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Journal of Cancer Research and Therapeutics, Vol. 5, No. 4, October-December, 2009, pp. 290-292 Case Report Spinal epidermoid cyst with sudden onset of paraplegia Munshi Anusheel, Talapatra Kaustav, Ramadwar Mukta, Jalali Rakesh Department of Radiation Oncology, Tata Memorial Hospital, Mumbai Code Number: cr09068 PMID: 20160364 DOI: 10.4103/0973-1482.59913 Abstract Spinal epidermoid cysts, whether congenital or iatrogenic, are relatively uncommon in the spinal cord. When they occur, the typical location is in the subdural, extramedullary space of the lumbo-sacral region. We describe an unusual presentation in a 3-year-old male child which mimicked astrocytoma clinicoradiologically. The child developed sudden onset of inability in walking and weakness of both lower limbs after a fall. There was a dramatic reversal of symptoms after surgery. Histopathology revealed an epidermoid cyst of the spine. On the first follow-up visit at 3 months, the child was asymptomatic.Keywords: Epidermoid cyst, spinal, paraplegia Introduction Spinal epidermoid cysts are relatively uncommon in the spinal cord. When they occur, they can be congenital or iatrogenic and are seen in the subdural, extramedullary space of the lumbosacral region. [1] This article discusses a child who presented with sudden neurological deterioration and was diagnosed as having an epidermoid cyst. Case Report A 3-year-old male child had a fall while walking. After a few hours he presented with sudden onset inability in walking and weakness of both lower limbs. The child had a history of a superficial discharging sinus in the upper lumbar region since birth. MRI of the spine showed abnormal hyperintense signal intensity involving the dorsal spine from D1 to D11 levels with no disc protrusion suggestive of an astrocytoma [Figure - 1]. There was no evident underlying pathology in the area of the superficial discharging sinus. There was no evidence of spinal dysraphism in MRI. The patient underwent a laminectomy along with near total excision of the intramedullary lesion and excision of the sinus. The plane between the lesion and the cord was not well defined in most areas. Histopathology showed a dense mixed chronic inflammatory infiltrate including several foreign body-type giant cells involving the meninges and spinal cord parenchyma. This florid inflammatory response was related to keratinous debris most probably representing enucleate squamous cells [Figure - 2]. Although the cyst wall was not evident in any of the multiple sections studied, the close association of the keratinous material to the inflammatory process, including invasion into the meninges and spinal cord parenchyma, favored the diagnosis of a ruptured epidermoid cyst. Immunohistochemistry using an antibody to cytokeratin highlighted this material confirming its epithelial/keratinous nature [[Figure - 2], inset]. Postoperatively, there was significant improvement in lower limb power and the child was able to walk without support. The patient was kept on follow-up and was doing fine at the first 3-monthly follow-up visit with normal power in all extremities. Epidermoid cysts represent less than 1% of all intraspinal tumors. Their etiology can either be congenital or iatrogenic, the latter as a result of epidermal cells implanted within the spinal canal. The genesis of congenital spinal epidermoids is an anomalous implantation of ectodermal cells during the closure of the neural tube between the third and fifth week of embryonic life. [2],[3],[4] These lesions are found most commonly in the subdural, extramedullary space of the lumbosacral region. [4] In a series of 15 patients with spinal intradural dysembryogenetic tumors, the tumor site was the thoracic region in three cases, the lumbar cord and conus in six, the cauda equina in four and the filum terminale in two. [1] Symptoms of spinal epidermoid cysts are directly related to tumor size and site. [5] These lesions remain asymptomatic for extended periods of time with various series reporting follow-up ranging from 2 to 10 years. [3],[5] Any incident which increases the intra-abdominal pressure and the intraspinal pressure, as happened in our case, is liable to rupture the cyst. Falls, severe cough, and sneezing are examples of such incidents. A study described weakness and urinary incontinence in a patient which reversed with surgery. [6] While gradual onset weakness has been described in these lesions, a sudden onset, as happened in our case, should put the clinician on guard for suspecting a cyst rupture. The development of sudden paraplegia in our case was thought to be due to the inflammation following the rupture of the epidermoid cyst. Though benign and relatively slow to cause symptoms, inflammatory reaction and chemical meningitis due to the rupture of the epidermoid cyst can produce grave symptoms and jeopardize a patient′s quality of life. It is extremely important to recognize it early as it can often mimic a malignant tumor as was the case in our patient. Skin lesions, including dyschromias, scar, or hairy nevus at the overlying midline may suggest an association with a dysembryogenic tumor. [1] Our patient too had a discharging sinus in the back, although we could not determine any other associated tumor, nor demonstrate any abnormality in MRI in relation to this area. Pathologically, an epidermoid cyst contains keratin, cholesterol crystals, and desquamated epithelial cells. [7] If ruptured, the disseminated material of the cyst produces a vigorous inflammatory reaction, as was the situation in the present case. Radiographic features of the intact epidermoid cyst include displacement of nerve roots or the spinal cord in the intradural space. They are commonly hypo- or isointense on T1-weighted MRI and hyperintense on T2-weighted MRI, and contrast enhancement is uncommon. However, the signal characteristics of epidermoid tumors are known to vary widely. [8] The differentials of a spinal epidermoid cyst include hemangioblastoma, neutrino, meningioma, metastasis, and scwannoma. Unlike the extramedullary presentation which is the norm for epidermoid cysts, our patient presented with an intramedullary lesion. Surgery is the treatment of choice for spinal epidermoids and maximal resection should be attempted. Adherence to medullary structures may preclude a safe total removal. Intraoperative concerns during removal of these cysts include the possible contamination of the field with spillage of cyst contents. [4] There is an isolated report stating that radical radiotherapy was delivered to a patient with an cervical intraspinal epidermoid cyst to a dose of 46 Gy in 23 fractions to the cervicothoracic spine by opposing anteroposterior high-energy photon beams. This patient had refused surgery. He was given radiotherapy; the patient′s clinical symptoms improved subsequently. [9] To summarize, the recognition of epidermoid cysts in the pediatric age group is important. There may be associated congenital anomalies which need to be carefully sought as an aid to diagnosis. Surgical excision forms the mainstay of treatment and the overall outcome of these lesions is good, even if they present with a ruptured cyst, as was the case with our patient. References
Copyright 2009 - Journal of Cancer Research and Therapeutics The following images related to this document are available:Photo images[cr09068f2.jpg] [cr09068f1.jpg] |
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