Intraspinal epidermoid cyst
3
Journal of Surgical Oncology 25:64-66 (1984) lntraspinal Epidermoid Cyst DEBA P. SARMA, MD, CLAREMONT F. CARTER, MD, AND THOMAS G. WEILBAECHER, MD From the Departments of Pathology and Radiology, Louisiana State University Medical School, and Veterans Administration Medical Center, New Orleans An intraspinal epidermoid cyst occurring in the lumbo-sacral region of a 38-year old man with congenital spina bifida occulta is described. Relevant literature on the subject is reviewed. KEY WORDS: intraspinal epidermoid, spinal epidermoid cyst, benign spinal tumor INTRODUCTION Epidermoid cysts of the spine are rare lesions. We would like to describe such a case and review the relevant literature. CASE REPORT A 38-year-old white man presented with a &week history of severe right hip pain and occasional numbness with radiation down the leg to the ankle. Pain was con- stant and dull with increase in intensity on bearing weight. Physical examination revealed an abnormal dermal impression, but no sinus tract at the level of L-5. The deep tendon reflexes were intact and Babinski’s sign was absent. Straight leg raising test elicited right hip pain. There were no signs and symptoms indicating bladder or bowel dysfunction. Vascular examination (Doppler fluo- rometer) was normal with 2 to 3+ pulses without bruits in all areas. Voiding cystogram, electromyography of lower extremities, and computed tomographic scan of brain were all normal. Lumbar myelogram revealed spina bifida extending from L-5 down to the tip of sacrum and a wellcircumscribed ovoid intradural mass at the level of L-5 vertebral body with suggestion of nerve root compression (Fig. 1). The patient underwent a laminectomy and excision of the intradural mass with no sacrifice of the nerve roots. Pathologic examination revealed a collapsed cyst contain- ing soft gray-white material. The cyst wall was lined by normal-appearing epidermis (Fig. 2) with no evidence of hair, sebaceous glands, or sweat glands in the wall. The cyst content was composed of keratinous material. The postoperative course was uneventful with com- plete relief of hip pain. DISCUSSION Epidermoid cyst is composed of a thin cyst wall lined by stratifical squamous epithelium similar to the epider- 0 1% Alan R. Liss. Inc. mis of skin. The cyst content is composed of dysqua- mated cells and keratinoous material. If in addition to epidermal layer, hairs, sebaceous glands, or sweat glands are present in the cyst wall, such a tumor is called dermoid cyst, contents of which include keratin, seba- ceous material, and hair. The epidermoid cyst of the spine can be intramedullary (within the spinal cord), intradural, or extradural. The lesion can be congenital or acquired. Many of the congenital lesions are associated with spinal malformations, such as, spina bifida [ 11 and fusion of vertebrae [2], cutaneous and dermal defect in the form of pilonidal and dermal sinuses [3,4], and spinal cord defects, such as, syringomelia [4]. These lesions are regarded as resulting from the inclusion of ectodermal cells at the time of closure of the neural groove. The displaced ectodermal cells may differentiate into epider- mis leading to the formation of epidermoid cyst or into epidermis with adnexal elements thereby forming a der- moid cyst [5]. The acquired or noncongenital epidermoid cysts have been described in the patients following repeated lumbar punctures for meningitis [6], myelography and discogra- phy [7], or trauma to the back [I]. Iatrogenic penetration of epidermis in the spinal area appears to be the cause of the cyst formation. Experimental evidence of epithelial cyst formation in the brain and spinal cord of rats after implanting minced epidermis in the cranial and spinal subarachnoid space lends support to the above mecha- nism of iatrogenic cyst formation [8]. Intraspinal epidermoid cysts are rare. In a review of literature up to 1941, List found only 24 such cases [ 11. In another critical review of literature in 1962, Manno et Accepted for publication May 6, 1983. Address reprint requests to D. Sarma. MD, 1601 Perdido Street, New Orleans, LA 70146.
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Transcript of Intraspinal epidermoid cyst
Journal of Surgical Oncology 25:64-66 (1984)
lntraspinal Epidermoid Cyst
DEBA P. SARMA, MD, CLAREMONT F. CARTER, MD, AND THOMAS G. WEILBAECHER, MD
From the Departments of Pathology and Radiology, Louisiana State University Medical School, and Veterans Administration Medical Center, New Orleans
An intraspinal epidermoid cyst occurring in the lumbo-sacral region of a 38-year old man with congenital spina bifida occulta is described. Relevant literature on the subject is reviewed.
KEY WORDS: intraspinal epidermoid, spinal epidermoid cyst, benign spinal tumor
INTRODUCTION Epidermoid cysts of the spine are rare lesions. We
would like to describe such a case and review the relevant literature.
CASE REPORT A 38-year-old white man presented with a &week
history of severe right hip pain and occasional numbness with radiation down the leg to the ankle. Pain was con- stant and dull with increase in intensity on bearing weight. Physical examination revealed an abnormal dermal impression, but no sinus tract at the level of L-5. The deep tendon reflexes were intact and Babinski’s sign was absent. Straight leg raising test elicited right hip pain. There were no signs and symptoms indicating bladder or bowel dysfunction. Vascular examination (Doppler fluo- rometer) was normal with 2 to 3+ pulses without bruits in all areas. Voiding cystogram, electromyography of lower extremities, and computed tomographic scan of brain were all normal. Lumbar myelogram revealed spina bifida extending from L-5 down to the tip of sacrum and a wellcircumscribed ovoid intradural mass at the level of L-5 vertebral body with suggestion of nerve root compression (Fig. 1).
The patient underwent a laminectomy and excision of the intradural mass with no sacrifice of the nerve roots. Pathologic examination revealed a collapsed cyst contain- ing soft gray-white material. The cyst wall was lined by normal-appearing epidermis (Fig. 2) with no evidence of hair, sebaceous glands, or sweat glands in the wall. The cyst content was composed of keratinous material.
The postoperative course was uneventful with com- plete relief of hip pain.
DISCUSSION Epidermoid cyst is composed of a thin cyst wall lined
by stratifical squamous epithelium similar to the epider- 0 1% Alan R. Liss. Inc.
mis of skin. The cyst content is composed of dysqua- mated cells and keratinoous material. If in addition to epidermal layer, hairs, sebaceous glands, or sweat glands are present in the cyst wall, such a tumor is called dermoid cyst, contents of which include keratin, seba- ceous material, and hair.
The epidermoid cyst of the spine can be intramedullary (within the spinal cord), intradural, or extradural. The lesion can be congenital or acquired.
Many of the congenital lesions are associated with spinal malformations, such as, spina bifida [ 11 and fusion of vertebrae [2], cutaneous and dermal defect in the form of pilonidal and dermal sinuses [3,4], and spinal cord defects, such as, syringomelia [4]. These lesions are regarded as resulting from the inclusion of ectodermal cells at the time of closure of the neural groove. The displaced ectodermal cells may differentiate into epider- mis leading to the formation of epidermoid cyst or into epidermis with adnexal elements thereby forming a der- moid cyst [5].
The acquired or noncongenital epidermoid cysts have been described in the patients following repeated lumbar punctures for meningitis [6], myelography and discogra- phy [7], or trauma to the back [I]. Iatrogenic penetration of epidermis in the spinal area appears to be the cause of the cyst formation. Experimental evidence of epithelial cyst formation in the brain and spinal cord of rats after implanting minced epidermis in the cranial and spinal subarachnoid space lends support to the above mecha- nism of iatrogenic cyst formation [8].
Intraspinal epidermoid cysts are rare. In a review of literature up to 1941, List found only 24 such cases [ 11. In another critical review of literature in 1962, Manno et
Accepted for publication May 6, 1983. Address reprint requests to D. Sarma. MD, 1601 Perdido Street, New Orleans, LA 70146.
Intraspinal Epidermoid Cyst 65
al [4] found 90 cases of which 37 (41%) cases were iatrogenic in nature. In a more recent study, of 4296 space-occupying lesions of the brain and spinal cord seen over a period of 21 years, only 3 spinal epidermoid cysts were seen [9].
Epidermoid cysts are slow-growing lesions. Age of presentation varies from early childhood to late adult- hood. Neurologic symptoms and signs depend on the level and extent of the lesion. Sensory and motor distur- bances, nerve root pain, and evidence of arachnoiditis may be present. Infected and draining dermal sinus over- lying the intraspinal lesion may be the presenting symp- tom. History of previous lumbar punctures may suggest acquired type of lesion. Roentgenograms and myelo- grams of the spine will show any spinal abnormality and the level and size of the tumor.
Surgical removal of the tumor is curative. Recurrence is due to incomplete removal. For a large tumor with a thin capsule firmly adherent to the cord or nerve roots, complete removal may not be possible. In such cases an intracapsular enucleation with partial removal of the cap- sule may give long-lasting symptomatic relief [ 1,101. Fig. 1. Myelogram showing spina bitida and intradural mass.
Fig. 2. Wall of the cyst showing stratified squamous epithelium resembling epidermis (hematoxylin & eosin, X 80).
66 Sarma, Carter, and Weilbaecher
Care should be be taken to avoid meningeal contamina- tion during the surgical procedure to reduce the risk of chemical meningitis and implantation of viable epidermal cells.
ACKNOWLEDGMENTS Mrs. Emily Chauvin provided the secretarial assistance.
REFERENCES I .
2.
3.
List CF: Intraspinal epidermoids. dermoids and dermal sinuses. Surg Gynecol Obstet 73525-538. 1941. Keville FJ, Wise BL: lntracranial epidermoid and dermoid tu- mors. J Neurosurg 16:564-569, 1959. Sachs E, Horrax G: A cervical and lumbar pilonidal sinus com- municating with intraspinal dermoids. J Neurosurg 6:97- 112.
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1949. Manno NJ, Uihlein A,Kernohan JW: Intraspinal epidermoids. J Neurosurg 19;754-765, 1962. Bostrijem E: Uber die pialen epidermoide, dermoide, und lipome und duralen dermoide. Zentralbi Allg Pathol 8: 1-98, 1897. 1897. Blockey NJ, Schorstein J: Intraspinal epidermoid tumors in the lumbar region of man. J Bone Joint Surg 43B:556-562, 1961. Boyd HR: Iatrogenic intraspinal epidermoid. J Neurosurg 24: 105- 107, 1966. Van Gilder JC, Schwartz HG: Growth of dermoids from skin implants to the nervous system and surrounding spaces of the newborn rat. J Neurosurg 26: 14-20, 1967. Guidetti B, Gagliardi FM: Epidermoid and dermoid cysts. Clini- cal evaluation and late surgical results. J Neurosurg 47: 12-18, 1977. Stevens WW, Schlesinger EB: Intramedullary epidermoid tumors of the thoracic spinal cord: Report of two cases. J Neurosurg 291296-299, 1968.
