Subependymoma
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Syed Musadiq Ali M.B.B.S.[2]Mohamadmostafa Jahansouz M.D.[3]Sujit Routray, M.D. [4]
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]
- Fourth ventricle: 50 - 60%
- Lateral ventricles (usually frontal horns): 30 - 40%
- Third ventricle: rare
- Central canal of the spinal cord: rare
- 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]
- Microcystic spaces and bland appearing cells without appreciable nuclear atypia or mitoses.
- The nuclei tend to form clusters.
- No high grade features (mitoses, Ki-67 / MIBI index > 1.5%, necrosis) are present.
- Loose pseudorosettes are observed.
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]
- Neoplasms of the ventricular wall and septum pellucidum
- Neoplasms of the choroid plexus
- Choroid plexus papilloma and carcinoma
- Others
Epidemiology and Demographics
Frequency and Incidence
- The frequency of asymptomatic subependymomas was 0.4% in 1,000 serial routine necropsies and 0.7% in symptomatic subependymomas from 1,000 serial surgical specimens of intracranial neoplasms.[7]
- The incidence of subependymoma was estimated to be 0.7 cases per 100,000 individuals with pathologically proven intracranial neoplasms.[8]
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
- If left untreated, patients with subependymoma may progress to develop seizures and obstructive hydrocephalus.[10]
- Subependymoma is a slow-growing tumor with an indolent course.[11][5]
- Obstructive hydrocephalus is a common complication of subependymoma.[10]
- The prognosis of subependymoma is excellent with complete excision of the tumor.[2][12]
- Common complications of subependymoma are hydrocephalus and focal neurological deficits due to mass effect.[3]
Diagnosis
Symptoms
- Typically patients of subependymoma are asymptomatic and small lesions are discovered incidentally.
- Symptoms of subependymoma include:[13][14][3]
- Symptoms due to elevated intracranial pressure
- Neurological symptoms
- Seizures
- Sudden loss of awareness
- Transient loss of memory
Physical Examination
- Patients with subependymoma usually appear normal.
- Physical examination may be remarkable for:[15][16][17][18][3][19]
- Abnormal pupillary reflex
- Visual field defects
- Gait changes
- Bilateral Babinski sign
- Depressed Glasgow coma score (GCS)
- Decreased muscle strength
- Decreased deep tendon reflexes
- Sensation defects
- Hearing problems and abnormal Rinne and Weber tests
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]
- Iso- and hypodense intraventricular mass
- Positive mass effect
- No enhancement
- If large, it may have cystic or even calcific components
- No vasogenic edema
MRI
Brain MRI is helpful in the diagnosis of subependymoma. On MRI, subependymoma is characterized by:
| MRI component | Findings |
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T1 weighted image |
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T2 weighted image |
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T1 weighted image with contrast |
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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]
- Symptomatic tumors
- Tumors without a clear imaging diagnosis
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
- ↑ 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.
- ↑ 2.0 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.
- ↑ 3.0 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.
- ↑ 4.0 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
- ↑ 5.0 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.
- ↑ 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
- ↑ 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.
- ↑ 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.
- ↑ 9.0 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
- ↑ 10.0 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
- ↑ 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.
- ↑ 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.
- ↑ KE, Changshu. "Subependymoma: a case report and the review of literatures". doi:10.3969/j.issn.1672-6731.2011.01.021.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ Perkins A, Liu G (2016). "Primary Brain Tumors in Adults: Diagnosis and Treatment". Am Fam Physician. 93 (3): 211–7. PMID 26926614.
- ↑ 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.
- ↑ 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
- ↑ 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.
- ↑ 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.