- Email: [email protected]
Spinal Intradural Extramedullary Dermoid Cyst
Journal Pre-proof Spinal Intradural, Extramedullary Dermoid Cyst: a Case Report Farnaz Khalighinejad, Mahsa Majizadeh, Ali Moktari, Reza Rakhshan, Majid Hajizadeh, Majid Rezvani PII:
S1878-8750(19)32911-0
DOI:
https://doi.org/10.1016/j.wneu.2019.11.073
Reference:
WNEU 13742
To appear in:
World Neurosurgery
Received Date: 10 October 2019 Revised Date:
11 November 2019
Accepted Date: 12 November 2019
Please cite this article as: Khalighinejad F, Majizadeh M, Moktari A, Rakhshan R, Hajizadeh M, Rezvani M, Spinal Intradural, Extramedullary Dermoid Cyst: a Case Report, World Neurosurgery (2019), doi: https://doi.org/10.1016/j.wneu.2019.11.073. This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. © 2019 Published by Elsevier Inc.
Spinal Intradural, Extramedullary Dermoid Cyst: a Case Report
Farnaz Khalighinejad 1, Mahsa Majizadeh* 2, Ali Moktari 3, Reza Rakhshan 4, Majid Hajizadeh 5, Majid Rezvani 6
1. University of Massachusetts Medical School, Worcester, MA, USA 2. Isfahan Research Committee of pathology, Isfahan University of Medical Sciences, Isfahan, Iran 3. Department of neurosurgery, Isfahan University of Medical Sciences, Isfahan, Iran 4. Department of pathology, Isfahan University of Medical Sciences, Isfahan, Iran 5. Department of Radiology, Abadan University of medical sciences, Abadan, Iran 6. Department of Neurosurgery, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran
Corresponding author: Mahsa Hajizadeh Email: [email protected] Address: Isfahan Research Committee of Pathology, Isfahan, Iran TEL: +98(913)43-60-869
Spinal Intradural, Extramedullary Dermoid Cyst: a Case Report
Farnaz Khalighinejad 1, Mahsa Majizadeh* 2, Ali Moktari 3, Reza Rakhshan 4, Majid Hajizadeh 5, Majid Rezvani 6
1. University of Massachusetts Medical School, Worcester, MA, USA 2. Isfahan Research Committee of pathology, Isfahan University of Medical Sciences, Isfahan, Iran 3. Department of neurosurgery, Isfahan University of Medical Sciences, Isfahan, Iran 4. Department of pathology, Isfahan University of Medical Sciences, Isfahan, Iran 5. Department of Radiology, Abadan University of medical sciences, Abadan, Iran
6. Department of Neurosurgery, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran
Corresponding author: Mahsa Hajizadeh Email: [email protected] Address: Isfahan Research Committee of Pathology, Isfahan, Iran TEL: +98(913)43-60-869
Abstract: Introduction: Dermoid cysts are benign congenital tumors that develop early in life. These tumors are classified by the presence of all three germ layers. Spinal intradural extramedullary teratoma is a rare disease, which is more common in children under 5 years old than in adults. Patient description: A 12-year-old girl with a dermoid cyst at the lower lumbar level, presenting with two month low back pain and intermittent lower-extremity radicular symptoms on the right side. MRI of the spine revealed an intradural extramedullary mass lesion at lumbar level 4-5. Surgical excision of the cyst was successfully performed. Surgical and histopathological findings confirmed extramedullary ruptured matured teratoma. Postoperatively, the patient had the remarkable clinical improvement. Conclusion: although dermoid cysts are uncommon, they should be considered in the differential diagnosis of spinal lesions in patients with lower back pain . It can be successfully treated with surgical excision.
Key words: Dermoid cyst; Intradural extramedullary; Spinal; Teratoma.
Background: Teratomas are classified based on the amount of cell differentiation: mature, immature, or malignant. Most of these tumors are identified in children and are rare in the adult population [1]. Dermoid cysts are benign congenital tumors that develop early in life in ectodermal and mesodermal tissues. Histologically, dermoid cysts are surrounded by keratinizing squamous cells and contain hair follicles, sweat glands, and sebaceous glands. About 70% of dermoid cysts are found in children 5 years old or younger [2]. Here we report a case of an intradural, extramedullary dermoid cyst in a child, emphasizing the pathologic investigations and surgical treatment.
Case Description: A 12 years old girl in October 2016 was visited due to her low back pain and intermittent lower-extremity radicular symptoms on the right side, which had been started 2 month earlier. The low back pain had been started gradually without any paresthesia or urinary incontinency. Also, she didn’t have anorexia or weight loss. Oral analgesics initially afforded her some relief. She had neither a history of spinal surgery nor any other spinal procedures. The intelligence and psychologic state of the girl were confirmed normal from the dialogues with her and the narratives of his parents. No positive sign of abnormal hairy patch or dimple was found through physical examinations on her back . Cranial nerves II to XII were grossly intact. Neurological examination revealed that the anal tone was within the normal range. Ultimately, she had no major illnesses and injuries and no history of seizure or hydrocephalus. Magnetic resonance imaging (MRI) was ordered to work up her low back pain. MRI study of the lumbar spine, with and without contrast, revealed an intradural extramedullary lesion with heterogenous signal measuring 51mm craniocaudally × 11mm transversely × 7mm anteroposteriory, located opposite L4- L5 vertebral levels predominantly on the right side leading to compression and displacement of cauda equine nerve roots toward the left side as shown in Figure1-D. The mass was hypointense to the cord on the sagittal T1-weighted images with some high signal areas and hyperintense axial T2-weighted images and showed minimal enhancement on the myelogram images (Figure1-A,B and C). There was no lateral extension of the mass into lateral recess. The rest of the spinal cord, cord termination, and conus medullaris were normal in signal thus ruling out spinal cord edema or myelomalacia. Intervertebral discs were found to be normal. No prevertebral and paravertebral collection were observed. The MRI study of the brain and cervical spine were normal.
She was subsequently taken to the operation room and the operation was performed in the prone position. The procedure involved laminectomy at the L4L5 levels for tumor resection. On incising the dura mater via the midline incision, a white-yellowish, oval‑shaped, fatty ruptured cyst was observed. Also, the hairy substance was seen. Total tumor resection was performed. The excised mass contained lumps of white-yellowish cheese-like material containing hair follicles and fragments of whitish soft tissue. [Figure 2] The tissue specimen was fixed in 10% formalin and routinely processed. The evaluation of haematoxylin-eosin (H&E) histological section revealed a lesion consisting of fibrous tissue with squamous epithelium and hair shafts. Also, no evidence of immature elements or malignancy was observed in the specimens. The overall features were consistent with ruptured mature cystic teratoma (Figure 3). The dura mater was repaired in a watertight manner. Postoperative period was uneventful, and the patient was discharged on day 3 without any neurological deficit. Moreover, the patient reported a drastic improvement in pain. During the next 3-month follow-up, no further neurological deterioration was observed.
Conclusion: We described here an intradural extramedullary dermoid cyst showing low back pain with radiation to the right lower limb. The gold standard diagnostic test for teratomas is the histopathologic examination. Although dermoid cysts are classified classically by the presence of all three germ layers, it has been previously found that the total number of layers may be difficult to find out in some cases because of overgrowth of 1 or 2 germ layers; as a result, the diagnosis cannot be ruled out if all three layers cannot be demonstrated in the tissue specimen [3]. Furthermore, these cysts show different appearances of the lining epithelial, ranging from cuboidal to pseudostratified columnar cells [4]. In our
case, histopathology exam showed ruptured mature cystic teratoma with the squamous epithelium, adipose tissue and hair shafts, with an inflammation reaction around the cyst. Dermoid cysts can rupture spontaneously and release their contents in subarachnoid space which would lead to chemical meningitis. The ruptured wall can heal, but if the healed wall ruptures again, it will cause recurrent signs [5]. In our case, the cyst was ruptured but fortunately it didn’t cause any consequenses. Dermoid cysts can develop anywhere on the body, but they are most frequently seen in the coccyx (44.5%) and ovaries (42.1%), followed by the head and neck (particularly in periorbital lateral eyebrow area) [4]. Spinal tumors can be extradural or intradural. Intradural tumors can further be classified as intramedullary or extramedullary [6]. The spinal Dermoid cyst is a rare disease. In 1863, the first case of this condition was described by Virchow. Only a few series of studies on spinal teratoma has been reported in the medical literature [7]. Cystic teratomas are more common in children but adult cystic cases are increasingly being reported, however, the Spinal intradural extramedullary teratoma is a rare entity in adults [6, 8]. In adults, teratomas are the mature subtype and occur most commonly at the level of the conus. In contrast, in the pediatric population, most reported cases are the other subtypes and are usually in the sacrococcygeal region [1]. Based on the last investigations, there are two theories proposed to explain these spinal tumors: misplaced germ cell theory and dysembryogenic theory. In the first theory, teratomas are described as disordered migrated and misplacement primordial germ cells from the yolk sac, typically into midline location. Also, they are not real neoplastic lesions. The latter theory suggests that teratomas originated from disordered differentiation of pluripotent cells in an aberrant developmental environment in the primitive streak or cauda cell mass. Moreover, Congenital
anomalies and dysraphic malformations are supported by the dysembryogenic theory. Some researchers support the misplaced germ cell theory more strongly [3]. Misplaced germ lines migrate to the caudal mass at the conus and lower thoracic regions of the developing embryo. This predicts the common location of mature teratomas at or near the conus. Mature spinal dermoid cysts are usually adherent to nervous tissues and are composed of cystic structures that may compress the neural elements [1].
Spinal teratomas constitute 1% of intraspinal tumors. They may rupture during surgery or spontaneously by trauma. In the spine, they occur predominantly in the lumbosacral region (60%) followed by the upper thoracic (10%) and cervical levels (5%). These tumors can be associated with bone malformation, myelomeningocele, hypertrichosis and dermal sinus tract [9]. Moreover, Diastematomyelia and tethered cord syndrome have been reported in spinal teratomas [7].
In the intradural extramedullary tumors, the MRI findings include displacement of the cord to the contralateral side of the thecal sac, widening of the space available for the cerebrospinal fluid above and below the tumor, and a sharp demarcation between the tumor and the cerebrospinal fluid. The spinal cord is often flattened against the dura mater on the contralateral side with larger tumors [6]. Regardless of advantages in MRI techniques, accurate preference of tumor histology based only on radiographic characteristics remains unreliable [10] , as in our case where MRI didn’t confirm the diagnosis of a teratoma. This MRI study revealed no lateral extension of the mass into lateral recess and the rest of the spinal cord, cord termination, and conus medullaris were normal in signal thus ruling out spinal cord edema or myelomalacia. The tumor was located predominantly on the right side leading to compression and displacement of cauda equine nerve roots toward the
left side and without any associated disease. High-resolution MRI with contrast can visualize the vascularity, tumor size, homogeneity and its relationship with the surrounding structures [10]. In our case, the mass was hyperintense in T2-weighted images and showed minimal enhancement of the contrast agent. The location of spinal teratomas defined the clinical manifestations. However, they did not show any specific symptoms. The literature revealed that intraspinal mature teratomas may cause long-time numbness, weakness of the lower extremities, cauda equine syndrome with a progressive neurological deterioration and urinary or erection disturbances [10]. Our 12 years old patient had insidious low back pain with no other previous clinical symptoms. The tumor compression and displacement of cauda equine nerve roots toward the left side describe the intermittent lower-extremity radicular symptoms of the patient. Surgical excision can be secure for most benign germ cell tumors. Treatment of intraspinal teratomas is surgical excision, which is also dependent upon the age of the patient and location of the tumor [5, 10]. However, particularly in symptomatic patients, Surgery is advocated as the treatment of choice. Commonly, this halts any further progression neurologic injury. By subtotal resection of these cysts, the chance of recurrence can increase; however, it is not necessary to excise a firmly attached tumor [8]. Dermoid cysts should be included in the differential diagnosis of spinal lesions in patients with lower back pain or neurological deficits of the lower extremities. We reported a rare case of a spinal intradural extramedullary ruptured mature cystic teratoma in a 12 years old girl. Our study provided additional information to the literature reporting a rare case of spinal intradural extramedullary dermoid cysts in a previously healthy patient with 2 month insidious low back pain and right side lower-extremity radicular symptoms.
ACKNOWLEDGMENT: We would like to thank the patient who consented to this publication. Declaration of Conflicting Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. Funding: The author(s) received no financial support for the research, authorship, and/or publication of this article.
Refreneces: 1. 2. 3. 4. 5. 6. 7. 8. 9.
10.
Danison, A.P., et al., Adult Thoracic Intradural Exophytic Mature Teratoma: Case Report and Literature Review. Asian J Neurosurg, 2018. 13(4): p. 1182-1185. Choi, J.S., et al., Dermoid cysts: Epidemiology and diagnostic approach based on clinical experiences. Arch Plast Surg, 2018. 45(6): p. 512-516. Turan, N., et al., Adult Intramedullary Teratoma of the Spinal Cord: A Case Report and Review of Literature. World Neurosurg, 2016. 87: p. 661.e23-30. Jayasuriya, N.S., et al., Malignant transformation of a long-standing submental dermoid cyst to a carcinosarcoma: a case report. J Med Case Rep, 2017. 11(1): p. 11. Mpayo, L.L., et al., Extramedullary spinal teratoma presenting with recurrent aseptic meningitis. Pediatr Neurol, 2014. 50(6): p. 655-7. Pandey, S., et al., Spinal intradural extramedullary mature cystic teratoma in an adult: A rare tumor with review of literature. Asian J Neurosurg, 2015. 10(3): p. 133-7. Sung, K.S., et al., Spinal intradural extramedullary mature cystic teratoma in an adult. J Korean Neurosurg Soc, 2008. 44(5): p. 334-7. Stevens, Q.E., et al., Intradural extramedullary mature cystic teratoma: not only a childhood disease. J Spinal Disord Tech, 2006. 19(3): p. 213-6. Kabbasch, C., et al., Rupture of a spinal dermoid cyst may lead to dissemination and progress of Fatty tissue in the central spinal canal and intracranial subarachnoid space. A case report. Neuroradiol J, 2014. 27(6): p. 759-63. Triantafyllidi, E., et al., Adult intradural extramedullary teratoma of the spinal cord: A case presentation. Clin Neurol Neurosurg, 2018. 175: p. 54-56.
Figure 1: A) The hyperintense mass on the axial T2-weighted image, B&C) hypointense to the cord on the sagittal T1-weighted images with some high signal areas and D) on the myelogram image, an intradural extramedullary lesion with heterogenous signal predominantly on the right side leading to compression and displacement of cauda equine nerve roots toward the left side. Figure 2: Lumps of white-yellowish cheese material in the excised mass, containing hair follicles and fragments of whitish soft tissue. Figure 3 (1&2&3): Histological sections consist of fibrous tissue with squamous epithelium and hair shafts.
ACKNOWLEDGMENT: We would like to thank the patient who consented to this publication. Declaration of Conflicting Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. Funding: The author(s) received no financial support for the research, authorship, and/or publication of this article.
MRI: Magnetic resonance imaging H&E: Haematoxylin & Eosin
S1878-8750(19)32911-0
DOI:
https://doi.org/10.1016/j.wneu.2019.11.073
Reference:
WNEU 13742
To appear in:
World Neurosurgery
Received Date: 10 October 2019 Revised Date:
11 November 2019
Accepted Date: 12 November 2019
Please cite this article as: Khalighinejad F, Majizadeh M, Moktari A, Rakhshan R, Hajizadeh M, Rezvani M, Spinal Intradural, Extramedullary Dermoid Cyst: a Case Report, World Neurosurgery (2019), doi: https://doi.org/10.1016/j.wneu.2019.11.073. This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. © 2019 Published by Elsevier Inc.
Spinal Intradural, Extramedullary Dermoid Cyst: a Case Report
Farnaz Khalighinejad 1, Mahsa Majizadeh* 2, Ali Moktari 3, Reza Rakhshan 4, Majid Hajizadeh 5, Majid Rezvani 6
1. University of Massachusetts Medical School, Worcester, MA, USA 2. Isfahan Research Committee of pathology, Isfahan University of Medical Sciences, Isfahan, Iran 3. Department of neurosurgery, Isfahan University of Medical Sciences, Isfahan, Iran 4. Department of pathology, Isfahan University of Medical Sciences, Isfahan, Iran 5. Department of Radiology, Abadan University of medical sciences, Abadan, Iran 6. Department of Neurosurgery, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran
Corresponding author: Mahsa Hajizadeh Email: [email protected] Address: Isfahan Research Committee of Pathology, Isfahan, Iran TEL: +98(913)43-60-869
Spinal Intradural, Extramedullary Dermoid Cyst: a Case Report
Farnaz Khalighinejad 1, Mahsa Majizadeh* 2, Ali Moktari 3, Reza Rakhshan 4, Majid Hajizadeh 5, Majid Rezvani 6
1. University of Massachusetts Medical School, Worcester, MA, USA 2. Isfahan Research Committee of pathology, Isfahan University of Medical Sciences, Isfahan, Iran 3. Department of neurosurgery, Isfahan University of Medical Sciences, Isfahan, Iran 4. Department of pathology, Isfahan University of Medical Sciences, Isfahan, Iran 5. Department of Radiology, Abadan University of medical sciences, Abadan, Iran
6. Department of Neurosurgery, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran
Corresponding author: Mahsa Hajizadeh Email: [email protected] Address: Isfahan Research Committee of Pathology, Isfahan, Iran TEL: +98(913)43-60-869
Abstract: Introduction: Dermoid cysts are benign congenital tumors that develop early in life. These tumors are classified by the presence of all three germ layers. Spinal intradural extramedullary teratoma is a rare disease, which is more common in children under 5 years old than in adults. Patient description: A 12-year-old girl with a dermoid cyst at the lower lumbar level, presenting with two month low back pain and intermittent lower-extremity radicular symptoms on the right side. MRI of the spine revealed an intradural extramedullary mass lesion at lumbar level 4-5. Surgical excision of the cyst was successfully performed. Surgical and histopathological findings confirmed extramedullary ruptured matured teratoma. Postoperatively, the patient had the remarkable clinical improvement. Conclusion: although dermoid cysts are uncommon, they should be considered in the differential diagnosis of spinal lesions in patients with lower back pain . It can be successfully treated with surgical excision.
Key words: Dermoid cyst; Intradural extramedullary; Spinal; Teratoma.
Background: Teratomas are classified based on the amount of cell differentiation: mature, immature, or malignant. Most of these tumors are identified in children and are rare in the adult population [1]. Dermoid cysts are benign congenital tumors that develop early in life in ectodermal and mesodermal tissues. Histologically, dermoid cysts are surrounded by keratinizing squamous cells and contain hair follicles, sweat glands, and sebaceous glands. About 70% of dermoid cysts are found in children 5 years old or younger [2]. Here we report a case of an intradural, extramedullary dermoid cyst in a child, emphasizing the pathologic investigations and surgical treatment.
Case Description: A 12 years old girl in October 2016 was visited due to her low back pain and intermittent lower-extremity radicular symptoms on the right side, which had been started 2 month earlier. The low back pain had been started gradually without any paresthesia or urinary incontinency. Also, she didn’t have anorexia or weight loss. Oral analgesics initially afforded her some relief. She had neither a history of spinal surgery nor any other spinal procedures. The intelligence and psychologic state of the girl were confirmed normal from the dialogues with her and the narratives of his parents. No positive sign of abnormal hairy patch or dimple was found through physical examinations on her back . Cranial nerves II to XII were grossly intact. Neurological examination revealed that the anal tone was within the normal range. Ultimately, she had no major illnesses and injuries and no history of seizure or hydrocephalus. Magnetic resonance imaging (MRI) was ordered to work up her low back pain. MRI study of the lumbar spine, with and without contrast, revealed an intradural extramedullary lesion with heterogenous signal measuring 51mm craniocaudally × 11mm transversely × 7mm anteroposteriory, located opposite L4- L5 vertebral levels predominantly on the right side leading to compression and displacement of cauda equine nerve roots toward the left side as shown in Figure1-D. The mass was hypointense to the cord on the sagittal T1-weighted images with some high signal areas and hyperintense axial T2-weighted images and showed minimal enhancement on the myelogram images (Figure1-A,B and C). There was no lateral extension of the mass into lateral recess. The rest of the spinal cord, cord termination, and conus medullaris were normal in signal thus ruling out spinal cord edema or myelomalacia. Intervertebral discs were found to be normal. No prevertebral and paravertebral collection were observed. The MRI study of the brain and cervical spine were normal.
She was subsequently taken to the operation room and the operation was performed in the prone position. The procedure involved laminectomy at the L4L5 levels for tumor resection. On incising the dura mater via the midline incision, a white-yellowish, oval‑shaped, fatty ruptured cyst was observed. Also, the hairy substance was seen. Total tumor resection was performed. The excised mass contained lumps of white-yellowish cheese-like material containing hair follicles and fragments of whitish soft tissue. [Figure 2] The tissue specimen was fixed in 10% formalin and routinely processed. The evaluation of haematoxylin-eosin (H&E) histological section revealed a lesion consisting of fibrous tissue with squamous epithelium and hair shafts. Also, no evidence of immature elements or malignancy was observed in the specimens. The overall features were consistent with ruptured mature cystic teratoma (Figure 3). The dura mater was repaired in a watertight manner. Postoperative period was uneventful, and the patient was discharged on day 3 without any neurological deficit. Moreover, the patient reported a drastic improvement in pain. During the next 3-month follow-up, no further neurological deterioration was observed.
Conclusion: We described here an intradural extramedullary dermoid cyst showing low back pain with radiation to the right lower limb. The gold standard diagnostic test for teratomas is the histopathologic examination. Although dermoid cysts are classified classically by the presence of all three germ layers, it has been previously found that the total number of layers may be difficult to find out in some cases because of overgrowth of 1 or 2 germ layers; as a result, the diagnosis cannot be ruled out if all three layers cannot be demonstrated in the tissue specimen [3]. Furthermore, these cysts show different appearances of the lining epithelial, ranging from cuboidal to pseudostratified columnar cells [4]. In our
case, histopathology exam showed ruptured mature cystic teratoma with the squamous epithelium, adipose tissue and hair shafts, with an inflammation reaction around the cyst. Dermoid cysts can rupture spontaneously and release their contents in subarachnoid space which would lead to chemical meningitis. The ruptured wall can heal, but if the healed wall ruptures again, it will cause recurrent signs [5]. In our case, the cyst was ruptured but fortunately it didn’t cause any consequenses. Dermoid cysts can develop anywhere on the body, but they are most frequently seen in the coccyx (44.5%) and ovaries (42.1%), followed by the head and neck (particularly in periorbital lateral eyebrow area) [4]. Spinal tumors can be extradural or intradural. Intradural tumors can further be classified as intramedullary or extramedullary [6]. The spinal Dermoid cyst is a rare disease. In 1863, the first case of this condition was described by Virchow. Only a few series of studies on spinal teratoma has been reported in the medical literature [7]. Cystic teratomas are more common in children but adult cystic cases are increasingly being reported, however, the Spinal intradural extramedullary teratoma is a rare entity in adults [6, 8]. In adults, teratomas are the mature subtype and occur most commonly at the level of the conus. In contrast, in the pediatric population, most reported cases are the other subtypes and are usually in the sacrococcygeal region [1]. Based on the last investigations, there are two theories proposed to explain these spinal tumors: misplaced germ cell theory and dysembryogenic theory. In the first theory, teratomas are described as disordered migrated and misplacement primordial germ cells from the yolk sac, typically into midline location. Also, they are not real neoplastic lesions. The latter theory suggests that teratomas originated from disordered differentiation of pluripotent cells in an aberrant developmental environment in the primitive streak or cauda cell mass. Moreover, Congenital
anomalies and dysraphic malformations are supported by the dysembryogenic theory. Some researchers support the misplaced germ cell theory more strongly [3]. Misplaced germ lines migrate to the caudal mass at the conus and lower thoracic regions of the developing embryo. This predicts the common location of mature teratomas at or near the conus. Mature spinal dermoid cysts are usually adherent to nervous tissues and are composed of cystic structures that may compress the neural elements [1].
Spinal teratomas constitute 1% of intraspinal tumors. They may rupture during surgery or spontaneously by trauma. In the spine, they occur predominantly in the lumbosacral region (60%) followed by the upper thoracic (10%) and cervical levels (5%). These tumors can be associated with bone malformation, myelomeningocele, hypertrichosis and dermal sinus tract [9]. Moreover, Diastematomyelia and tethered cord syndrome have been reported in spinal teratomas [7].
In the intradural extramedullary tumors, the MRI findings include displacement of the cord to the contralateral side of the thecal sac, widening of the space available for the cerebrospinal fluid above and below the tumor, and a sharp demarcation between the tumor and the cerebrospinal fluid. The spinal cord is often flattened against the dura mater on the contralateral side with larger tumors [6]. Regardless of advantages in MRI techniques, accurate preference of tumor histology based only on radiographic characteristics remains unreliable [10] , as in our case where MRI didn’t confirm the diagnosis of a teratoma. This MRI study revealed no lateral extension of the mass into lateral recess and the rest of the spinal cord, cord termination, and conus medullaris were normal in signal thus ruling out spinal cord edema or myelomalacia. The tumor was located predominantly on the right side leading to compression and displacement of cauda equine nerve roots toward the
left side and without any associated disease. High-resolution MRI with contrast can visualize the vascularity, tumor size, homogeneity and its relationship with the surrounding structures [10]. In our case, the mass was hyperintense in T2-weighted images and showed minimal enhancement of the contrast agent. The location of spinal teratomas defined the clinical manifestations. However, they did not show any specific symptoms. The literature revealed that intraspinal mature teratomas may cause long-time numbness, weakness of the lower extremities, cauda equine syndrome with a progressive neurological deterioration and urinary or erection disturbances [10]. Our 12 years old patient had insidious low back pain with no other previous clinical symptoms. The tumor compression and displacement of cauda equine nerve roots toward the left side describe the intermittent lower-extremity radicular symptoms of the patient. Surgical excision can be secure for most benign germ cell tumors. Treatment of intraspinal teratomas is surgical excision, which is also dependent upon the age of the patient and location of the tumor [5, 10]. However, particularly in symptomatic patients, Surgery is advocated as the treatment of choice. Commonly, this halts any further progression neurologic injury. By subtotal resection of these cysts, the chance of recurrence can increase; however, it is not necessary to excise a firmly attached tumor [8]. Dermoid cysts should be included in the differential diagnosis of spinal lesions in patients with lower back pain or neurological deficits of the lower extremities. We reported a rare case of a spinal intradural extramedullary ruptured mature cystic teratoma in a 12 years old girl. Our study provided additional information to the literature reporting a rare case of spinal intradural extramedullary dermoid cysts in a previously healthy patient with 2 month insidious low back pain and right side lower-extremity radicular symptoms.
ACKNOWLEDGMENT: We would like to thank the patient who consented to this publication. Declaration of Conflicting Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. Funding: The author(s) received no financial support for the research, authorship, and/or publication of this article.
Refreneces: 1. 2. 3. 4. 5. 6. 7. 8. 9.
10.
Danison, A.P., et al., Adult Thoracic Intradural Exophytic Mature Teratoma: Case Report and Literature Review. Asian J Neurosurg, 2018. 13(4): p. 1182-1185. Choi, J.S., et al., Dermoid cysts: Epidemiology and diagnostic approach based on clinical experiences. Arch Plast Surg, 2018. 45(6): p. 512-516. Turan, N., et al., Adult Intramedullary Teratoma of the Spinal Cord: A Case Report and Review of Literature. World Neurosurg, 2016. 87: p. 661.e23-30. Jayasuriya, N.S., et al., Malignant transformation of a long-standing submental dermoid cyst to a carcinosarcoma: a case report. J Med Case Rep, 2017. 11(1): p. 11. Mpayo, L.L., et al., Extramedullary spinal teratoma presenting with recurrent aseptic meningitis. Pediatr Neurol, 2014. 50(6): p. 655-7. Pandey, S., et al., Spinal intradural extramedullary mature cystic teratoma in an adult: A rare tumor with review of literature. Asian J Neurosurg, 2015. 10(3): p. 133-7. Sung, K.S., et al., Spinal intradural extramedullary mature cystic teratoma in an adult. J Korean Neurosurg Soc, 2008. 44(5): p. 334-7. Stevens, Q.E., et al., Intradural extramedullary mature cystic teratoma: not only a childhood disease. J Spinal Disord Tech, 2006. 19(3): p. 213-6. Kabbasch, C., et al., Rupture of a spinal dermoid cyst may lead to dissemination and progress of Fatty tissue in the central spinal canal and intracranial subarachnoid space. A case report. Neuroradiol J, 2014. 27(6): p. 759-63. Triantafyllidi, E., et al., Adult intradural extramedullary teratoma of the spinal cord: A case presentation. Clin Neurol Neurosurg, 2018. 175: p. 54-56.
Figure 1: A) The hyperintense mass on the axial T2-weighted image, B&C) hypointense to the cord on the sagittal T1-weighted images with some high signal areas and D) on the myelogram image, an intradural extramedullary lesion with heterogenous signal predominantly on the right side leading to compression and displacement of cauda equine nerve roots toward the left side. Figure 2: Lumps of white-yellowish cheese material in the excised mass, containing hair follicles and fragments of whitish soft tissue. Figure 3 (1&2&3): Histological sections consist of fibrous tissue with squamous epithelium and hair shafts.
ACKNOWLEDGMENT: We would like to thank the patient who consented to this publication. Declaration of Conflicting Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. Funding: The author(s) received no financial support for the research, authorship, and/or publication of this article.
MRI: Magnetic resonance imaging H&E: Haematoxylin & Eosin