myxopapillary ependymoma is excellent and varies from 90% to 100%. 

Patients who undergo gross total resection of intradural myxopapillary ependymomas have a good prognosis, with a mean survival time of 19 years.[1]

Although rarely encountered in children, myxopapillary ependymomas have a more aggressive clinical course with an increased risk of recurrence and dissemination when compared to the adult population. The reason for this more aggressive course in children is unclear.


Some authors reported that en-bloc removal of encapsulated myxopapillary ependymomas of the filum terminale showed a lower proportion of dissemination (10%) than myxopapillary ependymomas removed piecemeal (19%).[1] During surgery, the rupture of the capsule of myxopapillary ependymoma may result in cerebrospinal fluid seeding and dissemination.[24] According to the literature, in 30% of the cases, dissemination and treatment failure may occur.[25][24] 

The factors that increase the risk of recurrence and metastases are the extension of myxopapillary ependymoma into the substance of the spinal cord, a clinical history of less than one year, the extent of resection, and atypical histology of the tumor.[24]


  • Neurosurgeon
  • Neurologist
  • Radiotherapist
  • Radiologist
  • Pathologist

Deterrence and Patient Education

Patients should consult with a neurologist when they suffer from radicular pain and back pain. The interprofessional team should ensure that the patients are well informed about myxopapillary ependymoma. Patients should be informed about educational websites so as to help them better understand this benign neoplasm, its prognosis, and its treatment. Patient education plays a very important role in the deterrence of the processes that can cause a myxopapillary ependymoma. Specialty-trained nurses often play a crucial role.

Pearls and Other Issues

Although myxopapillary ependymomas are classified as benign tumors, dissemination, and local recurrence pose a major challenge in their clinical treatment.

Therefore, a close follow-up of the patients with myxopapillary ependymoma is mandatory in order to detect local recurrent or spread of the tumor.

Enhancing Healthcare Team Outcomes

Management of myxopapillary ependymoma needs an interprofessional approach involving a team that consists of a neurosurgeon, neurologist, radiotherapist, radiologist, and pathologist. The primary clinicians should refer patients with any neurological deficits to a neurologist or a neurosurgeon for further workup. All health professionals must coordinate their actions to enhance the management of patients with myxopapillary ependymoma. Coordination begins with proper communication between clinicians, nurses, and pharmacists. 

The optimal treatment of myxopapillary ependymoma relies on surgical removal of the tumor. The interprofessional team should perform an exhaustive preoperative workup to minimize postoperative complications of surgery.

Article Details

Article Author

Faten Limaiem

Article Editor:

Joe M Das


1/1/2023 4:18:40 AM



Sonneland PR,Scheithauer BW,Onofrio BM, Myxopapillary ependymoma. A clinicopathologic and immunocytochemical study of 77 cases. Cancer. 1985 Aug 15;     [PubMed PMID: 4016681]


Allen JC,Siffert J,Hukin J, Clinical manifestations of childhood ependymoma: a multitude of syndromes. Pediatric neurosurgery. 1998 Jan;     [PubMed PMID: 9693331]


Celli P,Cervoni L,Cantore G, Ependymoma of the filum terminale: treatment and prognostic factors in a series of 28 cases. Acta neurochirurgica. 1993;     [PubMed PMID: 8304078]


Koeller KK,Rosenblum RS,Morrison AL, Neoplasms of the spinal cord and filum terminale: radiologic-pathologic correlation. Radiographics : a review publication of the Radiological Society of North America, Inc. 2000 Nov-Dec;     [PubMed PMID: 11112826]


Feldman WB,Clark AJ,Safaee M,Ames CP,Parsa AT, Tumor control after surgery for spinal myxopapillary ependymomas: distinct outcomes in adults versus children: a systematic review. Journal of neurosurgery. Spine. 2013 Oct;     [PubMed PMID: 23971762]


Chan HS,Becker LE,Hoffman HJ,Humphreys RP,Hendrick EB,Fitz CR,Chuang SH, Myxopapillary ependymoma of the filum terminale and cauda equina in childhood: report of seven cases and review of the literature. Neurosurgery. 1984 Feb;     [PubMed PMID: 6709144]


Van Hoe W,Mebis W,Bali A,Gielen J,Dekeyzer S, Myxopapillary ependymoma of the sacrum. Acta neurologica Belgica. 2020 Feb;     [PubMed PMID: 31555979]


Specht CS,Smith TW,DeGirolami U,Price JM, Myxopapillary ependymoma of the filum terminale. A light and electron microscopic study. Cancer. 1986 Jul 15;     [PubMed PMID: 3521831]


Manasa PL,Uppin MS,Sundaram C, Analysis of squash smear cytology of ependymomas. Acta cytologica. 2012;     [PubMed PMID: 22378082]


Chinn DM,Donaldson SS,Dahl GV,Wilson JD,Huhn SL,Fisher PG, Management of children with metastatic spinal myxopapillary ependymoma using craniospinal irradiation. Medical and pediatric oncology. 2000 Oct;     [PubMed PMID: 11025481]


Wippold FJ 2nd,Smirniotopoulos JG,Moran CJ,Suojanen JN,Vollmer DG, MR imaging of myxopapillary ependymoma: findings and value to determine extent of tumor and its relation to intraspinal structures. AJR. American journal of roentgenology. 1995 Nov;     [PubMed PMID: 7572515]


Bates JE,Choi G,Milano MT, Myxopapillary ependymoma: a SEER analysis of epidemiology and outcomes. Journal of neuro-oncology. 2016 Sep;     [PubMed PMID: 27306443]


Klekamp J, Spinal ependymomas. Part 1: Intramedullary ependymomas. Neurosurgical focus. 2015 Aug;     [PubMed PMID: 26235023]


Tobin MK,Geraghty JR,Engelhard HH,Linninger AA,Mehta AI, Intramedullary spinal cord tumors: a review of current and future treatment strategies. Neurosurgical focus. 2015 Aug;     [PubMed PMID: 26235012]


Ferrante L,Mastronardi L,Celli P,Lunardi P,Acqui M,Fortuna A, Intramedullary spinal cord ependymomas--a study of 45 cases with long-term follow-up. Acta neurochirurgica. 1992;     [PubMed PMID: 1481757]


Bagley CA,Wilson S,Kothbauer KF,Bookland MJ,Epstein F,Jallo GI, Long term outcomes following surgical resection of myxopapillary ependymomas. Neurosurgical review. 2009 Jul;     [PubMed PMID: 19221818]


Fegerl G,Marosi C, Stabilization of metastatic myxopapillary ependymoma with sorafenib. Rare tumors. 2012 Jun 26;     [PubMed PMID: 23087798]


Schweitzer JS,Batzdorf U, Ependymoma of the cauda equina region: diagnosis, treatment, and outcome in 15 patients. Neurosurgery. 1992 Feb;     [PubMed PMID: 1545888]


Akyurek S,Chang EL,Yu TK,Little D,Allen PK,McCutcheon I,Mahajan A,Maor MH,Woo SY, Spinal myxopapillary ependymoma outcomes in patients treated with surgery and radiotherapy at M.D. Anderson Cancer Center. Journal of neuro-oncology. 2006 Nov;     [PubMed PMID: 16648988]


Gilhuis HJ,Kappelle AC,Beute G,Wesseling P,Grotenhuis A,Boerman RH, Radiotherapy for partially resected spinal ependymomas: a retrospective study of 60 cases. Oncology reports. 2003 Nov-Dec;     [PubMed PMID: 14534747]


Schild SE,Wong W,Nisi K, In regard to the radiotherapy of myxopapillary ependymomas. International journal of radiation oncology, biology, physics. 2002 Jul 1;     [PubMed PMID: 12062627]


Wahab SH,Simpson JR,Michalski JM,Mansur DB, Long term outcome with post-operative radiation therapy for spinal canal ependymoma. Journal of neuro-oncology. 2007 May;     [PubMed PMID: 17206474]


Huynh TR,Lu C,Drazin D,Lekovic G, Myxopapillary ependymoma with anaplastic features: A case report with review of the literature. Surgical neurology international. 2018;     [PubMed PMID: 30294495]


Rezai AR,Woo HH,Lee M,Cohen H,Zagzag D,Epstein FJ, Disseminated ependymomas of the central nervous system. Journal of neurosurgery. 1996 Oct;     [PubMed PMID: 8814165]


Weber DC,Wang Y,Miller R,Villà S,Zaucha R,Pica A,Poortmans P,Anacak Y,Ozygit G,Baumert B,Haller G,Preusser M,Li J, Long-term outcome of patients with spinal myxopapillary ependymoma: treatment results from the MD Anderson Cancer Center and institutions from the Rare Cancer Network. Neuro-oncology. 2015 Apr;     [PubMed PMID: 25301811]