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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 2  |  Issue : 2  |  Page : 107-109

Myxopapillary ependymoma: A rare case presentation


1 Department of Pathology, Faculty of Medicine, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India
2 Department of Radiation Oncology, Faculty of Medicine, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India

Date of Submission11-May-2022
Date of Decision01-Jun-2022
Date of Acceptance02-Jun-2022
Date of Web Publication18-Nov-2022

Correspondence Address:
Dr. Kafil Akhtar
Department of Pathology, Faculty of Medicine, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/aort.aort_14_22

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  Abstract 

Ependymomas are the preponderant glial tumors of the spinal cord. Myxopapillary ependymoma (MPE) is a subtype of ependymoma, first described by Kernohan in 1932. This is a slow-growing, benign tumor. It occurs in the third to fifth decade of life and constitutes 0.5% of cases of ependymomas. MPE is an intradural lesion that arises mainly in the conus medullaris, cauda equina, and filum terminale. The presenting features of these fleshly, sausage-shaped, vascular lesions are chronic low back pain with or without sciatica. Magnetic resonance imaging is helpful in the diagnosis of the primary and recurrent lesions. Regular close follow-up is recommended for better prognosis. We present a rare case of MPE in a 50-year-old male who presented with lower back pain and retention of urine for the last 1 year.

Keywords: Ependymoma, histopathology, myxopapillary


How to cite this article:
Akhtar K, Tirkey RC, Hussain B. Myxopapillary ependymoma: A rare case presentation. Ann Oncol Res Ther 2022;2:107-9

How to cite this URL:
Akhtar K, Tirkey RC, Hussain B. Myxopapillary ependymoma: A rare case presentation. Ann Oncol Res Ther [serial online] 2022 [cited 2022 Nov 29];2:107-9. Available from: http://www.aort.com/text.asp?2022/2/2/107/361489


  Introduction Top


Ependymomas arise from the ependymal cells lining the ventricles and the central canal of the spinal cord.[1] They comprise 5%–6.0% of all intracranial gliomas and 60.0% of spinal cord gliomas.[1] Myxopapillary ependymoma (MPE) is a subtype of ependymoma, which exclusively involves conus medullaris, cauda equina, and filum terminale.[2]

MPE is a slow-growing benign tumor and constitutes 0.5% of cases of ependymomas.[2] The mean age of occurrence is the third to fifth decade of life, with a male preponderance.[2] These are uncommon in children.[3]

The most common presenting feature is chronic low back pain with or without sciatica, seen in 96.0% of the cases.[4] Grossly, MPE shows microcystic vacuoles and mucoid material admixed with loose connective tissue. Microscopically, feature of anaplasia is lacking and systemic spread is rare, but it can spread throughout the neural axis via the cerebral spinal fluid (CSF).[2] Metastatic spread is common in children than in adults at presentation.[5],[6]


  Case Report Top


A 50-year-old male presented to the surgery clinics with complaints of pain in the lower back for the last 1 year and inability to hold feces and retention of urine for 1 month. On physical examination, the reflexes in the bilateral lower limb were exaggerated and power was reduced to 3/5.

Contrast-enhanced magnetic resonance imaging (MRI) of the lumbosacral spine revealed an intradural soft tissue intensity mass. The mass was 3.7 cm × 1.9 cm × 1.6 cm in size, localized in the anterior aspect of the spinal canal at D12–L1 level. This moderately enhancing lesion was seen compressing the conus medullaris and cauda equina nerve roots and was suggestive of a nerve sheath tumor.

Intraoperatively, a yellowish gray, moderately vascular, encapsulated tumor was seen arising from the left L1 nerve root compressing the cord and pushing it to the right side. Microscopic examination showed papillae with fibrovascular cores, lined by atypical cuboidal-to-low columnar cells with round-to-oval nuclei, fine granular chromatin, few inconspicuous nucleoli, and moderate eosinophilic cytoplasm [Figure 1]. Focally, the tumor cells showed spindled nuclei and fascicular arrangement. Numerous scattered microcyts filled with myxoid material were also seen [Figure 2]. Large areas of hemorrhage admixed with foci of necrosis and 0–1 mitosis per 10 high-power fields were also noted.
Figure 1: Microscopic examination showing papillaroid arrangement of atypical cuboidal-to-low columnar cells having round-to-oval nuclei, fine chromatin, inconspicuous nucleoli, and moderate eosinophilic cytoplasm (H & E stain, ×10)

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Figure 2: The papillae had fibrovascular cores and focally the tumor cells showing spindled nuclei and fascicular arrangement. Numerous scattered microcyts filled with myxoid material was also seen (H & E stain, ×40)

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On immunohistochemistry, the tumor cells showed diffuse strong cytoplasmic positivity for S100 [Figure 3] and glial fibrillary acidic protein (GFAP) [Figure 4] and focal weak cytoplasmic positivity for vimentin. Our patient was administered standard 6-week regimen of 50 Gy of Co-60 teletherapy with adjuvant chemotherapy comprising temozolomide, 100 mg/day, 5 days in a week for 12 weeks. He remains completely symptom-free and well 6 months into follow-up.
Figure 3: Immunohistochemistry showing tumor cells with diffuse strong cytoplasmic positivity for S100 (IHC S100, ×40)

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Figure 4: Immunohistochemistry showing tumor cells with diffuse strong cytoplasmic positivity for GFAP (IHC GFAP, ×40), GFAP = Glial fibrillary acidic protein

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  Discussion Top


MPE, as a variant of ependymoma, was first described by Kernohan in 1932.[7] It commonly originates in the cauda equina and filum terminale. Cauda equina MPEs arise from the ependymal cell nests normally present in the filum terminale. MPE is a benign mucoid tumor, characterized by papillary architecture and mucin production.[2]

MPE is rare in children and seen in adults in the third and fourth decades of life. The most common clinically feature is lumbar, sacral, or radicular pain.[7] Sensory changes, motor deficits, altered bladder, and bowel habits are seen in a few cases.[8],[9]

The precise diagnosis of MPE depends on radiographic imaging and histopathological examination of the lesion. MRI is a sensitive and specific diagnostic tool to highlight an hypodense or hyperdense intradural mass of MPE with intravenous contrast.[9] Grossly, the lesion is fleshly, sausage shaped, and vascular. The distinctive microscopic features of MPE are pseudopapillae formation lined by bland euchromatic cells with foci of vascular proliferation in a mucoid matrix.[8],[10] Immunohistochemically, the tumor cells show cytoplasmic positivity for GFAP and vimentin.[11],[12]

The treatment modality in MPE is mainly wide surgical excision.[13] Huang and Lin have reported that total surgical resection is possible in 80%–90.0% of spinal ependymomas.[14] Radiation therapy is also a treatment option, in large deep seated lesions, when en bloc excision cannot be performed.[15] The role of systemic therapy in the treatment of MPE remains largely unknown. There is limited evidence that systemic therapy can be curative. Some studies, however, suggest that tyrosine kinase inhibitors can halt disease progression.[16]

Metastasis is rarely seen in MPE and occurs if the tumor capsule is ruptured and if tumor is not confined to the filum terminale. The tumor usually disseminates via the CSF and infiltrates into the conus medullaris.[6],[16] The overall survival in MPE is good, but local recurrence is seen in 50.0% of the cases with large tumor burden at 2–15 years after adequate surgical excision.[14]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Louis DN, Perry A, Reifenberger G, von Deimling A, Figarella-Branger D, Cavenee WK, et al. The 2016 World Health Organization classification of tumors of the central nervous system: A summary. Acta Neuropathol 2016;131:803-20.  Back to cited text no. 1
    
2.
McLendon RE, Rosenblum MK, Schiffer D. Myxopapillary ependymoma. In: Louis DN, Ohgaki H, Weistler OD, Cavenee WK, Ellison DW, Figarella-Branger D, et al. editors. WHO Classification of Tumors of the Central Nervous System. Lyon: International Agency for Research on Cancer 2016:104-105.  Back to cited text no. 2
    
3.
Weber DC, Wang Y, Miller R, Villà S, Zaucha R, Pica A, et al. 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 Oncol 2015;17:588-95.  Back to cited text no. 3
    
4.
Ilhan A, Furtner J, Birner P, Rössler K, Marosi C, Preusser M. Myxopapillary ependymoma with pleuropulmonary metastases and high plasma glial fibrillary acidic protein levels. J Clin Oncol 2011;29:e756-7.  Back to cited text no. 4
    
5.
Straus D, Tan LA, Takagi I, O'Toole JE. Disseminated spinal myxopapillary ependymoma in an adult at initial presentation: A case report and review of the literature. Br J Neurosurg 2014;28:691-3.  Back to cited text no. 5
    
6.
Pencovich N, Bot G, Lidar Z, Korn A, Wostrack M, Meyer B, et al. Spinal ependymoma with regional metastasis at presentation. Acta Neurochir (Wien) 2014;156:1215-22.  Back to cited text no. 6
    
7.
Kernohan JW. Primary tumors of the spinal cord and intradural filum terminale. In: Penfield W, editor. Cytology and Cellular Pathology of the Nervous System. 3rd ed. New York: Hoeber; 2010. p. 993-1025.  Back to cited text no. 7
    
8.
Gilbert MR, Ruda R, Soffietti R. Ependymomas in adults. Curr Neurol Neurosci Rep 2010;10:240-7.  Back to cited text no. 8
    
9.
Rege SV, Narayan S, Patil H, Songara A. Spinal myxopapillary ependymoma with interval drop metastasis presenting as cauda equina syndrome: Case report and review of literature. J Spine Surg 2016;2:216-21.  Back to cited text no. 9
    
10.
Pajtler KW, Witt H, Sill M, Jones DT, Hovestadt V, Kratochwil F, et al. Molecular classification of ependymal tumors across all CNS compartments, histopathological grades, and age groups. Cancer Cell 2015;27:728-43.  Back to cited text no. 10
    
11.
Witt H, Gramatzki D, Hentschel B, Pajtler KW, Felsberg J, Schackert G, et al. DNA methylation-based classification of ependymomas in adulthood: Implications for diagnosis and treatment. Neuro Oncol 2018;20:1616-24.  Back to cited text no. 11
    
12.
Tsai CJ, Wang Y, Allen PK, Mahajan A, McCutcheon IE, Rao G, et al. Outcomes after surgery and radiotherapy for spinal myxopapillary ependymoma: Update of the MD Anderson Cancer Center experience. Neurosurgery 2014;75:205-14.  Back to cited text no. 12
    
13.
Abdulaziz M, Mallory GW, Bydon M, De la Garza Ramos R, Ellis JA, Laack NN, et al. Outcomes following myxopapillary ependymoma resection: The importance of capsule integrity. Neurosurg Focus 2015;39:E8.  Back to cited text no. 13
    
14.
Huang YH, Lin JW. Management and outcome of primary spinal ependymomas: A single center experience from Taiwan. Clin Neurol Neurosurg 2013;115:2130-5.  Back to cited text no. 14
    
15.
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. J Neurosurg Spine 2013;19:471-6.  Back to cited text no. 15
    
16.
Batich KA, Riedel RF, Kirkpatrick JP, Tong BC, Eward WC, Tan CL, et al. Recurrent extradural myxopapillary ependymoma with oligometastatic spread. Front Oncol 2019;9:1322.  Back to cited text no. 16
    


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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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