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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
Surgical Treatment of Congenital Dermal Sinus: An Experience of 56 Cases.
Song Y, Xia Z, Qiu S, Gao P, Yang B, Bao N
Pediatr Neurosurg 2021;56(5):416-423. Epub 2021 Aug 5 doi: 10.1159/000515515. PMID: 34352798
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
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
Epidermoid cyst.
Roosendaal M, Ceulemans R
J Belge Radiol 1997 Dec;80(6):309. PMID: 9479907

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
Epidermoid tumour of the lateral ventricle.
Bhatoe HS, Mukherji JD, Dutta V
Acta Neurochir (Wien) 2006 Mar;148(3):339-42; discussion 342. Epub 2005 Nov 28 doi: 10.1007/s00701-005-0678-0. PMID: 16311839
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

Posterior fossa epidermoid tumors: a single-center study and proposed classification system.
Bayatli E, Ozgural O, Eroglu U, Dogan I, Hasimoglu S, Bozkurt M, Kahilogullari G, Ugur HC, Unlu A
Br J Neurosurg 2024 Oct;38(5):1091-1099. Epub 2022 Jan 17 doi: 10.1080/02688697.2021.2022099. PMID: 35037530
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
Surgical Treatment of Congenital Dermal Sinus: An Experience of 56 Cases.
Song Y, Xia Z, Qiu S, Gao P, Yang B, Bao N
Pediatr Neurosurg 2021;56(5):416-423. Epub 2021 Aug 5 doi: 10.1159/000515515. PMID: 34352798
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

Posterior fossa epidermoid tumors: a single-center study and proposed classification system.
Bayatli E, Ozgural O, Eroglu U, Dogan I, Hasimoglu S, Bozkurt M, Kahilogullari G, Ugur HC, Unlu A
Br J Neurosurg 2024 Oct;38(5):1091-1099. Epub 2022 Jan 17 doi: 10.1080/02688697.2021.2022099. PMID: 35037530
Brain MRI findings in patients with bullous pemphigoid: A case-control study.
Ganji R, Mahboubi-Fooladi Z, Shahidi-Dadras M, Tehranchinia Z, Abdollahimajd F, Ghalamkarpour F, Robati RM, Gheisari M, Mozafari N, Dadkhahfar S, Nasiri S
Exp Dermatol 2023 Apr;32(4):542-546. Epub 2023 Jan 29 doi: 10.1111/exd.14748. PMID: 36645025
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
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

Recent systematic reviews

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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