Ependymoma classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Ahmad Al Maradni, M.D. [2]

Overview

Ependymoma may be classified into several subtypes based on WHO classification (grade I, II, III) and the site of origin.

Classification

  • In the most recent World Health Organization (WHO) classification of brain tumors, ependymal tumors are classified into the following four main subtypes:[1][2]
  • Myxopapillary ependymoma (WHO grade I): a myxopapillary ependymoma arises almost exclusively in the location of the conus medullaris, cauda equina, and filum terminale of the spinal cord and is characterized histologically by tumor cells arranged in a papillary manner around vascularized myxoid stromal cores.[4]
  • Ependymoma (WHO grade II): the ependymoma, which is considered a grade II neoplasm originating from the walls of the ventricles or from the spinal canal, is composed of neoplastic ependymal cells. Ependymomas are subdivided, based on histological findings, into the following four subtypes[5]:
  • Cellular ependymoma: the most common subtype, this subtype usually demonstrates significant cellularity without an increase in mitotic activity[6].
  • Papillary ependymoma: forms linear, epithelial-like surfaces along cerebrospinal fluid.
  • Clear cell ependymoma: displays an oligodendroglial-like appearance with perinuclear halos, this variant is preferentially located in the supratentorial compartment of the brain[7].
  • Tanycytic ependymoma: the rarest form of grade II ependymoma, this subtype is most commonly found in the spinal cord; tumor cells are arranged in fascicles of variable width and cell density and are poorly intertwined[8].
  • Anaplastic ependymoma (WHO grade III): also known as malignant ependymoma. An anaplastic ependymoma is considered a malignant glioma of ependymal differentiation and, compared with the grade II ependymomas, shows increased cellularity and increased mitotic activity. It is often associated with microvascular proliferation and necrosis[9].
  • Ependymal tumors are also classified based on their sites of origin into:[2][10]

References

  1. Louis DN, Ohgaki H, Wiestler OD, et al., eds.: WHO Classification of Tumours of the Central Nervous System. 4th ed. Lyon, France: IARC Press, 2007.
  2. 2.0 2.1 Eoendymoma. http://www.cancer.gov/types/brain/hp/child-ependymoma-treatment-pdq#section/_35 URL Accessed on 10 6 2015.
  3. Huang Y (2015). "Subependymoma with extensive microcystic transformation: a case report". Int J Clin Exp Pathol. 8 (2): 2191–4. PMC 4396238. PMID 25973126.
  4. Weber DC, Wang Y, Miller R, Villà S, Zaucha R, Pica A; et al. (2015). "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. 17 (4): 588–95. doi:10.1093/neuonc/nou293. PMC 4483075. PMID 25301811.
  5. Grier JT, Batchelor T (2006). "Low-grade gliomas in adults". Oncologist. 11 (6): 681–93. doi:10.1634/theoncologist.11-6-681. PMID 16794247.
  6. Wu J, Armstrong TS, Gilbert MR (2016). "Biology and management of ependymomas". Neuro Oncol. 18 (7): 902–13. doi:10.1093/neuonc/now016. PMC 4896548. PMID 27022130.
  7. Lee BH, Kwon JT, Park YS (2011). "Supratentorial clear cell ependymoma mimicking oligodendroglioma : case report and review of the literature". J Korean Neurosurg Soc. 50 (3): 240–3. doi:10.3340/jkns.2011.50.3.240. PMC 3218185. PMID 22102956.
  8. Tomek M, Jayajothi A, Brandner S, Jaunmuktane Z, Lee CH, Davagnanam I (2016). "Imaging features of spinal tanycytic ependymoma". Neuroradiol J. 29 (1): 61–5. doi:10.1177/1971400915621322. PMC 4978340. PMID 26755489.
  9. Sarıkafa Ş, Çelik SE, Yarikkaya E, Sayılgan A (2015). "Malignant Transformation of Grade II Ependymoma in a 2-Year-Old Child: Case Report". J Neurol Surg Rep. 76 (1): e151–5. doi:10.1055/s-0035-1549311. PMC 4520988. PMID 26251794.
  10. Andreiuolo F, Puget S, Peyre M, Dantas-Barbosa C, Boddaert N, Philippe C; et al. (2010). "Neuronal differentiation distinguishes supratentorial and infratentorial childhood ependymomas". Neuro Oncol. 12 (11): 1126–34. doi:10.1093/neuonc/noq074. PMC 3098029. PMID 20615923.
  11. Mangalore S, Aryan S, Prasad C, Santosh V (2015). "Imaging characteristics of supratentorial ependymomas: Study on a large single institutional cohort with histopathological correlation". Asian J Neurosurg. 10 (4): 276–81. doi:10.4103/1793-5482.162702. PMC 4558802. PMID 26425155.
  12. Mandera M, Makarska J, Sobol G, Musioł K (2015). "Infratentorial ependymomas--a study of the centre in Katowice". Childs Nerv Syst. 31 (7): 1089–96. doi:10.1007/s00381-015-2683-9. PMC 4493855. PMID 25822933.
  13. Armstrong TS, Vera-Bolanos E, Bekele BN, Aldape K, Gilbert MR (2010). "Adult ependymal tumors: prognosis and the M. D. Anderson Cancer Center experience". Neuro Oncol. 12 (8): 862–70. doi:10.1093/neuonc/noq009. PMC 2940672. PMID 20511182.

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