spinal_myxopapillary_ependymoma

Spinal myxopapillary ependymoma

see also Extraspinal myxopapillary ependymoma.


The spinal myxopapillary ependymoma is a rare subtype of ependymoma that develops almost exclusively within the spinal cord.

In 1932, James Watson Kernohan 1) was the first to use the term myxopapillary in the division of his large series of spinal ependymomas into epithelial, cellular, and myxopapillary types on the basis of cytological architecture.

Spinal Myxopapillary Ependymoma Epidemiology

The myxopapillary variant is histologically designated as Grade I and is found in the cauda equina region. Most arise from the filum terminale 2) 3) 4) 5).

They are thought to arise from the ependymal glia of the filum terminale or conus medullaris. The vast majority are intradural and extramedullary, however, rarely they occur in the extradural space. Occasionally CSF dissemination occurs and multiple lesions are seen in 14-43% cases.

In children, these tumours may have more aggressive behaviour.

Macroscopic appearance

They are typically multilobulated and encapsulated. They often have associated haemorrhage and may calcify or undergo cystic degeneration.

Microscopic appearance

Histologically, they contain papillary elements arranged radially around a hyalinized fibrovascular core, forming perivascular pseudorosettes, with myxoid material between the blood vessel and tumour cells.

“Balloons” - rounded eosinophilic PAS positive structures - are sometimes encountered

Clinically, the most common finding is lumbar, sacral or radicular pain 6) 7). which is often worse in the recumbent position and, thus, at night 8). To a lesser extent, sensory changes, motor deficits, bladder abnormalities and impotence/ejaculation dysfunctions also occur 9) 10).

The average duration of symptoms preceding diagnosis ranges from 13 months to 8.3 years 11) 12) 13) since they are slow growing.

Plain radiograph / CT

If they become large, myxopapillary ependymomas may expand the spinal canal, cause scalloping of the vertebral bodies and extend out of the neural exit foramina.

MRI

Diagnosis of MPE is best accomplished with magnetic resonance imaging (MRI). MRI findings typically include an intradural mass that tends to be hypointense or isointense with the spinal cord on T1 weighted images, hyperintense on T2 weighted images and will have intense homogenous enhancement after the administration of intravenous contrast material 14) 15) 16) 17). 18) 19). On average, the lesions affect 2 to 4 vertebral body levels 20) 21) 22) 23) 24).

Confirmation of the diagnosis cannot be made until the excised tumour tissues are examined pathologically 25).

From:http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3661182/#b11-jcca57_2_150

(A) Hyperintense intradural spinal canal mass noted on T2-weighted MRI; (B) The mass was hypointense on T1-weighted MRI and showed avid enhancement (C). Both images demonstrate that the upper extent of the mass is inseparable from the distal spinal cord with a large exophytic component. The spinal canal is expanded with posterior vertebral body scalloping extending from T12 to L4. Intra- and extra-lesional flow voids represent hypervascularity at the upper aspect of the mass surrounding the distal spinal cord.

Signal characteristics

T1

usually isointense prominent mucinous component occasionally results in T1 hyperintensity

haemorrhage and calcification can also lead to regions of hyper- or hypointensity.

T2

overall high intensity low intensity may be seen at the tumour margins because of haemorrhage (myxopapillary ependymomas are the subtype of ependymomas that are most prone to haemorrhage

calcification may also lead to regions of low T2 signal

T1 C+ (Gd)

enhancement is virtually always seen

the enhancement pattern is typically homogeneous. However, they can have a variable enhancement pattern that, in part, depends on the amount of haemorrhage preseny.

Differential diagnoses for MPE include other primary spinal cord tumours and metastases, or possibly disc herniation depending on the symptoms 26).

Differential diagnosis of a small conus and filum terminale myxopapillary ependymoma includes:

Spinal schwannoma: often indistinguishable from ependymoma

Spinal paraganglioma

Differential diagnosis of a large myxopapillary ependymoma that causes sacral destruction:

Aneurysmal bone cyst: involving the spine

Chordoma.

giant cell tumour: involving the spine

Treatment for MPE mainly involves surgical excision of the tumour.

Radiotherapy

If the capsule ruptures or the tumour is not confined to the filum terminale, the mass could infiltrate and adhere to the cauda equina and/or conus medullaris or disseminate via the cerebral spinal fluid 27) 28).

Therefore, adjuvant radiotherapy is recommended when en bloc excision (removal of the entire tumour as one piece) cannot be accomplished 29) 30).

However, the efficacy of radiation therapy has not been established and can result in adverse effects such as radiation myelopathy 31) 32) and residual dysuria 33).

In the series of Tsai et al., the median age at diagnosis was 35 years (range, 8-63 years). Twenty patients (39%) had surgery alone, 30 (59%) had surgery plus radiotherapy (RT), and 1 (2%) had RT only. At a median follow-up of 11 years (range, 0.2-37 years), 10-year overall survival (OS), progression free survival (PFS), and local control (LC) for the entire group were 93%, 63%, and 67%, respectively. Nineteen patients (37%) had disease recurrence, and the recurrence was mostly local (79%). Twenty-eight of 50 patients who had surgery (56%) had gross total resection; 10-year LC was 56% after surgery vs 92% after surgery and RT (log-rank P = .14); the median time of LC was 10.5 years for patients receiving gross total resection plus RT, and 4.75 years for gross total resection only (P = .03). Among 16 patients with subtotal resection and follow-up data, 10-year LC was 0% after surgery vs 65% for surgery plus RT (log-rank P = .008). On multivariate analyses adjusting for resection type, age older that 35 years at diagnosis and receipt of adjuvant radiation were associated with improved PFS (hazard ratio [HR]: 0.14, P = .003 and HR: 0.45, P = .009) and LC (HR: 0.22, P = .02 and HR: 0.45, P = .009).

Postoperative radiotherapy after resection of MPE was associated with improved PFS and LC 34).

see Spinal myxopapillary ependymoma outcome.

Abdallah et al. retrospectively reviewed the medical records of 38 primary spinal myxopapillary ependymoma cases who underwent surgery at 2 neurosurgical centers spanning 16 years, from 2004 to 2019. All pediatric cases (patient age <18 years) who were diagnosed with MPE and re-presented with spinal seeding/drop metastases (SSM) were selected as the core sample for this study. Relevant literature was briefly reviewed.

Three pediatric MPE cases (2 females and 1 male) experienced SSM. The mean age at first presentation was 12.0 ± 1.0 years. The mean preoperative course was 2.9 ± 1.2 months. The predominant location was the lumbar spine in 2 tumors (both originated from filum terminale [FT]). Two tumors were located intradural intramedullary. Gross-total resection was achieved in 2 patients. No patient had neurofibromatosis type 2. No adjuvant treatment was given after the first surgery. The mean period between the first diagnosis and diagnosis of SSM was 44.0 ± 31.5 months. The location of SSM in all patients was the sacral spine (1 patient experienced distant metastasis in her brain besides her sacral metastasis). The mean follow-up was 68.3 ± 53.7 months.

They found a statistically significant relationship between SSM in pediatric MPEs and the intramedullary location, FT origin, and number of affected segments. Close clinical and radiological follow-up is essential for pediatric MPE patients. 35).

2017

Twenty-seven patients who underwent resection of MPE were enrolled. The authors determined their demographic features, imaging characteristics, clinical presentations and outcomes, surgical procedures and histological properties by chart review, telephone contact, reviewing of surgical notes, pre-/postoperative imaging and immunohistological staining.

GTR (gross total resection) was achieved in 18 patients (66.7 %) and STR (subtotal resection) in 9 (33.3 %). Although GTR rendered a better disease control rate, the difference was not significant. Pediatric patients suffered from a greater risk of recurrence as well as a shorter period to disease relapse. In the majority of cases, we observed the overexpression of platelet-derived growth factor receptor α (PDGFRα), matrix metalloproteinase-2 (MMP2) and matrix metalloproteinase-14 (MMP14). Epidermal growth factor receptor (EGFR) was observed in the tumors of 7 of 23 nonrecurrent patients, but not in any recurrent tumors.

The results of the present study indicate that the extent of resection and age are major factors related to tumor recurrence. Therefore, gross total resection is recommended whenever possible unless following neurological dysfunction is predictable. Moreover, pediatric patients need considerable attention after surgery, particularly in the early stages. PDGFRα, MMP2 and MMP14 may be new diagnostic and therapeutic targets and EGFR a potential predictor of improved prognosis for MPE 36).

A 48-year-old male police officer was referred to a chiropractic clinic by a general practitioner for the evaluation of recurrent acute low back pain (LBP). Although the first episode of LBP was resolved, the clinical examination during the second episode revealed subtle changes that warranted referral to magnetic resonance imaging (MRI). The MRI revealed a spinal myxopapillary ependymoma.

Because the primary symptoms of spinal intramedullary ependymomas can mimic ordinary LBP presentations, in particular lumbar intervertebral disc herniations, clinicians need to be sensitive to subtle changes in the clinical presentation of LBP patients. Prompt referral to advanced medical imaging such as MRI and early neurosurgical intervention is key to achieve best possible outcomes for patients with spinal intramedullary ependymomas 37).


1)
Kernohan JW: Primary tumors of the spinal cord and intradural filum terminale, in Penfield W (ed): Cytology and Cellular Pathology of the Nervous System. New York, Paul B. Hoeber, 1932, vol 3, pp 993–1025.
2)
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 14:204–210, 1984.
3)
Fokes EC Jr, Earle KM: Ependymomas: Clinical and pathological aspects.J Neurosurg 30:585–594, 1969.
4)
Schild SE, Nisi K, Scheithauer BW, Wong WW, Lyons MK, Schomberg PJ, Shaw EG: The results of radiotherapy for ependymomas: The Mayo Clinic experience. Int J Radiat Oncol Biol Phys 42:953–958, 1998.
5)
Yamada CY, Whitman GJ, Chew FS: Myxopapillary ependymoma of the filum terminale. AJR Am J Roentgenol 168:366, 1997.
6) , 9) , 11) , 15) , 21) , 27) , 31)
Sakai Y, Matsuyama Y, Katayama Y, et al. Spinal myxopapillary ependymoma: Neurological deterioration in patients treated with surgery. Spine. 2009;34:1619–1624.
7) , 10)
Hanbali F, Fourney DR, Marmor E, et al. Spinal cord ependymoma: Radical surgical resection and outcome. Neurosurgery. 2002;51:1162–74.
8) , 17) , 26)
Bagley CA, Wilson S, Kothbauer KF, Bookland MJ, Epstein F, Jallo GI. Long term outcomes following surgical resection of myxopapillary ependymomas. Neurosurg Rev. 2009;32:321–334.
12) , 30) , 32)
Volpp PB, Han K, Kagan AR, Tome M. Outcomes in treatment for intradural spinal cord ependymomas. Int J Radiation Oncology Biol Phys. 2007;69:1199–1204.
13)
Asazuma T, Toyama Y, Suzuki N, et al. Ependymomas of the spinal cord and cauda equine: An analysis of 26 cases and a review of the literature. Spinal Cord. 1999;37:753–59.
14) , 20)
Choi JY, Chang KH, Yu IK, et al. Intracranial and spinal ependymomas: Review of MR images in 61 patients. Korean J Radiol. 2002;3:219–228.
16) , 22)
Wippold FJ, Smirniotopoulos JG, Moran CJ, Suojanen JN, Vollmer DG. MR imaging of myxopapillary ependymoma: Findings and value to determine extent of tumour and its relation to intraspinal structures. Am J Radiol. 1995;165:1263–67.
18) , 23) , 28) , 29) , 33)
Nakamura M, Ishii K, Watanabe K, et al. Long-term surgical outcomes for myxopapillary ependymomas of the cauda equine. Spine. 2009;34:E756–760.
19) , 24)
Sun B, Wang C, Wang J, Liu A. MRI features of intramedullary spinal cord ependymomas. J Neuroimaging. 2003;13:346–351.
25)
Ngo TP, Dufton J, Stern PJ, Islam O. Myxopapillary ependymoma as a cause of back pain in a young male - A case report. J Can Chiropr Assoc. 2013 Jun;57(2):150-5. PubMed PMID: 23754860; PubMed Central PMCID: PMC3661182.
34)
Tsai CJ, Wang Y, Allen PK, Mahajan A, McCutcheon IE, Rao G, Rhines LD, Tatsui CE, Armstrong TS, Maor MH, Chang EL, Brown PD, Li J. Outcomes after surgery and radiotherapy for spinal myxopapillary ependymoma: update of the MD anderson cancer center experience. Neurosurgery. 2014 Sep;75(3):205-14. doi: 10.1227/NEU.0000000000000408. PubMed PMID: 24818785.
35)
Abdallah A. Spinal Seeding Metastasis of Myxopapillary Ependymoma: Report of Three Pediatric Patients and a Brief Literature Review [published online ahead of print, 2020 Aug 10]. Pediatr Neurosurg. 2020;1-14. doi:10.1159/000509061
36)
Chen X, Li C, Che X, Chen H, Liu Z. Spinal myxopapillary ependymomas: a retrospective clinical and immunohistochemical study. Acta Neurochir (Wien). 2016 Jan;158(1):101-7. doi: 10.1007/s00701-015-2637-8. Epub 2015 Nov 17. PubMed PMID: 26577638.
37)
Petersen D, Lystad RP. Spinal myxopapillary ependymoma in an adult male presenting with recurrent acute low back pain: a case report. Chiropr Man Therap. 2016 Apr 18;24:11. doi: 10.1186/s12998-016-0094-y. eCollection 2016. PubMed PMID: 27092234; PubMed Central PMCID: PMC4834819.
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