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Page 2 of 5 Rahman et al. Neuroimmunol Neuroinflammation 2018;5:14 I http://dx.doi.org/10.20517/2347-8659.2017.57
5 tumors that they classified as being intramedullary. The incidence of intraspinal epidermoid cysts in
[1]
children is 3% and in adults is 1% . True intramedullary epidermoid cysts are rare, with < 60 cases having
been reported in the literature since the 1st reporting of the entity by Chiari in 1833. Of these, a very few
have detailed radiographic evaluation. Intramedullary epidermoid cysts are common in the dorsal and
lumbosacral region. Regions with two frequent localizations are T4-T6 and T11-T12, while only three cases
[5-7]
have been reported with cervical cord involvement . However, our case showed that there is epidermoid
in the dorsal 2/3 level, which is very uncommon site and no association with dermal sinus.
CASE REPORT
A 21-year-old male was suffering from weakness of both lower limbs for 6 months, difficulty in walking
for same duration, diminished sensation below mid chest for same duration along with constipation for
5 months and bladder disturbance for 1 month with erectile dysfunction. Weakness of both lower limbs
which was insidious in onset, gradually progressive in nature, more on left side in comparison to right
side of body below mid chest. His weakness was so severe nowadays that he encountered difficulties in
walking and it was difficult to lift his body from kneeling position. Sometimes shoes run out of his feet.
He complained about the numbness in his mid chest and diminished sensation of all modalities below
mid chest and also complained of occasional incontinence of urine for last 1 month. He also complained
of pain in back of mid chest for 3 months which was dull aching, mild in severity. There was no history of
rest pain or night pain, and no definite aggravating or relieving factor. He didn’t give any history of trauma
to back or any part of body, and there was no scar mark over back fever, cough or hemoptysis. Clinical
examination showed upper limb normal and muscle power of lower limbs Medical Research Council grade
3, all jerks exaggerated and planter extensor with ankle and pateller clonus were present. Sensory level
dorsal 4 and coordinations and gate could not be assessed. Regarding spine examinations revealed normal.
Magnetic resonance imaging (MRI) of dorsal spine showed intramedullary cyst like space occupying
lesions at the level of D2/3 which was contrast uptake only margin of the lesions [Figure 1]. MR myelogram
revealed that it completely cut off the sign of cerebrospinal fluid (CSF) flow [Figure 1]. Operation is
performed by laminectomy and complete removal of tumor. Intraoperative tumor exhibited a typical
pearly white appearance, such as other epidermis-like tissues, which were removed by piece meal approach.
Only the capsule that is firmly attached to the capsule can be removed. After proper homeostasis dura
closed and skin also closed in layers. Postoperatively, muscle power was decrease and subsequent follow-up
gradually improved along with autonomic functions. Now he can walk without support but better with aid.
Confirmation of histopathology as epidermoid carcinoma from two deferent centers [Figure 2].
DISCUSSION
Epidermoid cysts (sebaceous cysts) are benign congenital lesions of ectodermal origin. Intracerebral
epidermoid cysts are rare and may account for approximately 1.5% of all intracranial epidermoids and
approximately 1% of all intra-cranial tumors. Epidermoids usually present around 20 to 40 years of age
and occur in both men and women with the same frequency. They can be congenital or acquired. They can
[8]
be both intradural and extradural . Epidermoids are in good condition, smooth, lobulated, cystic lesions.
Histologically, their inner layer is composed of stratified squamous epithelium with a layer of capsule.
They tend to slowly enlarge as epithelial cells desquamate, foming keratin and cholesterol crystals in the
[9]
center of the lesion. Handa et al. analyzed the aspirates of epidermal inclusion body of the epidermis
to determine the cytological characteristics. Epidermoid cysts often occur in older age groups and show
slower natural progression than most craniopharyngiomas. The epidermoid cyst wall consists of multiple
layers of keratinized squamous epithelium which is located in the outer layer of collagen. The contents are
more likely to be solid, keratinous, rather than the typical craniopharyngiomas oily fluid. The center of
larger cysts sometimes degenerates, the keratin flakes are replaced with greasy brownish fluid that contain
[10]
cholesterol crystals . However, the reported case was a 21-year-old boy, which may be a congenital cause
of no history of trauma or previous surgery. Pathogenesis of spinal epidermoid is either congenital or acquired.