Skip to main content

Advertisement

SpringerLink
Log in

Cerebellopontine angle epidermoid cysts: clinical presentations and surgical outcome

  • Original Article
  • Published:
Neurosurgical Review Aims and scope Submit manuscript

Abstract

Epidermoid cysts constitute less than 1 % of intracranial tumors with the majority of them involving cerebellopontine angle (CPA). Although several mechanisms for cranial nerve dysfunction due to these tumors have been proposed, no direct evaluation for hyper- or hypoactive dysfunction has been done. In this case series, pathophysiology of cranial nerve dysfunction in CPA epidermoid cysts was evaluated with special attention to a new mechanism of capsule strangulation caused by stratified tumor capsule. Twenty-two cases with epidermoid cysts of CPA micro-neurosurgically treated in our departments since 2005 were reviewed. Clinical status of the patients before the surgery and post-operative functional outcome were recorded. Available data from the English literature were summarized for comparison. Mass reduction of cyst contents in most cases was usually associated with prompt and marked improvement of the symptoms suggesting neuroapraxia caused by compression of the tumor content and/or mild ischemia. Among them, two cases showed strangulation of the affected nerves by the tumor capsule whose preoperative dysfunction did not improve after surgery in spite of meticulous microsurgical removal of the lesion. Involved facial and abducent nerves in these two cases showed distortion of nerve axis and nerve atrophy distal to the strangulation site. We report the first direct evidence of etiology of cranial nerve dysfunction caused by cerebellopontine angle epidermoid tumors. Young age and rapidly progressive neurological deficit might be the characteristics for strangulation of the affected nerves by the cyst capsule. Even though the number of cases might be limited, immediate decompression and release of the strangulating band might be urged in such patients to prevent irreversible deficits.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Abramson RC, Morawetz RB, Schlitt M (1989) Multiple complications from an intracranial epidermoid cyst: case report and literature review. Neurosurgery 24:574–578

    Article  CAS  PubMed  Google Scholar 

  2. Altschuler EM, Jungreis CA, Sekhar LN, Jannetta PJ, Sheptak PE (1990) Operative treatment of intracranial epidermoid cysts and cholesterol granulomas: report of 21 cases. Neurosurgery 26:606–614

    Article  CAS  PubMed  Google Scholar 

  3. Alvord EC Jr (1977) Growth rates of epidermoid tumors. Ann Neurol 2:367–370

    Article  PubMed  Google Scholar 

  4. Antoli-Candela F Jr, Stewart TJ (1974) The pathophysiology of otologic facial paralysis. Otolaryngol Clin N Am 7:309–330

    Google Scholar 

  5. Apfelbaum MI (1987) Epidermoid cysts and cholesterol granulomas centered on the posterior fossa: twenty years of diagnosis and management. Neurosurgery 21:805

    Google Scholar 

  6. Bauman CH, Bucy PC (1956) Paratrigeminal epidermoid tumors. J Neurosurg 13:455–468

    Article  Google Scholar 

  7. Berger MS, Wilson CB (1985) Epidermoid cysts of the posterior fossa. J Neurosurg 62:214–219

    Article  CAS  PubMed  Google Scholar 

  8. Committee of Brain Tumor Registry of Japan (2014) Report of brain tumor resistor of Japan (2001–2004). Neurol Med Chir 54(Suppl):9–102

    Google Scholar 

  9. De Souza CE, de Souza R, da Costa S et al (1989) Cerebellopontine angle epidermoid cysts: a report on 30 cases. J Neurol Neurosurg Psychiatry 52:986–990

    Article  Google Scholar 

  10. Gagliardi FM, Vagnozzi R, Caruso R, Delfini R (1980) Epidermoids of the cerebellopontine angle (CPA): usefulness of CT scan. Acta Neurochir (Wien) 54:271–281

    Article  CAS  Google Scholar 

  11. Gopalakrishnan CV, Ansari KA, Nair S, Menon G (2014) Long term outcome in surgically treated posterior fossa epidermoids. Clin Neurol Neurosurg 117:93–99

    Article  PubMed  Google Scholar 

  12. Grant FC, Austin GM (1950) Epidermoids; clinical evaluation and surgical results. J Neurosurg 7(3):190–198

    Article  CAS  PubMed  Google Scholar 

  13. Guidetti B, Gagliardi FM (1977) Epidermoid and dermoid cysts: clinical evaluation and late surgical results. J Neurosurg 47:12–18

    Article  CAS  PubMed  Google Scholar 

  14. Hamel E, Frowein RA, Karimi-Nejad A (1980) Intracranial intradural epidermoids and dermoids. Neurosurg Rev 3:215–219

    Article  CAS  PubMed  Google Scholar 

  15. Hori T, Numata H, Hokama Y, Muraoka K, Tamaki M, Saito Y (1983) Trigeminal pain caused by a parapontine epidermoid cyst. Surg Neurol 19:517–519

    Article  CAS  PubMed  Google Scholar 

  16. Karantanas AH (2001) MR imaging of intracranial epidermoid tumors: specific diagnosis with Turbo-FLAIR pulse sequence. Comput Med Imaging Graph 25:249–255

    Article  CAS  PubMed  Google Scholar 

  17. Kobata H, Kondo A, Iwasaki K, Nishioka T (1998) Combined hyperactive dysfunction syndrome of the cranial nerves: trigeminal neuralgia, hemifacial spasm and glossopharyngeal neuralgia—11-year experience and review. Neurosurgery 43:1351–1362

    CAS  PubMed  Google Scholar 

  18. Kobata H, Kondo A, Iwasaki K (2002) Cerebellopontine angle epidermoids presenting with cranial nerve hyperactive dysfunction: pathogenesis and long-term surgical results in 30 patients. Neurosurgery 50:276–285

    PubMed  Google Scholar 

  19. Lee SH, Rhee BA, Choi SK, Koh JS, Lim YJ (2010) Cerebellopontine angle tumors causing hemifacial spasm: types, incidence, and mechanism in nine reported cases and literature review. Acta Neurochir (Wien) 152(11):1901–1908

    Article  Google Scholar 

  20. Liu P, Saida Y, Yoshioka H, Itai Y (2003) MR imaging of epidermoids at the cerebellopontine angle. Magn Reson Med Sci 2:109–115

    Article  PubMed  Google Scholar 

  21. Mallucci CL, Ward V, Carney AS, O’Donoghue GM, Robertson I (1999) Clinical features and outcomes in patients with non-acoustic cerebellopontine angle tumours. J Neurol Neurosurg Psychiatry 66(6):768–771

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  22. Mattsson P, Meijer B, Svensson M (1999) Extensive neuronal cell death following intracranial transection of the facial nerve in the adult rat. Brain Res Bull 49:333–341

    Article  CAS  PubMed  Google Scholar 

  23. Mohanty A, Venkatrama SK, Rao BR, Chandramouli BA, Jayakumar PN, Das BS (1997) Experience with cerebellopontine angle epidermoids. Neurosurgery 40(1):24–29

    CAS  PubMed  Google Scholar 

  24. Netsky MG (1988) Epidermoid tumors. Review of literature. Surg Neurol 29:477–483

    Article  CAS  PubMed  Google Scholar 

  25. Obrador S, Lopez-Zafra JJ (1969) Clinical features of the epidermoids of the basal cisterns of the brain. J Neurol Neurosurg Psychiatry 32:450–454

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  26. Olivecrona H (1932) On suprasellar cholesteatomas. Brain 55:122–134

    Article  Google Scholar 

  27. Peng Y, Yu L, Li Y, Fan J, Qiu M, Qi S (2014) Pure endoscopic removal of epidermoid tumors of the cerebellopontine angle. Childs Nerv Syst 30(7):1261–1267

    Article  PubMed  Google Scholar 

  28. Rengachary A, Kishore PRS, Watanabe I (1978) Intradiploic epidermoid cyst of the occipital bone with torcular obstruction. J Neurosurg 48(3):475–478

    Article  CAS  PubMed  Google Scholar 

  29. Rubin G, Scienza R, Pasqualin A, Rosta L, Da Pian R (1989) Craniocerebral epidermoids and dermoids. A review of 44 cases. Acta Neurochir (Wien) 97:1–16

    Article  CAS  Google Scholar 

  30. Sabin HI, Bordi LT, Symon L (1987) Epidermoid cysts and cholesterol granulomas centered on the posterior fossa: twenty years of diagnosis and management. Neurosurgery 21:798–805

    Article  CAS  PubMed  Google Scholar 

  31. Safain MG, Dent WC, Heilman CB (2014) An endoscopic assisted retrosigmoid approach to the cerebello-pontine angle for resection of an epidermoid cyst. Neurosurg Focus 36(1 Suppl):1. doi:10.3171/2014

    Article  PubMed  Google Scholar 

  32. Safavi-Abbasi S, Di Rocco F, Bambakidis N et al (2008) Has management of epidermoid tumors of the cerebellopontine angle improved? A surgical synopsis of the past and present. Skull Base 18(2):85–98

    Article  PubMed  PubMed Central  Google Scholar 

  33. Salazar J, Vaquero J, Saucedo G, Bravo G (1987) Posterior fossa epidermoid cysts. Acta Neurochir 85:34–39

    Article  CAS  PubMed  Google Scholar 

  34. Samii M, Draf W (eds) (1989) Surgery of the skull base. An interdisciplinary approach. Springer-Verlag, Berlin, pp 340–342

    Google Scholar 

  35. Samii M, Tatagiba M, Piquer J, Carvalho GA (1996) Surgical treatment of epidermoid cysts of the cerebellopontine angle. J Neurosurg 84(1):14–19

    Article  CAS  PubMed  Google Scholar 

  36. Schiefer TK, Link MJ (2008) Epidermoids of the cerebellopontine angle: a 20-year experience. Surg Neurol 70(6):584–590

    Article  PubMed  Google Scholar 

  37. Schroeder HW, Oertel J, Gaab MR (2004) Endoscope-assisted microsurgical resection of epidermoid tumors of the cerebellopontine angle. J Neurosurg 101(2):227–232

    Article  PubMed  Google Scholar 

  38. Shimamoto Y, Kawase T, Sasaki H, Shiobara R, Yamada F (1999) Anterior transpetrosal approach to the prepontine epidermoids. Skull Base Surg 9(2):75–80

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  39. Shulev Y, Trashin A, Gordienko K (2011) Secondary trigeminal neuralgia in cerebellopontine angle tumors. Skull Base 21(5):287–294

    Article  PubMed  PubMed Central  Google Scholar 

  40. Talacchi A, Sala F, Alessandrini F, Turazzi S, Bricolo A (1998) Assessment and surgical management of posterior fossa epidermoid tumors: report of 28 cases. Neurosurgery 42(2):242–251

    Article  CAS  PubMed  Google Scholar 

  41. Tsuruda JS, Chew WM, Moseley ME, Norman D (1990) Diffusion-weighted MR imaging of the brain: value of differentiating between extraaxial cysts and epidermoid tumors. AJR Am J Roentgenol 155:1059–1065

    Article  CAS  PubMed  Google Scholar 

  42. Ulrich J (1964) Intracranial epidermoids. A study on their distribution and spread. J Neurosurg 21:1051–1058

    Article  CAS  PubMed  Google Scholar 

  43. Vinchon M, Pertuzon B, Lejeune JP, Assaker R, Pruvo JP, Christiaens JL (1995) Intradural epidermoid cysts of the cerebellopontine angle: diagnosis and surgery. Neurosurgery 36(1):52–56

    Article  CAS  PubMed  Google Scholar 

  44. Wakabayashi T, Tamaki N, Satoh H, Matsumoto S (1983) Epidermoid tumor presenting as painful tic convulsif. Surg Neurol 19(3):244–246

    Article  CAS  PubMed  Google Scholar 

  45. Yamakawa K, Shitara N, Genka S, Manaka S, Takakura K (1989) Clinical course and surgical prognosis of 33 cases of intracranial epidermoid tumors. Neurosurgery 24:568–573

    Article  CAS  PubMed  Google Scholar 

  46. Yasargil MG, Abernathey CD, Sarioglu AÇ (1989) Microsurgical treatment of intracranial dermoid and epidermoid tumors. Neurosurgery 24:561–567

    Article  CAS  PubMed  Google Scholar 

Download references

Acknowledgments

The authors confirm that no governmental, institutional, or industrial fund or grant was given for the material of this study or to support any of the authors.

Author information

Authors and Affiliations

  1. Department of Neurosurgery, Fujita Health University Hospital, 1-98, Dengakugakubo, Kutsukake, Toyoake, Aichi, 470-1192, Japan

    Mitsuhiro Hasegawa, Mohsen Nouri, Shinya Nagahisa, Koichiro Yoshida, Kazuhide Adachi, Joji Inamasu & Yuichi Hirose

  2. Department of Neurosurgery, Razi Hospital, Zahedan University of Medical Sciences, Saravan, Sistan, Balouchestan, Iran

    Mohsen Nouri

  3. Department of Neurosurgery, Fukui Prefectural Hospital, Fukui, 910-8526, Japan

    Hironori Fujisawa

Authors
  1. Mitsuhiro Hasegawa
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Mohsen Nouri
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Shinya Nagahisa
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Koichiro Yoshida
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Kazuhide Adachi
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Joji Inamasu
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Yuichi Hirose
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Hironori Fujisawa
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Mitsuhiro Hasegawa.

Additional information

Comments

Kazunari Yoshida, Tokyo, Japan

The symptoms of the brain tumor usually occur by compression or destruction of the neural tissue by the tumor mass. Epidermoid cysts in the cerebellopontine angle (CPA) are known to present variable dysfunction of the cranial nerves, including hemifacial spasm, trigeminal neuralgia, and variable cranial nerve palsy. The distinct finding, described in this manuscript, is that the mechanism of the cranial nerve dysfunction in some cases is the nerve degeneration of the distal to the strangulation site by the tumor capsule. Nobody has recognized this type of the mechanism of the cranial nerve dysfunction so far. Dr. Hasegawa and his colleagues have clearly demonstrated this mechanism of the cranial nerve dysfunction in cases of the CPA epidermoid cysts. We should notice this mechanism of the cranial nerve dysfunction and decide the appropriate timing of the management of these tumors.

Henry W. S. Schroeder, Greifswald, Germany

The authors report their experience with the microsurgical treatment of 22 CPA epidermoids. The discussion of the mechanisms of the cranial nerve deficits is interesting. In two of their patients, the authors found a strangulation of the facial and abducent nerve respectively. Although the neurological deficits improved in most of the patients, in the two patients with strangulation of the nerve, there was no satisfying improvement. The authors conclude that in the case of rapidly developing cranial nerve palsy, early surgical intervention is required to increase the chance of recovery of the cranial nerve function. This is an interesting finding that has not frequently been reported in the literature. In our series, the cranial nerve dysfunction was caused by nerve compression and distortion, but we never observed such a strangulation as described by the authors. Early nerve decompression seems to be very reasonable in case of a rapid neurological deficit.

I agree that gross total removal of the epidermoid including the cyst capsule should be the aim of the surgery for epidermoids. I always try a very aggressive resection of the cyst capsule. However, in most patients, I had to leave a small portion of the capsule behind because it was very adherent to the nerves, vessels, or brain stem. Mostly, in virgin cases, that was only a very tiny part (a few square millimeters), sometimes even at only one location in the surgical field. This is referred to as near total resection.

The authors stated that in case of contralateral or supratentorial tumor extension, the tumor would be resected through the ipsilateral corridor as much as possible and the contralateral remnant would be followed for any symptomatic or radiological growth. In epidermoid resections, I almost always use endoscope assistance to increase the rate of total removal of the cyst contents. With 30° or 45° endoscopes, one may look around the tentorium or the brain stem to the contralateral side and remove the residual tumor under endoscopic visualization in a safe way. Additionally, I found the endoscope very helpful to remove extensions of the epidermoid located in Meckel’s cave.

Thomas Kretschmer, Oldenburg, Germany

The paper’s emphasis is on a considerable case series of CP-angle epidermoids with evaluation of functional outcome and correlation to intraoperative findings. This is compared to the pertinent literature. As such, the reader finds condensed information on clinical findings, functional deficits and expectable outcomes from microsurgery. An additional mechanism for cranial nerve deficit other than neurapraxia is identified and introduced: namely “strangulation” of CN. The proposed mechanism is detailed by two cases. As the functional results in these cases were that CNs were unfavorable and distinct from other cases, the authors drew the following conclusion: A rapidly progressive CN deficit in a young patient with CP-angle epidermoid is suggestive of a strangulation mechanism. In such cases early surgery is recommended.

This is an interesting observation in a large case series, which was nicely worked-up for outcome and compared to existing series. The paper adds to our knowledge about cp-angle epidermoid, congratulation.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Hasegawa, M., Nouri, M., Nagahisa, S. et al. Cerebellopontine angle epidermoid cysts: clinical presentations and surgical outcome. Neurosurg Rev 39, 259–267 (2016). https://doi.org/10.1007/s10143-015-0684-5

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10143-015-0684-5

Keywords

Search

Navigation