Subependymoma

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mohamadmostafa Jahansouz M.D.[2]Sujit Routray, M.D. [3]

Historical Perspective

Subependymoma was first discovered by Ilya Mark Scheinker, a Russian physician, in 1945.[1]

Classification

There is no established system for the classification of subependymoma.

Pathophysiology

Pathogenesis

Subependymoma arises from subependymal glial cells, although it can also arise from astrocytes from the subependymal plate, ependymal cells, and mixed ependymal and astrocytic cells.[2][3]

Gross Pathology

Subependymoma is most commonly seen in the fourth ventricle, but it can arise anywhere where there is ependyma. The distribution in the ventricular system is as follows:[4][3]

  • On gross pathology, subependymoma is characterized by a small, white to grey, firm, well circumscribed, solid, avascular mass attached to the ventricular wall by a narrow pedicle.[2][3]

Microscopic Pathology

On microscopic histopathological analysis, subependymoma is characterized by the following features:[4]

Immunohistochemistry

Subependymoma is characterized by positive tumor marker GFAP. Mixed populations of cells may be variably positive for:[4][5]

Causes

The cause of the development of subependymoma has not been identified.

Differentiating Subependymoma from Other Diseases

Subependymoma must be differentiated from:[6][3]

Epidemiology and Demographics

Frequency and Incidence

Age

  • Patients of all age groups may develop subependymoma.
  • Subependymoma is a rare disease that tends to affect middle-aged adults and the elderly population (typically in the 5th to 6th decades).[9]

Gender

  • Males are more commonly affected with subependymoma than females.
  • The male to female ratio is approximately 2.3 to 1.[9]

Risk Factors

The risk factors in the development of subependymoma are not well defined.

Natural History, Complications, and Prognosis

Diagnosis

Symptoms

Physical Examination

Laboratory Findings

There are no specific laboratory findings associated with subependymoma.

Electrocardiogram

There are no ECG findings associated with subependymoma.

X-ray

There are no x-ray findings associated with subependymoma.

Echocardiography or Ultrasound

There are no echocardiography/ultrasound findings associated with sybependymoma.

CT scan

Head CT scan is helpful in the diagnosis of subependymoma. On CT scan, subependymoma is characterized by:[20]

MRI

Brain MRI is helpful in the diagnosis of subependymoma. On MRI, subependymoma is characterized by:

MRI component Findings

T1 weighted image

T2 weighted image

T1 weighted image with contrast

  • Little or no enhancement

Other Imaging Findings

There are no other imaging findings associated with subependymoma.

Other Diagnostic Studies

There are no other diagnostic studies associated with subependymoma.

Medical Therapy

There is no medical therapy available for the treatment of subependymoma.

Surgery

Surgery is the mainstay of therapy for subependymoma. Incidental intraventricular subependymoma can be managed conservatively through MRI surveillance. Surgical resection is indicated for:[3][21][22]

Primary Prevention

There are no established measures for the secondary prevention of subependymoma.

Secondary Prevention

There are no established measures for the primary prevention of subependymoma.

References

  1. Kurian KM, Jones DT, Marsden F, Openshaw SW, Pearson DM, Ichimura K; et al. (2008). "Genome-wide analysis of subependymomas shows underlying chromosomal copy number changes involving chromosomes 6, 7, 8 and 14 in a proportion of cases". Brain Pathol. 18 (4): 469–73. doi:10.1111/j.1750-3639.2008.00148.x. PMC 2659379. PMID 18397339.
  2. 2.0 2.1 2.2 Saad AF, Bidiwala SB, Layton KF, Snipes GJ, Opatowsky MJ (2013). "Fourth ventricular subependymoma presenting as worsening headache". Proc (Bayl Univ Med Cent). 26 (1): 52–4. PMC 3523772. PMID 23382616.
  3. 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 Jain A, Amin AG, Jain P, Burger P, Jallo GI, Lim M; et al. (2012). "Subependymoma: clinical features and surgical outcomes". Neurol Res. 34 (7): 677–84. doi:10.1179/1743132812Y.0000000064. PMC 4618470. PMID 22747714.
  4. 4.0 4.1 4.2 Pathology of subependymoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/subependymoma. Accessed on January 12, 2016
  5. 5.0 5.1 D'Amico RS, Praver M, Zanazzi GJ, Englander ZK, Sims JS, Samanamud JL; et al. (2017). "Subependymomas Are Low-Grade Heterogeneous Glial Neoplasms Defined by Subventricular Zone Lineage Markers". World Neurosurg. 107: 451–463. doi:10.1016/j.wneu.2017.08.009. PMID 28804038.
  6. Intraventricular neoplasms and lesions. Dr Henry Knipe and Dr Vinod G Maller et al. Radiopaedia 2016. http://radiopaedia.org/articles/intraventricular-neoplasms-and-lesions. Accessed on January 12, 2016
  7. Matsumura A, Ahyai A, Hori A, Schaake T (1989). "Intracerebral subependymomas. Clinical and neuropathological analyses with special reference to the possible existence of a less benign variant". Acta Neurochir (Wien). 96 (1–2): 15–25. PMID 2929389.
  8. Kurukumbi M, Muley A, Ramidi G, Wynn Z, Trouth AJ (2011). "A rare case of subependymoma with an atypical presentation: a case report". Case Rep Neurol. 3 (3): 227–32. doi:10.1159/000333061. PMC 3223030. PMID 22121350.
  9. 9.0 9.1 Epidemiology of subependymoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/subependymoma. Accessed on January 12, 2016
  10. 10.0 10.1 Clinical presentation of subependymoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/subependymoma. Accessed on January 12, 2016
  11. Ragel BT, Osborn AG, Whang K, Townsend JJ, Jensen RL, Couldwell WT (2006). "Subependymomas: an analysis of clinical and imaging features". Neurosurgery. 58 (5): 881–90, discussion 881-90. doi:10.1227/01.NEU.0000209928.04532.09. PMID 16639322.
  12. Prayson RA, Suh JH (1999). "Subependymomas: clinicopathologic study of 14 tumors, including comparative MIB-1 immunohistochemical analysis with other ependymal neoplasms". Arch Pathol Lab Med. 123 (4): 306–9. doi:10.1043/0003-9985(1999)123<0306:S>2.0.CO;2. PMID 10320142.
  13. KE, Changshu. "Subependymoma: a case report and the review of literatures". doi:10.3969/j.issn.1672-6731.2011.01.021.
  14. Park YK, Choi WS, Leem W, Kim YW, Yang MH (1990). "Symptomatic subependymoma--a case report". J Korean Med Sci. 5 (2): 111–5. doi:10.3346/jkms.1990.5.2.111. PMC 3053733. PMID 2278665.
  15. Bokhari R, Ghanem A, Alahwal M, Baeesa S (2013). "Primary isolated lymphoma of the fourth ventricle in an immunocompetent patient". Case Rep Oncol Med. 2013: 614658. doi:10.1155/2013/614658. PMC 3625557. PMID 23607015.
  16. Hamilton W, Kernick D (2007). "Clinical features of primary brain tumours: a case-control study using electronic primary care records". Br J Gen Pract. 57 (542): 695–9. PMC 2151783. PMID 17761056.
  17. Wilne SH, Ferris RC, Nathwani A, Kennedy CR (2006). "The presenting features of brain tumours: a review of 200 cases". Arch Dis Child. 91 (6): 502–6. doi:10.1136/adc.2005.090266. PMC 2082784. PMID 16547083.
  18. Perkins A, Liu G (2016). "Primary Brain Tumors in Adults: Diagnosis and Treatment". Am Fam Physician. 93 (3): 211–7. PMID 26926614.
  19. Bi Z, Ren X, Zhang J, Jia W (2015). "Clinical, radiological, and pathological features in 43 cases of intracranial subependymoma". J Neurosurg. 122 (1): 49–60. doi:10.3171/2014.9.JNS14155. PMID 25361493.
  20. Radiographic features of subependymoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/subependymoma. Accessed on January 12, 2016
  21. Nguyen HS, Doan N, Gelsomino M, Shabani S (2017). "Intracranial Subependymoma: A SEER Analysis 2004-2013". World Neurosurg. 101: 599–605. doi:10.1016/j.wneu.2017.02.019. PMID 28232153.
  22. Varma A, Giraldi D, Mills S, Brodbelt AR, Jenkinson MD (2018). "Surgical management and long-term outcome of intracranial subependymoma". Acta Neurochir (Wien). 160 (9): 1793–1799. doi:10.1007/s00701-018-3570-4. PMC 6105212. PMID 29915887.


References

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