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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 9  |  Issue : 1  |  Page : 51-54

Spinal intramedullary ependymoma presenting with atypical facial pain: A rare occurrence


1 Department of Neurosurgery, LTMMC and LTMG Hospital, Karad, Maharashtra, India
2 Department of General Surgery, Krishna Institute of Medical Sciences University and Research Centre, Karad, Maharashtra, India

Date of Submission08-Oct-2021
Date of Acceptance10-Nov-2021
Date of Web Publication9-Mar-2022

Correspondence Address:
Batuk Diyora
Department of Neurosurgery, Second Floor, College Building, LTMG Hospital, Sion, Mumbai - 400 022, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/joss.joss_9_21

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  Abstract 


Spinal cord ependymomas are the most common neuroepithelial tumors in adults accounting for two-third of all spinal gliomas. Although most patients with spinal cord tumors present with typical symptoms, the presence of a nonspecific clinical presentation with low clinical suspicion results in delayed diagnosis. We report a case of spinal intramedullary ependymoma in a 39-year-old man who presented with atypical pain over the lower half of the right side of the face for 3 months. Magnetic resonance imaging revealed a high cervical intramedullary contrast-enhancing solid mass lesion. The lesion was excised via cervical laminectomy. Histopathological examination confirmed the presence of an ependymoma. Complete resolution of facial pain was achieved postsurgery. At 2 years of follow-up, there was no evidence of clinical or radiological recurrence. Atypical facial pain can be the presenting feature of spinal intramedullary ependymoma. Complete relief of facial symptoms can be achieved by microsurgical excision.

Keywords: Atypical facial pain, ependymoma, intramedullary neoplasm, spinal cord


How to cite this article:
Diyora B, Patel M, Bhende B, Dhall G, Vekariya MA, Mulla M. Spinal intramedullary ependymoma presenting with atypical facial pain: A rare occurrence. J Spinal Surg 2022;9:51-4

How to cite this URL:
Diyora B, Patel M, Bhende B, Dhall G, Vekariya MA, Mulla M. Spinal intramedullary ependymoma presenting with atypical facial pain: A rare occurrence. J Spinal Surg [serial online] 2022 [cited 2022 May 27];9:51-4. Available from: http://www.jossworld.org/text.asp?2022/9/1/51/339279




  Introduction Top


Spinal cord ependymomas are the most common neuroepithelial tumors in adults.[1] Almost two-third of the spinal cord ependymomas are found at the cervical level. Depending on the location, these tumors present with diverse symptomatology ranging from neck pain or back pain to symptoms of weakness and sensory deficits.[1] Unusual presentation includes hyperhidrosis[2] or cervical pruritus[3] trigeminal neuralgia.[4] Spinal intramedullary tumor presenting with atypical facial pain (AFP) has not been reported. We hence describe clinical features, radiology, and surgical outcome in a case of spinal intramedullary ependymoma.


  Case Report Top


A 39-year-old male on medications for a major depressive disorder, presented with pain of mild-moderate intensity over the right side of the face for 3 months. The pain started spontaneously as an abnormal, painful sensation in the lower half of the right side of the face, which was pulling type, intermittent, and progressively increasing in nature. It radiated to the back of the neck, was aggravated by neck movements, and was relieved spontaneously. It was absent in the mornings and progressively increased as the day progressed, thereby lasting for 7–8 h. No history of difficulty breathing or eating. The patient was treated with neuropathic medications by multiple physicians, but in vain, following which he has advised a magnetic resonance imaging (MRI) of the Brain. Due to an abnormality noted in the upper part of the spinal cord during MRI brain study, Spine MRI was asked for. The patient was referred to our neurosurgical unit following an abnormal spinal MRI report. Neurological examination revealed hyperpathia over the right side of the face, mainly over the mandible, neck, and occipital region. There was hypoesthesia on the right side of the face over the V2 and V3 divisions and C3, C4, and C5 dermatomes. Cranial nerve examination revealed no abnormalities. Motor power was Medical Research Council (MRC) grade V/V in all four limbs. No sensory abnormalities were noted in the lower limbs. Superficial reflexes were absent, while deep tendon reflexes were exaggerated bilaterally in the upper and lower limbs. Babinski sign was positive bilaterally. MRI of the spine revealed a contrast-enhancing mass lesion of about 1.5 cm × 0.8 cm × 1.2 cm in size [Figure 1]c and [Figure 1]d in the upper cervical cord consisting of solid and cystic components with the nonenhancing cyst component lying above and below the solid component. The solid component was homogeneously isointense on T1 weighted images [Figure 1]a and hyperintense on T2 weighted images [Figure 1]b. No evidence of any vascular loop around the trigeminal nerve was noted on MRI brain study.
Figure 1: Magnetic resonance imaging of the cervical spine (sagittal and axial view) showing contrast-enhancing mass lesion of about 1.5 cm × 0.8 cm × 1.2 cm size (c and d) in the upper cervical cord consisting of solid and cystic components with the nonenhancing cyst lying above and below the solid component. The solid component appeared homogeneously isointense on T1-weighted images (a) and hyperintense on T2-weighted images (b). Photomicrograph showing flattened ependymal cells with clusters of the oval to spindle cells containing oval to spindle cell nuclei with evidence of pseudorosettes suggestive of ependymoma (e)

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In view of signs of cervical myelopathy, the patient was counseled regarding surgical excision of an intramedullary mass lesion; however, due to lack of patient compliance, the procedure was delayed by 6 months when patient consent was obtained due to the appearance of new motor and sensory symptoms on the right lower limb. Under general anesthesia, in prone position via midline vertical skin incision from the occiput to T1 vertebra level, subcutaneous tissue and fascia were cut opened. Muscles were then dissected, laminae were exposed, and C2 to C4 laminectomy was performed. The dura was opened in the midline under the operating microscope. Standard midline myelotomy was performed, and the cysts were decompressed. The greyish brown, soft to firm, well-defined, minimally vascular solid tumor was excised precisely using microsurgical techniques under continuous spinal cord monitoring. Hemostasis was skillfully achieved during the surgery. At the end of the surgery, the spinal cord became lax and was adequately pulsatile. The dura was then sutured continuously.

The postoperative course was uneventful except for a mild impairment in the dorsal-column sensations over the left side of the body, which also eventually recovered. There was complete relief of facial pain and pain over the nape of the neck. The patient was discharged on the 7th postoperative day. Microscopic examination of the specimen revealed sheets of spindle and epithelioid cells containing round to oval nuclei and small nucleoli [Figure 1]e. Perivascular pseudorosettes were noted around the central blood vessels. The pathological diagnosis was WHO Grade II ependymoma.

At 1-year follow-up, he had no facial pain, but occasional dull aching pain over the lower neck following prolonged work. Follow-up MRI of the cervical spine showed no evidence of residual or recurrent tumor [Figure 2]a, [Figure 2]b, [Figure 2]c, [Figure 2]d.
Figure 2: Postoperative magnetic resonance imaging showing very small residual cystic cavity on sagittal T1-weighted images (a), T2-weighted images (b). Postcontrast administration sagittal and axial images revealed no evidence of residual or recurrent tumor on sagittal (c) and axial images (d)

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  Discussion Top


Spinal cord tumors constitute 15% of all central nervous system tumors. Most patients present with neck pain, back pain, tingling, numbness, weakness, difficulty in walking, imbalance, and occasionally bladder or bowel impairment.[5] Occasionally, they present with atypical features such as hyperhidrosis, cervical itch, and symptoms of trigeminal neuralgia.[1],[2],[3] However, AFP has never been reported with intramedullary ependymomas in adults. Hyperhidrosis is a very rare clinical presentation that results from the overactivity of the sympathetic fibers due to irritation by the tumor.[2] Cervical itch or neuropathic itch due to spinal intramedullary cavernoma reported due to involvement of lamina I in dorsal horn due to gliotic rim containing hemosiderin-laden macrophages.[3] Trigeminal neuralgia is the most common cause of typical neuropathic hemifacial pain. Typical trigeminal neuralgia results from irritation or compression of the nerve. Irritation may be due to an abnormal vascular loop formed around the nerve;[6] compression of the nerve occurs due to mass lesion at a cerebellopontine angle,[7] intrinsic brainstem lesion,[8] or spinal intramedullary tumors.[3] Facial pain that does not follow anatomical pathways of either cranial or peripheral nerves is termed as AFP.[9],[10] This pain is burning, aching, pulling, or throbbing type felt deep in the soft tissue or bone and is often poorly localized and occurs on one side of the face. AFP is always associated with poor objective signs and normal investigation findings.[11] AFP is often considered a psychosomatic disease with a psychogenic origin. Many patients have depression or anxiety disorders. They often do not respond to standard medication and require antidepressant medications. AFP pain is often attributed to muscular activity such as bruxism or demyelination or infection.[11],[12] AFP due to intramedullary tumors has not been reported. It might be the result of the intramedullary tumor compressing the pars caudalis (extending from the obex to C3) part of the sensory spinal trigeminal nucleus at the level of C3. Our patient was under treatment for depressive disorder when he developed new symptoms. In view of his present illness, his new symptoms were not concentrated properly. Due to the progressive nature of new clinical symptoms that were not responding to medication, he was referred for imaging which revealed an intramedullary tumor. Complete relief of AFP following surgical excision of intramedullary tumor reveals that it was responsible for clinical symptoms.

The management of intramedullary tumors varies depending on the individual case scenarios, ranging from conservative to gross total resection.[1] Nevertheless, surgery is the treatment of choice.[13] While subtotal resection may be performed in difficult and complex cases, gross total resection is the best treatment modality for the spinal intramedullary tumor and is associated with the complete resolution of symptoms and a lesser degree of recurrence.[13]

In our case, because of very rare symptoms of AFP it was extremely difficult to justify and convince the patient and his family for excision of the intramedullary tumor. Furthermore, there was a major concern about whether AFP will be relieved following surgery. However, further worsening of clinical symptoms and repeated positive clinical meetings and counseling of the patient and family led to consent for surgery and eventual successful excision of the tumor.


  Conclusion Top


Atypical facial pain can be the presenting feature of spinal intramedullary ependymoma. In cases where atypical facial pain does not respond to the medical management, further imaging is warranted. Our case clearly shows that microsurgical excision of tumor results in complete relief from AFP.

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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Aghakhani N, Messerer M, David P, Herbrecht A, Parker F. Intramedullary ependymomas: A French retrospective multicenter study of 221 cases. Neurochirurgie 2017;63:391-7.  Back to cited text no. 1
    
2.
Haddadi K. A huge ependymoma of the cervical spinal cord with subtle atypical manifestations and hyperhidrosis: Case report. Int J Surg Case Rep 2016;19:100-2.  Back to cited text no. 2
    
3.
Shuhui G, Jiagang L, Siqing H, Haifeng C, Qingrong T, Bohao Z. Rare cervical intramedullary cavernous angioma with trigeminal neuralgia and cervical itch: Case report and review of the literature. Iran Red Crescent Med J 2016;18:e25151.  Back to cited text no. 3
    
4.
Saito N, Yamakawa K, Sasaki T, Saito I, Takakura K. Intramedullary cavernous angioma with trigeminal neuralgia: A case report and review of the literature. Neurosurgery 1989;25:97-101.  Back to cited text no. 4
    
5.
Hoang S, Mesfin FB. Cancer, spinal cord, intramedullary. In: Stat Pearls. Treasure Island (FL): Stat Pearls Publishing; 2018-2017.  Back to cited text no. 5
    
6.
Love S, Coakham HB. Trigeminal neuralgia: Pathology and pathogenesis. Brain 2001;124:2347-60.  Back to cited text no. 6
    
7.
Shulev Y, Trashin A, Gordienko K. Secondary trigeminal neuralgia in cerebellopontine angle tumors. Skull Base 2011;21:287-94.  Back to cited text no. 7
    
8.
Stellmann JP, Kuhn M, Töpper R. Chronic facial pain due to a brainstem cavernoma. Fortschr Neurol Psychiatr 2007;75:552-4.  Back to cited text no. 8
    
9.
Pascual J, Colás R, Castillo J. Epidemiology of chronic daily headache. Curr Pain Headache Rep 2001;5:529-36.  Back to cited text no. 9
    
10.
Zakrzewska JM. Multi-dimensionality of chronic pain of the oral cavity and face. J Headache Pain 2013;14:37.  Back to cited text no. 10
    
11.
Feinmann C, Harris M, Cawley R. Psychogenic facial pain: Presentation and treatment. Br Med J (Clin Res Ed) 1984;288:436-8.  Back to cited text no. 11
    
12.
Harris M. Psychogenic facial pain. Int J Oral Surg 1981;10 Suppl 1:183-6.  Back to cited text no. 12
    
13.
Svoboda N, Bradac O, de Lacy P, Benes V. Intramedullary ependymoma: Long-term outcome after surgery. Acta Neurochir (Wien) 2018;160:439-47.  Back to cited text no. 13
    


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