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Central Nervous System Epidermoid Cyst

MedGen UID:
163485
•Concept ID:
C0854911
•
Anatomical Abnormality
Synonyms: Central Nervous System Epidermoid Neoplasm; Central Nervous System Epidermoid Tumor; CNS Epidermoid Cyst; CNS Epidermoid Neoplasm; CNS Epidermoid Tumor; Epidermoid Cyst of Central Nervous System; Epidermoid Cyst of CNS; Epidermoid Cyst of the Central Nervous System; Epidermoid Cyst of the CNS; Epidermoid Neoplasm of Central Nervous System; Epidermoid Neoplasm of CNS; Epidermoid Neoplasm of the Central Nervous System; Epidermoid Neoplasm of the CNS; Epidermoid Tumor of Central Nervous System; Epidermoid Tumor of CNS; Epidermoid Tumor of the Central Nervous System; Epidermoid Tumor of the CNS

Definition

A lesion which is characterized by an irregularly nodular opalescent wall which has caused it to be termed a ""pearly tumor"". The Intramural contents are soft and waxy with a variable amount of cholesterol. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCentral Nervous System Epidermoid Cyst

Professional guidelines

PubMed

Rare and challenging extra-axial brain lesions: CT and MRI findings with clinico-radiological differential diagnosis and pathological correlation.
Demir MK, Yapıcıer O, Onat E, Toktaş ZO, Akakın A, Urgun K, Kılıç T
Diagn Interv Radiol 2014 Sep-Oct;20(5):448-52. doi: 10.5152/dir.2014.14031. PMID: 25010368Free PMC Article
Keratinized squamous cells in fine needle aspiration of the brain. Cytopathologic correlates and differential diagnosis.
Parwani AV, Taylor DC, Burger PC, Erozan YS, Olivi A, Ali SZ
Acta Cytol 2003 May-Jun;47(3):325-31. doi: 10.1159/000326528. PMID: 12789909
Pineal epidermoid cysts: diagnosis and management.
Konovalov AN, Spallone A, Pitzkhelauri DI
J Neurosurg 1999 Sep;91(3):370-4. doi: 10.3171/jns.1999.91.3.0370. PMID: 10470809

Recent clinical studies

Etiology

Pineal/germ cell tumors and pineal parenchymal tumors.
Ogiwara H, Liao YM, Wong TT
Childs Nerv Syst 2023 Oct;39(10):2649-2665. Epub 2023 Oct 13 doi: 10.1007/s00381-023-06081-1. PMID: 37831207
Combined Endoscopic and Exoscopic Resection of Intracranial Epidermoid Cysts.
Li Z, Gui S, Zhao P, Bai J, Cao L, Cheng S, Liu C, Zhu H, Zhang Y, Li C
World Neurosurg 2022 Dec;168:e28-e33. Epub 2022 Aug 31 doi: 10.1016/j.wneu.2022.08.111. PMID: 36055616
Endonasal endoscopic skullbase surgery in children.
Deopujari CE, Shah NJ, Shaikh ST, Karmarkar VS, Mohanty CB
Childs Nerv Syst 2019 Nov;35(11):2091-2098. Epub 2019 May 12 doi: 10.1007/s00381-019-04167-3. PMID: 31079184
Intraventricular epidermoids.
Meng L, Yuguang L, Shugan Z, Xingang L, Chengyuan W
J Clin Neurosci 2006 May;13(4):428-30. Epub 2006 Mar 27 doi: 10.1016/j.jocn.2005.04.022. PMID: 16564173
Benign cysts in the central nervous system: neuropathological observations of the cyst walls.
Hirano A, Hirano M
Neuropathology 2004 Mar;24(1):1-7. doi: 10.1111/j.1440-1789.2003.00526.x. PMID: 15068166

Diagnosis

Pineal/germ cell tumors and pineal parenchymal tumors.
Ogiwara H, Liao YM, Wong TT
Childs Nerv Syst 2023 Oct;39(10):2649-2665. Epub 2023 Oct 13 doi: 10.1007/s00381-023-06081-1. PMID: 37831207
Nonneoplastic and noninfective cysts of the central nervous system: A histopathological study.
Poyuran R, Kalaparti VSVG, Thomas B, Kesavapisharady K, Narasimhaiah D
Neuropathology 2023 Jun;43(3):221-232. Epub 2022 Oct 10 doi: 10.1111/neup.12867. PMID: 36210745
Low-grade developmental and epilepsy associated brain tumors: a critical update 2020.
Slegers RJ, Blumcke I
Acta Neuropathol Commun 2020 Mar 9;8(1):27. doi: 10.1186/s40478-020-00904-x. PMID: 32151273Free PMC Article
Liponeurocytoma of the Cerebellopontine Angle.
Chiaramonte C, Rabaste S, Jacquesson T, Meyronet D, Cotton F, Jouanneau E, Berhouma M
World Neurosurg 2018 Apr;112:18-24. Epub 2018 Jan 8 doi: 10.1016/j.wneu.2018.01.012. PMID: 29325939
Intraventricular epidermoids.
Meng L, Yuguang L, Shugan Z, Xingang L, Chengyuan W
J Clin Neurosci 2006 May;13(4):428-30. Epub 2006 Mar 27 doi: 10.1016/j.jocn.2005.04.022. PMID: 16564173

Therapy

Subarachnoid fat dissemination secondary to intrathecal pump.
Riestenberg RA, Omofoye OA, Lee D, Shahlaie K
J Clin Neurosci 2020 Aug;78:416-417. Epub 2020 Apr 30 doi: 10.1016/j.jocn.2020.04.120. PMID: 32362469
Epidermoid Cyst of the Lumbar Spine After Lumbar Puncture: A Clinical, Radiographic, and Pathologic Correlation.
Dodson V, Majmundar N, Sharer LR, Gillick JL
World Neurosurg 2020 May;137:363-366. Epub 2020 Feb 10 doi: 10.1016/j.wneu.2020.02.008. PMID: 32058114
Intraventricular epidermoids.
Meng L, Yuguang L, Shugan Z, Xingang L, Chengyuan W
J Clin Neurosci 2006 May;13(4):428-30. Epub 2006 Mar 27 doi: 10.1016/j.jocn.2005.04.022. PMID: 16564173
Neuroendoscopic approach to intraventricular lesions.
Gaab MR, Schroeder HW
J Neurosurg 1998 Mar;88(3):496-505. doi: 10.3171/jns.1998.88.3.0496. PMID: 9488304
Epidermoid and dermoid sequestration cysts.
Pear BL
Am J Roentgenol Radium Ther Nucl Med 1970 Sep;110(1):148-55. doi: 10.2214/ajr.110.1.148. PMID: 5469090

Prognosis

Combined Endoscopic and Exoscopic Resection of Intracranial Epidermoid Cysts.
Li Z, Gui S, Zhao P, Bai J, Cao L, Cheng S, Liu C, Zhu H, Zhang Y, Li C
World Neurosurg 2022 Dec;168:e28-e33. Epub 2022 Aug 31 doi: 10.1016/j.wneu.2022.08.111. PMID: 36055616
Split-Pons Syndrome by Epidermoid Cyst: A Case Report and Review of the Literature.
You E, Bokhari R, Sirhan D
World Neurosurg 2019 Nov;131:275-280.e1. Epub 2019 Jul 12 doi: 10.1016/j.wneu.2019.07.072. PMID: 31306846
Infundibular Epidermoid Cyst: Case Report and Systematic Review.
Khan AB, Goethe EA, Hadley CC, Rouah E, North R, Srinivasan VM, Gallagher KK, Fuentes A, Vaz-Guimaraes F
World Neurosurg 2019 Oct;130:110-114. Epub 2019 Jul 5 doi: 10.1016/j.wneu.2019.06.205. PMID: 31284058
Liponeurocytoma of the Cerebellopontine Angle.
Chiaramonte C, Rabaste S, Jacquesson T, Meyronet D, Cotton F, Jouanneau E, Berhouma M
World Neurosurg 2018 Apr;112:18-24. Epub 2018 Jan 8 doi: 10.1016/j.wneu.2018.01.012. PMID: 29325939
Intraventricular epidermoids.
Meng L, Yuguang L, Shugan Z, Xingang L, Chengyuan W
J Clin Neurosci 2006 May;13(4):428-30. Epub 2006 Mar 27 doi: 10.1016/j.jocn.2005.04.022. PMID: 16564173

Clinical prediction guides

Combined Endoscopic and Exoscopic Resection of Intracranial Epidermoid Cysts.
Li Z, Gui S, Zhao P, Bai J, Cao L, Cheng S, Liu C, Zhu H, Zhang Y, Li C
World Neurosurg 2022 Dec;168:e28-e33. Epub 2022 Aug 31 doi: 10.1016/j.wneu.2022.08.111. PMID: 36055616
Obstructive Hydrocephalus and Chemical Meningitis Secondary to a Ruptured Spinal Epidermoid Cyst.
Fernández-de Thomas RJ, Vicenty-Padilla JC, Sánchez-Jiménez JG, Labat EJ, Carballo-Cuello CM, De Jesús O, Vigo-Prieto JA
World Neurosurg 2019 Dec;132:173-176. Epub 2019 Sep 3 doi: 10.1016/j.wneu.2019.08.187. PMID: 31491571
Craniopharyngioma and other cystic epithelial lesions of the sellar region: a review of clinical, imaging, and histopathological relationships.
Zada G, Lin N, Ojerholm E, Ramkissoon S, Laws ER
Neurosurg Focus 2010 Apr;28(4):E4. doi: 10.3171/2010.2.FOCUS09318. PMID: 20367361
Benign cysts in the central nervous system: neuropathological observations of the cyst walls.
Hirano A, Hirano M
Neuropathology 2004 Mar;24(1):1-7. doi: 10.1111/j.1440-1789.2003.00526.x. PMID: 15068166
Benign cystic lesions in the central nervous system. Light and electron microscopic observations of cyst walls.
Hirano A, Hirano M
Childs Nerv Syst 1988 Dec;4(6):325-33. doi: 10.1007/BF00270605. PMID: 3073001

Recent systematic reviews

Intrasellar Dermoid Cyst: Case Report of a Rare Lesion and Systematic Literature Review Comparing Intrasellar, Suprasellar, and Parasellar Locations.
Donofrio CA, Bertazzoni G, Riccio L, Pinacoli A, Pianta L, Generali D, Ungari M, Servadei F, Roncaroli F, Fioravanti A
World Neurosurg 2024 Feb;182:83-90. Epub 2023 Nov 22 doi: 10.1016/j.wneu.2023.11.057. PMID: 37995988
Infundibular Epidermoid Cyst: Case Report and Systematic Review.
Khan AB, Goethe EA, Hadley CC, Rouah E, North R, Srinivasan VM, Gallagher KK, Fuentes A, Vaz-Guimaraes F
World Neurosurg 2019 Oct;130:110-114. Epub 2019 Jul 5 doi: 10.1016/j.wneu.2019.06.205. PMID: 31284058
Suprasellar Epidermoid Cyst: Case Report of Extended Endoscopic Transsphenoidal Resection and Systematic Review of the Literature.
Bobeff EJ, Sánchez-Viguera C, Arráez-Manrique C, Arráez-Sánchez MÁ
World Neurosurg 2019 Aug;128:514-526. Epub 2019 May 20 doi: 10.1016/j.wneu.2019.05.100. PMID: 31121364
Predictors of Outcomes in Fourth Ventricular Epidermoid Cysts: A Case Report and a Review of Literature.
Chung LK, Beckett JS, Ong V, Lagman C, Nagasawa DT, Yang I, Kim W
World Neurosurg 2017 Sep;105:689-696. Epub 2017 Jun 12 doi: 10.1016/j.wneu.2017.06.037. PMID: 28619489

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