|Year : 2018 | Volume
| Issue : 4 | Page : 1288-1291
Surgical management of intracranial giant epidermoid cysts in adult: A case-based update
Antonella Mangraviti1, Edoardo Mazzucchi1, Alessandro Izzo1, Cosimo Sturdà1, Alessio Albanese1, Enrico Marchese1, Alessandro Olivi1, Alfredo Puca1, Carmelo Lucio Sturiale2
1 Institute of Neurosurgery, Fondazione Policlinico Universitario A. Gemelli – IRCCS, Università Cattolica del Sacro Cuore, Rome, Italy
2 Institute of Neurosurgery, Fondazione Policlinico Universitario A. Gemelli – IRCCS, Rome, Italy
|Date of Web Publication||23-Oct-2018|
Dr. Carmelo Lucio Sturiale
Institute of Neurosurgery, Fondazione Policlinico Universitario A. Gemelli – IRCCS, Largo A. Gemelli 8, 00168 Rome
Source of Support: None, Conflict of Interest: None
Epidermoid cysts (ECs) are benign and slow-growing lesions that account for about 0.2%–2% of all intracranial tumors. Symptoms appear slowly and tumors may have already grown to giant proportions when patients receive their first diagnosis. The optimal treatment for ECs is surgical removal, which includes the total resection of the entire capsule of the lesion in order to minimize the risk of malignant transformation associated with partial removal. However, considering the giant size that the ECs can reach at the time of the diagnosis, and their adherence to the surrounding structures, the risks and benefits of total versus subtotal resections in the short- and long-term patients' outcome are still under debate. Here, we report a case of an extensive giant EC and offer a discussion of its characteristics, surgical management, and postoperative outcome, taking a cue to argue about the recent literature based in the latest case studies.
Keywords: Adult, brain cyst, giant epidermoid, surgical treatment, total resection
|How to cite this article:|
Mangraviti A, Mazzucchi E, Izzo A, Sturdà C, Albanese A, Marchese E, Olivi A, Puca A, Sturiale CL. Surgical management of intracranial giant epidermoid cysts in adult: A case-based update. Asian J Neurosurg 2018;13:1288-91
|How to cite this URL:|
Mangraviti A, Mazzucchi E, Izzo A, Sturdà C, Albanese A, Marchese E, Olivi A, Puca A, Sturiale CL. Surgical management of intracranial giant epidermoid cysts in adult: A case-based update. Asian J Neurosurg [serial online] 2018 [cited 2019 Dec 15];13:1288-91. Available from: http://www.asianjns.org/text.asp?2018/13/4/1288/243739
| Introduction|| |
Epidermoid cysts (ECs) are benign and slow-growing masses that account for about 0.2%–2% of all intracranial tumors. ECs are congenital and formed out of aberrant ectodermal cells, which become trapped during the embryogenesis between the 3rd and 5th gestational weeks. They histopathologically appear as pearly lesions lined with stratified squamous keratinizing epithelium.
Intracranial ECs are most often intradural, but they can also occur extradurally in the intradiploic space in up to 10% of cases. They tend to insinuate into several intracranial compartments by filling the subarachnoid space. The most common intradural locations are cerebellopontine angle (60%), fourth ventricle (5%–18%), parasellar area, and middle cranial fossa (15%); less frequently, they are located within ventricles and brain parenchyma., ECs of the posterior fossa usually arise in the lateral subarachnoid cisterns, and those involving the brainstem and spine are rare.,,,,,
Surgical resection is the only affective treatment,, but the debate is still open regarding the advantages of a total resection versus a decompressive procedure with subtotal removal. Relatively few case series have been yet reported on giant ECs, and accounts vary in consideration of recurrence risk and patient outcomes in the short and long term [Table 1].,,,,,,,,,,
|Table 1: Summary of studies (case series) about surgery of giant epidermoid cysts|
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Here, we report a case of a giant EC and provide a discussion of its characteristics, surgical management, and postoperative outcome, taking a cue to argue about the recent pertinent literature.
| Case Report|| |
A 60-year-old woman presented with a history of mild right hemiparesis, complex partial seizures, aphasia, and gait disturbances, without headache. Neurological examination confirmed the right hemiparesis, gait ataxia, and nystagmus on lateral gaze. Magnetic resonance imaging (MRI) showed a giant cystic lesion (90 mm × 80 mm × 60 mm) centered in the third ventricle, with bilateral extension toward cerebral hemispheres. The cyst was isointense on T1- and T2-weighted images and hyperintense on diffusion-weighted imaging (DWI), without contrast enhancement. It was composed of an anterior part compressing the septum pellucidum and extending in the intraventricular space; an anteroinferior portion insinuating toward the posterior fossa compressing the brainstem, aqueduct, basal cisterns, and left cerebral peduncle; and a superoposterior portion occupying the semioval centrum, with bilateral mesiotemporal extension to the hippocampus regions. Perilesional edema was not present [Figure 1].
|Figure 1: Preoperative MRI. Axial T1-weighted (a) and T2-weighted (b) MRI showing the low- and high-intensity signal of the lesion which seems to be pineal gland centered with extending from the posterior to the anterior fossa and insinuating into the brainstem, aqueduct, basal cisterns, and left cerebral peduncle. The lesion has no contrast enhancement (c). Diffusion-weighted imaging (d) shows the typical hyperintense and bright tones of the lesion. MRI – Magnetic resonance imaging|
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A median occipital craniotomy was performed in prone position. After bilateral dural opening hinged on sagittal sinus, the cyst was evident on both sides beneath the pia mater, which was easily distinguished as a pearly and avascularized lesion. A small bilateral corticotomy was performed and the cyst was gradually suctioned and microsurgically separated from the surrounding structures of the internal venous system. The capsule appeared tenaciously attached to the neurovascular structures, impossible to be removed. The postoperative course was uneventful, with progressive improvement of the preoperative motor and visual field deficits. Histopathologic diagnosis consisted with epidermoid tumor.
Postoperative MRI revealed subtotal resection with residual tissue deeply located [Figure 2].
|Figure 2: Postoperative MRI. Axial T1-weighted (a), T2-weighted (b), T1-gadolinium-enhanced (c), and DWI (d) images showing the subtotal resection of the lesion. DWI images show the residual pathological tissue around the surgical cavity, mainly in the mesial temporal lobe bilaterally and perimesencephalic cistern. MRI – Magnetic resonance imaging; DWI – Diffusion-weighted imaging|
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Neurological examination at discharge (1 week after surgery) revealed a complete resolution of the hemiparesis but with the persistence of gait ataxia and nystagmus.
At 1-year follow-up, gait ataxia and nystagmus were completely absent and the patient did not complain headache anymore.
MRI appeared superimposable to the postoperative one, showing neither cyst regrowth nor signs of chemical meningitis.
| Discussion|| |
Benign ECs are prevalently diagnosed in males during the 3rd–4th decade, but turn malignant predominantly in females., ECs form from the accumulation of keratin and cholesterol, which desquamate into a pearly material within their walls, growing in the cisternal spaces and remaining asymptomatic for years due to the absence of initial mass effect.,
The usual pattern of EC growth is linear, and symptoms appear when ECs have grown to giant proportions, varying according to their location. They include hearing loss, dizziness, gait disturbance, trigeminal neuralgia, tinnitus, diplopia, visual impairment, apathy, headache, gait ataxia, epilepsy, raised intracranial pressure, and recurrent aseptic meningitis. EC rupture is uncommon and may cause chemical meningitis and hydrocephalus.
On computed tomography scans, benign ECs appear as hypodense or isodense areas, without contrast enhancement, although the presence of protein, lipid, calcium, and hemosiderin can occasionally make them appear hyperdense.
On MRI, ECs show a multilobulated appearance and are hypointense on T1-weighted and hyperintense on T2-weighted images and DWI. Benign ECs usually exhibit no contrast enhancement and peritumoral edema., Fluid-attenuated inversion recovery and DWI sequences represent the main sequences to test for solidity and diagnose ECs. DWI offers a superior view of the EC borders and their characteristic bright tones, which help to distinguish them from arachnoid cysts, dermoids, lipomas, cholesterol granulomas, hamartomatous, cystic neoplasms, neurocysticercosis, and neuroenteric cysts.,, The presence of edema, tissue invasion, rapid growth, and new contrast enhancement suggests malignant transformation.,
Hydrocephalus is not typical, as the ECs' pace of growth is gradual and its crevices occasionally awash with cerebrospinal fluid.
Surgical debulking with capsule removal is a definitive treatment,, although it presents numerous challenges, especially in case of giant multicompartmentalized ECs because of the capsule adherence to many neurovascular structures. The cyst content can be easily suctioned, but its spillage into the subarachnoid space may cause postoperative aseptic meningitis (2%–50% of cases), leading to dense adhesions between the residual capsule and cranial nerves and vessels. A limited resection is sometimes recommended to minimize comorbidities,, but it needs to take into account the higher risks of recurrence (occurring on an average after a symptom-free interval of 7.74 years in 1%–54% of cases), and more rarely, of malignant transformation.,
In consideration of the higher morbidity of recurrent ECs, the latest studies take the view that total resection is associated with improved function and low mortality and should be recommended as the ideal goal of treatment.,
In our view, the case for total or subtotal resection should be evaluated on the basis of the following demographic, clinical, and neuroradiological parameters. First, older patients have a lower risk of recurrence compared to younger ones. Second, in patients with minor symptoms, the primary goal of treatment is not to cause iatrogenic morbidity. Third, resectability depends, in all cases, on preoperative neuroradiological considerations about size, number of compartments, and relationships with neurovascular structures.
| Conclusion|| |
In the management of giant intracranial ECs, a total debulking with capsule removal dramatically reduces the risk of recurrence and malignant transformation. In the surgical setting, however, it is crucial to consider all the features associated with a risk of iatrogenic morbidity, which is best avoided in case of benign lesions.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Moscote-Salazar LR, Satyarthee GD, Calderon-Miranda WG, Agrawal A, Alvis-Miranda HR, Alcala-Cerra G, et al.
Intradiploic pterional epidermoid tumor: A case report and review of literature. J Pediatr Neurosci 2017;12:262-4.
] [Full text]
Olson JJ, Beck DW, Crawford SC, Menezes AH. Comparative evaluation of intracranial epidermoid tumors with computed tomography and magnetic resonance imaging. Neurosurgery 1987;21:1-3.
Aboud E, Abolfotoh M, Pravdenkova S, Gokoglu A, Gokden M, Al-Mefty O. Giant intracranial epidermoids: is total removal feasible? Journal of neurosurgery 2015;122:743-56.
Zhou F, Yang Z, Zhu W, Chen L, Song J, Quan K, et al.
Epidermoid cysts of the cavernous sinus: Clinical features, surgical outcomes, and literature review. J Neurosurg 2017;22:1-11.
Gopalakrishnan CV, Dhakoji A, Nair S. Epidermoid cyst of the brainstem in children: Case-based update. J Child Neurol 2012;27:105-12.
Sirin S, Gonul E, Kahraman S, Timurkaynak E. Imaging of posterior fossa epidermoid tumors. Clin Neurol Neurosurg 2005;107:461-7.
Son DW, Choi CH, Cha SH. Epidermoid tumors in the cerebellopontine angle presenting with trigeminal neuralgia. J Korean Neurosurg Soc 2010;47:271-7.
Recinos PF, Roonprapunt C, Jallo GI. Intrinsic brainstem epidermoid cyst. Case report and review of the literature. J Neurosurg 2006;104:285-9.
Yin H, Zhang D, Wu Z, Zhou W, Xiao J. Surgery and outcomes of six patients with intradural epidermoid cysts in the lumbar spine. World J Surg Oncol 2014;12:50.
Kumar A, Singh P, Jain P, Badole CM. Intramedullary spinal epidermoid cyst of the cervicodorsal region: A rare entity. J Pediatr Neurosci 2010;5:49-51.
] [Full text]
Funao H, Isogai N, Daimon K, Mima Y, Sugiura H, Koyanagi T, et al.
A rare case of intradural and extramedullary epidermoid cyst after repetitive epidural anesthesia: Case report and review of the literature. World J Surg Oncol 2017;15:131.
Gopalakrishnan CV, Ansari KA, Nair S, Menon G. Long term outcome in surgically treated posterior fossa epidermoids. Clin Neurol Neurosurg 2014;117:93-9.
Nakao Y, Nonaka S, Yamamoto T, Oyama K, Esaki T, Tange Y, et al.
Malignant transformation 20 years after partial removal of intracranial epidermoid cyst – Case report. Neurol Med Chir (Tokyo) 2010;50:236-9.
Tancredi A, Fiume D, Gazzeri G. Epidermoid cysts of the fourth ventricle: Very long follow up in 9 cases and review of the literature. Acta Neurochir (Wien) 2003;145:905-10.
Lynch JC, Aversa A, Pereira C, Nogueira J, Gonçalves M, Lopes H, et al.
Surgical strategy for intracranial dermoid and epidermoid tumors: An experience with 33 patients. Surg Neurol Int 2014;5:163.
] [Full text]
Kato K, Ujiie H, Higa T, Hayashi M, Kubo O, Okada Y, et al.
Clinical presentation of intracranial epidermoids: A surgical series of 20 initial and four recurred cases. Asian J Neurosurg 2010;5:32-40. [Full text]
Darrouzet V, Franco-Vidal V, Hilton M, Nguyen DQ, Lacher-Fougere S, Guerin J, et al.
Surgery of cerebellopontine angle epidermoid cysts: Role of the widened retrolabyrinthine approach combined with endoscopy. Otolaryngol Head Neck Surg 2004;131:120-5.
Mohanty A, Venkatrama SK, Rao BR, Chandramouli BA, Jayakumar PN, Das BS, et al.
Experience with cerebellopontine angle epidermoids. Neurosurgery 1997;40:24-30.
Samii M, Tatagiba M, Piquer J, Carvalho GA. Surgical treatment of epidermoid cysts of the cerebellopontine angle. J Neurosurg 1996;84:14-9.
Goel A, Muzumdar D, Desai K. Anterior tentorium-based epidermoid tumours: Results of radical surgical treatment in 96 cases. Br J Neurosurg 2006;20:139-45.
Yasargil MG, Abernathey CD, Sarioglu AC. Microneurosurgical treatment of intracranial dermoid and epidermoid tumors. Neurosurgery 1989;24:561-7.
Safavi-Abbasi S, Di Rocco F, Bambakidis N, et al.
Has Management of Epidermoid Tumors of the Cerebellopontine Angle Improved? A Surgical Synopsis of the Past and Present. Skull Base. 2008;18:85-98.
Chowdhury FH, Haque MR, Sarker MH. Intracranial epidermoid tumor; microneurosurgical management: An experience of 23 cases. Asian J Neurosurg 2013;8:21-8.
] [Full text]
Pikis S, Margolin E. Malignant transformation of a residual cerebellopontine angle epidermoid cyst. J Clin Neurosci 2016;33:59-62.
Braun IF, Naidich TP, Leeds NE, Koslow M, Zimmerman HM, Chase NE, et al.
Dense intracranial epidermoid tumors. Computed tomographic observations. Radiology 1977;122:717-9.
Bergui M, Zhong J, Bradac GB, Sales S. Diffusion-weighted images of intracranial cyst-like lesions. Neuroradiology 2001;43:824-9.
Doll A, Abu Eid M, Kehrli P, Esposito P, Gillis C, Bogorin A, et al.
Aspects of FLAIR sequences, 3D-CISS and diffusion-weight MR imaging of intracranial epidermoid cysts. J Neuroradiol 2000;27:101-6.
Karantanas AH. MR imaging of intracranial epidermoid tumors: Specific diagnosis with turbo-FLAIR pulse sequence. Comput Med Imaging Graph 2001;25:249-55.
Sturiale CL, Mangiola A, Pompucci A, D'Ercole M, Di Muro L, Anile C, et al.
Interdural giant dermoid cyst of the petrous apex. J Clin Neurosci 2009;16:1498-502.
Roh TH, Park YS, Park YG, Kim SH, Chang JH. Intracranial squamous cell carcinoma arising in a cerebellopontine angle epidermoid cyst: A case report and literature review. Medicine (Baltimore) 2017;96:e9423.
Lakhdar F, Hakkou el M, Gana R, Maaqili RM, Bellakhdar F. Malignant transformation six months after removal of intracranial epidermoid cyst: A case report. Case Rep Neurol Med 2011;2011:525289.
Berger MS, Wilson CB. Epidermoid cysts of the posterior fossa. J Neurosurg 1985;62:214-9.
Obana WG, Wilson CB. Epidermoid cysts of the brain stem. Report of three cases. J Neurosurg 1991;74:123-8.
[Figure 1], [Figure 2]