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Year : 2020  |  Volume : 16  |  Issue : 6  |  Page : 1538-1540

Recurrent intramedullary epidermoid cyst of spinal cord: A rare entity

1 Department of Neurosurgery, Command Hospital (Air Force), Bengaluru, Karnataka, India
2 Department of Neurosurgery, Army Hospital (Research and Referral), New Delhi, India
3 Department of Neurosurgery, Command Hospital (Central Command), Lucknow, Uttar Pradesh, India

Date of Submission29-Jun-2017
Date of Decision02-Oct-2017
Date of Acceptance07-Apr-2018
Date of Web Publication30-Oct-2018

Correspondence Address:
Sanjay Kumar
Department of Neurosurgery, Command Hospital (Central Command), Cantonment, Lucknow - 226 002, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcrt.JCRT_544_17

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 > Abstract 

Epidermoid cysts of the spinal cord without spinal dysraphisms are rare lesions and intramedullary cysts are still rarer. Approximately only over 60 cases are reported in literature worldwide. Magnetic resonance imaging is the imaging modality of choice for the diagnosis of these lesions. Management of these lesions is by total surgical excision, although at times, it is not possible due to adherence of the capsule to the parenchyma of the spinal cord which may be the cause of recurrence. To our knowledge, this is the second case of recurrent intramedullary epidermoid cyst of spinal cord. We report a case of intramedullary epidermoid cyst which recurred after 7 years following near-total excision of tumor at our institute.

Keywords: Dorsal, epidermoid cyst, intramedullary, magnetic resonance imaging, recurrent

How to cite this article:
Yadav KK, Verma SK, Kumar S. Recurrent intramedullary epidermoid cyst of spinal cord: A rare entity. J Can Res Ther 2020;16:1538-40

How to cite this URL:
Yadav KK, Verma SK, Kumar S. Recurrent intramedullary epidermoid cyst of spinal cord: A rare entity. J Can Res Ther [serial online] 2020 [cited 2021 Dec 4];16:1538-40. Available from: https://www.cancerjournal.net/text.asp?2020/16/6/1538/244467

 > Introduction Top

Epidermoid cysts are generally congenital and originate from the inclusion of anomalous ectoderm during early fetal life.[1],[2] The incidence of intraspinal epidermoid cysts is <1% and they usually present in intradural extramedullary location with frequent association of spinal dysraphisms. Intramedullary epidermoid cysts are rare[3] tumors and only over 60 cases have been reported in literature.[4] The treatment of choice is surgical excision of these lesions. Recurrence of epidermoid cysts is known; however, only one case of recurrent intramedullary epidermoid cyst has been reported in literature.[5] We describe the second case of recurrent intramedullary epidermoid cyst of dorsal spine which recurred after 7 years of the first surgery.

 > Case Report Top

A 29-year-old male who had undergone resection of intramedullary epidermoid cyst DV-DV4 in 2006 presented with progressive weakness, tightness, and diminished sensation of both lower limbs of 2-month duration. He also had increased frequency, urgency, and precipitancy of micturition of 15 days' duration at the time of presentation. Neurological examination revealed spastic paraparesis (power Medical Research Council 3/5), exaggerated knee and ankle jerks bilaterally with well-sustained ankle clonus, Babinski's sign, and diminished sensation to all modalities below DV5. A well-healed midline scar over the upper dorsal spine was present.

Magnetic resonance imaging (MRI) showed 3.5 cm × 1.5 cm × 1.0 cm, well-defined intramedullary lesion which was hypointense on T1, hyperintense on T2, and showed minimal enhancement of the capsule as seen on postcontrast images with no enhancement of the tumor tissue. Expansion of the cord was also noted at the level of the lesion, i.e., DV3-DV4 [Figure 1]a, [Figure 1]b and [Figure 2]a, [Figure 2]b. Evidence of previous surgery was also noted in the posterior elements of the vertebra at DV2-DV5. A diagnosis of recurrence of the epidermoid cyst was entertained.
Figure 1: (a) T2 axial image depicting hyperintense intramedullary lesion occupying almost the complete spinal canal with thinned out cord. (b) T1 postcontrast image depicting minimal enhancement of capsule in the left lateral region

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Figure 2: (a) T2 sagittal image depicting hyperintense intramedullary lesion occupying almost the complete spinal canal with thinned out cord. (b) T1 postcontrast image depicting minimal enhancement of the capsule of the lesion

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The patient underwent reexploration through the previous incision and durotomy was performed under a microscope. The spinal cord was noticed to be expanded at the level of DV3-DV-4, with area of gliosis noted at the site of previous myelotomy. A midline myelotomy was performed at the site of previous incision. A pearly white and flaky lesion was found with a well-defined capsule, which was firmly adherent to the cord at places. Near-total excision of the lesion was performed, leaving the adherent capsule to the cord parenchyma at places. Postoperatively, the patient had an uneventful recovery and, at 24-month follow-up, the patient is ambulant without support, continent, and has some residual spasticity. Histopathological examination of the lesion confirmed the diagnosis of an intramedullary epidermoid cyst.

 > Discussion Top

Epidermoids are mainly congenital in origin. They originate from inclusion of ectoderm tissue during the closure of neural tube in fetal life.[1],[2] They are generally associated with various entities of spinal dysraphism such as dermal sinus, syrinx, and vertebral anomalies. The incidence of spinal epidermoid cyst is 0.7%.[3] Intramedullary location is even rarer. Approximately only over sixty cases are reported in literature. The most common location is the dorsal spine followed by the lumbar region. MRI is the investigation of choice.[6] These cysts are characteristically hypointense on T1 and hyperintense on T2, with no or minimal enhancement of the capsule (as seen in our case). They typically cause expansion of the cord when seen in intramedullary location [Figure 2]a and [Figure 2]b.

The treatment of choice is total surgical excision of the cyst and the capsule. However, it may be impossible to completely excise the capsule because it may be adherent to the spinal cord tissue, and an attempt to completely excise the capsule may lead to unwarranted worsening of the neurological tissue.[7] Although literature supports the fact that a risk of recurrence exists,[7] there are very few reports of recurrent intramedullary epidermoid cyst. A review of literature revealed the first case of recurrent epidermoid cyst by Fleming et al.[5] To our knowledge, the present case is the second case of recurrent intramedullary epidermoid cyst of the spinal cord. We managed this case by redo surgery and excision of the lesion along with removal of the capsule. At 24 months of follow-up, the patient had no evidence of recurrence of the cyst and neurologically the patient remained intact with no further deterioration in his condition.

MRI remains the modality of choice for preoperative diagnosis[6] of the epidermoid cyst. Treatment of choice remains surgical excision, and recurrent intramedullary epidermoid cyst of the spinal cord can occur if capsule of the cyst is not excised during the initial surgery. Re-excision of the lesion is safe with improvement of the patient's neurological condition as demonstrated in our patient. Radiotherapy also has been advocated for recurrent intraspinal epidermoid cysts[8] although no long-term studies are available at present, with a statistically significant number of cases to recommend this modality as an alternative to redo surgery for recurrent intramedullary epidermoid cysts of spinal cord. Gamma knife surgery (GKS) has been advocated for intracranial epidermoid cysts (mainly for cerebellopontine angle epidermoids);[9] however, extrapolating the same therapy for recurrent intramedullary spinal cord epidermoids may be difficult due to the technical challenges involved in administering the dose to spinal cord. GKS may be advocated in small residual or recurrent epidermoid cysts of spinal cord with minimal neurological deficits, but patients presenting with significant deficits may still require surgical decompression to alleviate the symptoms as these are slow-growing tumors and the therapeutic effect of GKS is not evident early in the course of treatment.

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.

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Conflicts of interest

There are no conflicts of interest.

 > References Top

Manno NJ, Uihlein A, Kernohan JW. Intraspinal epidermoids. J Neurosurg 1962;19:754-65.  Back to cited text no. 1
Roux A, Mercier C, Larbrisseau A, Dube LJ, Dupuis C, Del Carpio R, et al. Intramedullary epidermoid cysts of the spinal cord. Case report. J Neurosurg 1992;76:528-33.  Back to cited text no. 2
Guidetti B, Gagliardi FM. Epidermoid and dermoid cysts. Clinical evaluation and late surgical results. J Neurosurg 1977;47:12-8.  Back to cited text no. 3
Lee JH, Shin JJ, Hwang YS, Kim TH, Shin HS, Park SK. Intramedullary epidermoid cyst in thoracolumbar junction: A case report. Kor J Spine 2008;5:219-24.  Back to cited text no. 4
Fleming C, Kaliaperumal C, O'Sullivan M. Recurrent intramedullary epidermoid cyst of conus medullaris. BMJ Case Rep 2011;2011. pii: bcr1120115090.  Back to cited text no. 5
Kukreja K, Manzano G, Ragheb J, Medina LS. Differentiation between pediatric spinal arachnoid and epidermoid-dermoid cysts: Is diffusion-weighted MRI useful? Pediatr Radiol 2007;37:556-60.  Back to cited text no. 6
Chandra PS, Manjari T, Devi BI, Chandramouli BA, Srikanth SG, Shankar SK, et al. Intramedullary spinal epidermoid cyst. Neurol India 2000;48:75-7.  Back to cited text no. 7
[PUBMED]  [Full text]  
Bretz A, Van den Berge D, Storme G. Intraspinal epidermoid cyst successfully treated with radiotherapy: Case report. Neurosurgery 2003;53:1429-31.  Back to cited text no. 8
Vasquez JA, Fonnegra JR, Diez JC, Fonnegra A. Treatment of epidermoid tumors with gamma knife radiosurgery: Case series. Surg Neurol Int 2016;7:S116-20.  Back to cited text no. 9


  [Figure 1], [Figure 2]


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