05 April 2023: Articles
Sporadic Spinal Lumber Epidermoid Cyst in an Obese Adolescent: A Radiological and Pathological Review
Unusual clinical course, Challenging differential diagnosis, Unusual or unexpected effect of treatment, Educational Purpose (only if useful for a systematic review or synthesis)Abdulmohsen M. Alkhalaf 1ABEF*, Khaled W. Altowayan1AEF, Abdullah H. AlHindi2AEF, Ahmed Alhumidi3BEF, Yahya M. Al Aseri4AEF
Am J Case Rep 2023; 24:e938811
BACKGROUND: We report a case of a sporadic lumbar epidermoid cyst in a patient with no apparent risk factors for the condition. The lesion is considered an uncommon lesion that has a potentially debilitating effect on the spinal cord.
CASE REPORT: Our patient was a 17-year-old boy who presented to the neurosurgery clinic with lower back pain, accompanied by an electrical sensation radiating bilaterally to the buttocks, thighs, and knees. He has been increasingly reliant on a walking cane over the past few months. The patient was considered obese, with a BMI of 44. Otherwise, his physical examination was unremarkable, with no signs of dysraphism. He underwent magnetic resonance imaging (MRI) of the spine, which revealed a lumbar spine lesion, compressing the adjacent cauda equina nerve roots. MRI imaging showed the lesion was an intradural extramedullary mass showing hypointense signal on T1- and hyperintense on T2-weighted images, with diffusion restriction on diffusion-weighted imaging (DWI). The imaging findings were consistent with an epidermoid cyst.
CONCLUSIONS: Epidermoid cysts are benign lesions, usually found in the head and trunk. When found in the spine, they can cause a range of symptoms that can be debilitating. Patients presenting with signs and symptoms of spinal cord compression should be promptly investigated. MRI is an excellent tool for characterizing an epidermoid cyst. The lesion appears oval and hypointense on T1-weighted imaging, and characteristically shows diffusion restriction on diffusion-weighted imaging (DWI). With surgical treatment, the outcome is usually favorable.
Keywords: Neuroradiography, Neurosurgery, Adolescent
Primary tumors of the central nervous system (CNS) are predominantly located in the cranium. Spinal tumors account for only 20% of CNS tumors, and they tend to be benign, unlike cranial tumors. Extradural tumors are by far the most common, followed by intradural extramedullary tumors, and lastly by intramedullary tumors .
Epidermoid cysts are cysts lined by stratified squamous epithelium. They grow linearly by desquamation. They contain keratin, cholesterol, and cellular debris. They are void of hair follicles and sebaceous glands, unlike dermoid cysts . They are thought to arise from retained or implanted cells from the cutaneous ectoderm into the spinal canal, either from failed separation during embryogenesis, or from trauma, surgery, or procedures such as lumbar puncture [2–4].
The most common sites are the face and trunk. When found in the CNS, they are more likely to be in the cerebellopontine angle. Spinal epidermoid cysts are quite rare but can cause debilitating compression symptoms .
We present the case of a 17-year-old boy who presented to the clinic with lower back pain that began 2 months ago. The pain was aggravated by coughing and sneezing. He felt an electrical sensation radiating from the buttocks to the posterior aspect of his thighs and knees. It started on the left then became bilateral. He did not have urine retention or fecal incontinence. He occasionally used a cane for walking. He had no history of trauma, surgery, or procedures.
On physical examination, his vital signs were stable. He was able to sit comfortably on the examination table, and was oriented to place, time, and person. He weighed 138 Kg and was 177 cm tall, with a body mass index (BMI) of 44. Examination of the back did not show any signs of dysraphism such as meningocele, nor did it show any discoloration, protrusions, or asymmetry. Cranial nerve examination was unremarkable and his pupils were equally reactive. Passive movement of the extremities revealed bilateral spasticity in the lower limbs. Upper-and lower-limb power was intact and symmetrical and reflexes were normal. Plantar reflex elicited flexion of the foot. No sensory deficit was noted and the proprioception examination was normal.
Routine laboratory test results, including complete blood count, coagulation profile, liver function test, and renal function tests, were unremarkable.
MRI of the lumbar spine with contrast and diffusion-weighted images was ordered for a suspected compressing mass. An oval-shaped intradural extramedullary mass was found (Figures 1–3).
He was admitted to the hospital 1 week later and underwent L5 laminectomy with excision of the intradural lesion. Surgery was done successfully, and the postoperative course was uneventful. He was discharged 3 days after admission, after his pain had improved. He was walking well and passing urine and feces normally. The pathology report was issued days later and confirmed the diagnosis of an epidermoid cyst. The pathology slides and gross image are shown in Figure 4.
The clinical presentation of spinal epidermoid cyst depends on its location and size. Back and leg pain are the most common symptoms, followed by a localized neurological deficit. Other presenting symptoms may include motor deficit, muscle atrophy, numbness, paresthesia, bowel dysfunction, and bladder dysfunction [5,6]. The cyst can suddenly rupture, which results in a sudden onset of symptoms. Rupture can be caused by increased intra-abdominal pressure, such as with coughing or sneezing . The differential diagnosis of epidermoid cyst includes dermoid, lipoma, neurofibroma, hemangioblastoma, neuroendocrine tumors, meningioma, metastasis, and schwannoma [5,6].
According to Beechar et al, the median age at diagnosis of epidermoid cysts for both sexes is around 23 years. Of the 65 spinal epidermoid cyst cases reviewed by Beechar et al, 30 cases were acquired, 26 were idiopathic, and 9 were congenital . Epidermoid cysts can occur along any portion of the spine, from the cervical spine to the cauda equina . Various studies yielded different results about the most common site for spinal epidermoid cysts. Munshi et al assessed a series of 15 patients with spinal epidermoid and found the most common sites were lumbar levels and conus region, followed by cauda equina, thoracic region, and, lastly, the filum terminale .
A history of previous surgery or procedure appears to be correlated with the development of an epidermoid cyst. There are reports of the development of lumbar epidermoid cysts in a patient with a history of more than 70 spinal injections and another case of a 24-year-old man with a history of an old lumbar puncture [2,3]. In addition, 1 patient was reported to have developed an epidermoid cyst after disc herniation surgery . However, around 40% of cases are idiopathic . Our literature review found no association between spinal epidermoid and obesity.
Radiologically, MRI is the criterion standard for the diagnosis of spinal epidermoid cysts. CT scans may be of use to evaluate vertebral deformities and bone remodeling, but perform poorly in characterizing the mass [2,5,7,8].
In the case of spinal epidermoid cyst, MRI with contrast can reveal an intradural extramedullary or intramedullary, oval, non-enhancing or peripherally-enhancing lesion, that is hypointense on T1- and hyperintense on T2-weighted imaging, showing diffusion restriction on DWI [2,3,5,7,8].
Total excision of the tumor is the definitive treatment. Whenever possible, total excision of the mass should be attempted to avoid recurrence or chemical meningitis. Although there is no high-quality evidence comparing total with subtotal resection, total resection appears to be superior, even for an adherent mass. According to Beechar et al, out of the 20 patients who underwent total resection, 18 had a positive outcome. In comparison, only 1 out of 4 in the subtotal excision group had a positive outcome [5,8].
Although rare, epidermoid cysts of the spine should be among the differential diagnoses in patients with a suspected spinal mass, especially patients with a surgical or procedural history of the spine. They can cause a wide range of non-specific symptoms, from motor deficit to numbness and paresthesia. The most common presenting symptom is back and leg pain. MRI is the criterion standard investigation and can provide a definitive diagnosis. Total excision of the mass usually leads to a good prognosis.
FiguresFigure 1.. Sagittal MRI sections. White arrows point to the lesion. (A) Sagittal MRI T1-weighted image without contrast shows a well-defined hypointense intradural extramedullary cystic lesion (solid arrow) at the Level of L5. (B) T2-weighted image shows hyperintensity of the lesion. (C) T1-weighted image with contrast and fat suppression show no enhancement of the lesion. (D) T1-weighted image delayed imaging shows no enhancement. Figure 2.. (A) Diffusion-weighted imaging. (B) Apparent diffusion coefficient (ADC). White arrows point to the lesion. (A) The lesion demonstrated restricted diffusion on DWI. (B) ADC (Apparent diffusion coefficient) is consistent with epidermoid cyst. Figure 3.. MRI Axial view. White arrows point to the lesion. (A) Axial T1-weighted image post contrast MRI shows the intradural extramedullary lesion along the cauda equina nerve roots without significant post contrast enhancement. No extension along the bilateral neural foramina. (B) Axial T2-weighted image shows hyperintense signal of the lesion. Figure 4.. (A) Gross picture of the lesion during surgery. The pathology report described the lesion grossly as a white tan friable soft tissue measuring 5×4×1 cm. (B) Low-power view shows cyst containing lamellated keratin material (original magnification ×20, H/E stain). (C) High-power view reveals cyst lining of stratified squamous epithelium with granular cell layer (original magnification ×200, H/E stain).
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3.. Dodson V, Majmundar N, Sharer LR, Gillick JL, Epidermoid cyst of the lumbar spine after lumbar puncture: A clinical, radiographic, and pathologic correlation: World Neurosurg, 2020; 137; 363-66
4.. Rkhami M, Gader G, Loukil B, Iatrogenic epidermoid cyst of the cauda equina: A late complication of lumbar disc herniation surgery: World Neurosurg, 2020; 133; 271-74
5.. Beechar VB, Zinn PO, Heck KA, Spinal epidermoid tumors: Case report and review of the literature: Neurospine, 2018; 15(2); 117-22
6.. Munshi A, Talapatra K, Ramadwar M, Jalali R, Spinal epidermoid cyst with sudden onset of paraplegia: J Cancer Res Ther, 2009; 5(4); 290-92
7.. Kumar A, Singh P, Jain P, Badole CM, Intramedullary spinal epidermoid cyst of the cervicodorsal region: A rare entity: J Pediatr Neurosci, 2010; 5(1); 49-51
8.. Yin H, Zhang D, Wu Z, Surgery and outcomes of six patients with intradural epidermoid cysts in the lumbar spine: World J Surg Oncol, 2014; 12; 50
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