[Downloaded free from http://www.neurologyindia.com on Thursday, November 13, 2014, IP: 202.177.173.189] || Click here to download free Android application for journal
Neuroimage
White epidermoid at the foramen magnum Raghvendra Ramdasi, Amit Mahore, Aadil Chagla, Juhi Kawale1 Departments of Neurosurgery, and 1Medicine, King Edward Memorial Hospital and Seth Gordhandas Sunderdas Medical College, Parel, Mumbai, Maharashtra, India A 20‑year‑old female presented with episodic occipital headaches and imbalance on walking of 2 years duration. Neurological examination showed hyper‑reflexia of all four limbs. Computed tomographic (CT) brain scan showed a hyperdense lesion in premedullary cistern [Figure 1a]. Magnetic resonance imaging (MRI) of brain revealed a well‑defined extra‑axial lesion, 13 × 25 × 14 mm in dimensions with marked hyperintensity on T1‑weighted images (T1WI) and hypointensity on T2‑weighted image (T2WI). The lesion did not show any restriction of diffusion on diffusion‑weighted imaging (DWI) and no enhancement on giving gadolinium [Figure 1b‑g]. Gradient echo (GRE) sequence of MR imaging did not show any blooming [Figure 1h]. Based on the imaging features, a diagnosis of a high protein‑containing cystic lesion was postulated. During surgery by suboccipital approach, a thin‑walled extra axial cyst containing pearly and pultaceous material with viscous fluid was excised. There was no evidence of calcification. Patient was relieved of her symptoms after the surgery and was well at follow‑up of 2 years. On histopathological examination, the cyst wall consisted of lamellated keratinizing squamous epithelium suggestive of epidermoid cyst [Figure 2a and b]. Intracranial epidermoids are rare lesions accounting for 1% of all intracranial tumors.[1] Epidermoid tumors typically have long T1 and T2 relaxation times due to the presence of cholesterol in a solid crystalline state and keratin within the tumor. These are hypointense on T1‑weighted, hyperintense on T2‑weighted MR image and have no enhancement on post‑contrast MR Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.144518
Neurology India | Sep-Oct 2014 | Vol 62 | Issue 5
images. Epidermoid cysts are hypodense on CT scan. These typically restrict diffusion on DWI. In our case, the lesion was hyperdense on CT scan, hyperintense on T1‑weighted image, hypointense on T2‑weighted image, not enhancing by gadolinium and was not restricting diffusion on DWI. Epidermoid tumors showing unusual signal intensity changes have been reported. These lesions are referred to as white epidermoid when these have short T1 values and fatty attenuation on CT as in our case. Their variable appearance can be linked to the biochemical nature and viscosity of the contents. It can show hyperintensity on T1‑ and T2‑weighted MR images, caused by a semi‑liquid cystic content with high protein concentration mixed with high triglycerides. It also can have high signal intensity on T1‑weighted and low signal intensity on T2‑weighted images, caused by even thicker toffee‑like content with a combination of high protein content and high viscosity as in our case. An epidermoid tumor can show low signal intensity on both T1‑ and T2‑weighted images, with a usual composition of epithelial debris, crystals of cholesterol and keratin. Variable signal on T1‑ and T2‑weighted images may be because of recurrent intracystic hemorrhage and granulation tissue, often documented on histology.[2,3] In our case histology did not reveal any hemosiderin‑rich macrophages or granulation tissue. High protein content and high viscosity may be the reason of the unusual radiological picture. The differential diagnosis of such radiological picture in the foramen magnum area can be neuroenteric cyst, post‑hemorrhagic cystic mass as cystic meningioma and arachnoid cyst.[2] Ectopic Rathke’s cleft cyst constitute a rare possiblity.[4] Neuroenteric cysts are T2 hyperintense lesions, meningioma enhance on giving contrast and have dural attachments. The arachnoid cyst and Rathke’s cleft cyst have smooth margins unlike epidermoids.[2,4] After first reporting by Braun et al., in 1977, few cases of white epidermoid have 577
[Downloaded free from http://www.neurologyindia.com on Thursday, November 13, 2014, IP: 202.177.173.189] || Click here to download free Android application for journal Ramdasi, et al.: White epidermoid in the premedullary cistern
5DGLRORJLFDOO\DW\SLFDOHSLGHUPRLGF\VW
+\SHULQWHQVHRQ 7: 7:
+\SHULQWHQVHRQ7: +\SRLQWHQVHRQ7:
+\SRLQWHQVHRQ 7: 7:
9DULDEOHLQWHQVLWLHV RQ7: 7:
+LJKSURWHLQ+LJKSURWHLQ(SLWKHOLDOGHEULV5HFXUUHQWEOHHGRI +LJKOLSLG +LJKYLVFRVLW\&KROHVWHUROGLIIHUHQWFKURQRORJ\ 6HPLYLVFRXV .HUDWLQ ,QIODPPDWLRQ
a
e
d
c
b
f
g
h
Figure 1: (a) Axial image of plain CT scan reveals a hyperdense lesion in the premedullary cistern (b) Axial and Sagittal (d) Images of plain T1-weighted MR scan showing a hyperintense lesion in the premedullary cistern without contrast enhancement (c). T2 fast spin echo axial (e) and Fluid Attenuated Inversion Recovery (FLAIR) axial MR (f) Images showing homogenously decreased T2 signal; (g) DWI does not reveal restricted diffusion; (h) GRE sequence of MR showing absence of blooming
a
b
Figure 2: Photomicrograph showing the cyst wall, which is lined by keratinizing squamous epithelium and filled with lamellated keratinous debris ((a) H and E ×10. (b) H and E ×40)
been reported in the literature, most of which are in the cerebellopontine angle and few in the supratentorial 578
region.[1‑3] To the best of our knowledge, this is the first case of white epidermoid in the premedullary cistern near foramen magnum in the midline. The capsule of the epidermoid cyst often adheres to the surrounding structures. Accurate preoperative diagnosis can help prevent inadvertent damage to the surrounding vital structures as in our case and chemical meningitis due to spillage during surgery. Address of correspondence: Dr. Raghvendra Ramdasi, Department of Neurosurgery, King Edward VII Memorial Hospital, Parel, Mumbai ‑ 400 012, India. E‑mail: [email protected] Neurology India | Sep-Oct 2014 | Vol 62 | Issue 5
[Downloaded free from http://www.neurologyindia.com on Thursday, November 13, 2014, IP: 202.177.173.189] || Click here to download free Android application for journal Ramdasi, et al.: White epidermoid in the premedullary cistern
References 1. Puranik A, Sankhe S, Goel N, Mahore A. Cerebral shading sign in a giant intraparenchymal white epidermoid. Neurol India 2012;60:265‑6. 2. Chen CY, Wong JS, Hsieh SC, Chu JS, Chan WP. Intracranial epidermoid cyst with hemorrhage: MR imaging findings. AJNR Am J Neuroradiol 2006;27:427‑9. 3. Timmer FA, Sluzewski M, Treskes M, van Rooij WJ, Teepen JL,
Neurology India | Sep-Oct 2014 | Vol 62 | Issue 5
4.
Wijnalda D. Chemical analysis of an epidermoid cyst with unusual CT and MR characteristics. AJNR Am J Neuroradiol 1998;19:1111‑2. Kim E. A Case of Ectopic Rathke’s Cleft Cyst in the Prepontine Cistern. J Korean Neurosurg Soc 2012;52:152‑5.
How to cite this article: Ramdasi R, Mahore A, Chagla A, Kawale J. White epidermoid at the foramen magnum. Neurol India 2014;62:577-9. Source of Support: Nil, Conflict of Interest: None declared.
579
Copyright of Neurology India is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Neuroimage
White epidermoid at the foramen magnum Raghvendra Ramdasi, Amit Mahore, Aadil Chagla, Juhi Kawale1 Departments of Neurosurgery, and 1Medicine, King Edward Memorial Hospital and Seth Gordhandas Sunderdas Medical College, Parel, Mumbai, Maharashtra, India A 20‑year‑old female presented with episodic occipital headaches and imbalance on walking of 2 years duration. Neurological examination showed hyper‑reflexia of all four limbs. Computed tomographic (CT) brain scan showed a hyperdense lesion in premedullary cistern [Figure 1a]. Magnetic resonance imaging (MRI) of brain revealed a well‑defined extra‑axial lesion, 13 × 25 × 14 mm in dimensions with marked hyperintensity on T1‑weighted images (T1WI) and hypointensity on T2‑weighted image (T2WI). The lesion did not show any restriction of diffusion on diffusion‑weighted imaging (DWI) and no enhancement on giving gadolinium [Figure 1b‑g]. Gradient echo (GRE) sequence of MR imaging did not show any blooming [Figure 1h]. Based on the imaging features, a diagnosis of a high protein‑containing cystic lesion was postulated. During surgery by suboccipital approach, a thin‑walled extra axial cyst containing pearly and pultaceous material with viscous fluid was excised. There was no evidence of calcification. Patient was relieved of her symptoms after the surgery and was well at follow‑up of 2 years. On histopathological examination, the cyst wall consisted of lamellated keratinizing squamous epithelium suggestive of epidermoid cyst [Figure 2a and b]. Intracranial epidermoids are rare lesions accounting for 1% of all intracranial tumors.[1] Epidermoid tumors typically have long T1 and T2 relaxation times due to the presence of cholesterol in a solid crystalline state and keratin within the tumor. These are hypointense on T1‑weighted, hyperintense on T2‑weighted MR image and have no enhancement on post‑contrast MR Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.144518
Neurology India | Sep-Oct 2014 | Vol 62 | Issue 5
images. Epidermoid cysts are hypodense on CT scan. These typically restrict diffusion on DWI. In our case, the lesion was hyperdense on CT scan, hyperintense on T1‑weighted image, hypointense on T2‑weighted image, not enhancing by gadolinium and was not restricting diffusion on DWI. Epidermoid tumors showing unusual signal intensity changes have been reported. These lesions are referred to as white epidermoid when these have short T1 values and fatty attenuation on CT as in our case. Their variable appearance can be linked to the biochemical nature and viscosity of the contents. It can show hyperintensity on T1‑ and T2‑weighted MR images, caused by a semi‑liquid cystic content with high protein concentration mixed with high triglycerides. It also can have high signal intensity on T1‑weighted and low signal intensity on T2‑weighted images, caused by even thicker toffee‑like content with a combination of high protein content and high viscosity as in our case. An epidermoid tumor can show low signal intensity on both T1‑ and T2‑weighted images, with a usual composition of epithelial debris, crystals of cholesterol and keratin. Variable signal on T1‑ and T2‑weighted images may be because of recurrent intracystic hemorrhage and granulation tissue, often documented on histology.[2,3] In our case histology did not reveal any hemosiderin‑rich macrophages or granulation tissue. High protein content and high viscosity may be the reason of the unusual radiological picture. The differential diagnosis of such radiological picture in the foramen magnum area can be neuroenteric cyst, post‑hemorrhagic cystic mass as cystic meningioma and arachnoid cyst.[2] Ectopic Rathke’s cleft cyst constitute a rare possiblity.[4] Neuroenteric cysts are T2 hyperintense lesions, meningioma enhance on giving contrast and have dural attachments. The arachnoid cyst and Rathke’s cleft cyst have smooth margins unlike epidermoids.[2,4] After first reporting by Braun et al., in 1977, few cases of white epidermoid have 577
[Downloaded free from http://www.neurologyindia.com on Thursday, November 13, 2014, IP: 202.177.173.189] || Click here to download free Android application for journal Ramdasi, et al.: White epidermoid in the premedullary cistern
5DGLRORJLFDOO\DW\SLFDOHSLGHUPRLGF\VW
+\SHULQWHQVHRQ 7: 7:
+\SHULQWHQVHRQ7: +\SRLQWHQVHRQ7:
+\SRLQWHQVHRQ 7: 7:
9DULDEOHLQWHQVLWLHV RQ7: 7:
+LJKSURWHLQ+LJKSURWHLQ(SLWKHOLDOGHEULV5HFXUUHQWEOHHGRI +LJKOLSLG +LJKYLVFRVLW\&KROHVWHUROGLIIHUHQWFKURQRORJ\ 6HPLYLVFRXV .HUDWLQ ,QIODPPDWLRQ
a
e
d
c
b
f
g
h
Figure 1: (a) Axial image of plain CT scan reveals a hyperdense lesion in the premedullary cistern (b) Axial and Sagittal (d) Images of plain T1-weighted MR scan showing a hyperintense lesion in the premedullary cistern without contrast enhancement (c). T2 fast spin echo axial (e) and Fluid Attenuated Inversion Recovery (FLAIR) axial MR (f) Images showing homogenously decreased T2 signal; (g) DWI does not reveal restricted diffusion; (h) GRE sequence of MR showing absence of blooming
a
b
Figure 2: Photomicrograph showing the cyst wall, which is lined by keratinizing squamous epithelium and filled with lamellated keratinous debris ((a) H and E ×10. (b) H and E ×40)
been reported in the literature, most of which are in the cerebellopontine angle and few in the supratentorial 578
region.[1‑3] To the best of our knowledge, this is the first case of white epidermoid in the premedullary cistern near foramen magnum in the midline. The capsule of the epidermoid cyst often adheres to the surrounding structures. Accurate preoperative diagnosis can help prevent inadvertent damage to the surrounding vital structures as in our case and chemical meningitis due to spillage during surgery. Address of correspondence: Dr. Raghvendra Ramdasi, Department of Neurosurgery, King Edward VII Memorial Hospital, Parel, Mumbai ‑ 400 012, India. E‑mail: [email protected] Neurology India | Sep-Oct 2014 | Vol 62 | Issue 5
[Downloaded free from http://www.neurologyindia.com on Thursday, November 13, 2014, IP: 202.177.173.189] || Click here to download free Android application for journal Ramdasi, et al.: White epidermoid in the premedullary cistern
References 1. Puranik A, Sankhe S, Goel N, Mahore A. Cerebral shading sign in a giant intraparenchymal white epidermoid. Neurol India 2012;60:265‑6. 2. Chen CY, Wong JS, Hsieh SC, Chu JS, Chan WP. Intracranial epidermoid cyst with hemorrhage: MR imaging findings. AJNR Am J Neuroradiol 2006;27:427‑9. 3. Timmer FA, Sluzewski M, Treskes M, van Rooij WJ, Teepen JL,
Neurology India | Sep-Oct 2014 | Vol 62 | Issue 5
4.
Wijnalda D. Chemical analysis of an epidermoid cyst with unusual CT and MR characteristics. AJNR Am J Neuroradiol 1998;19:1111‑2. Kim E. A Case of Ectopic Rathke’s Cleft Cyst in the Prepontine Cistern. J Korean Neurosurg Soc 2012;52:152‑5.
How to cite this article: Ramdasi R, Mahore A, Chagla A, Kawale J. White epidermoid at the foramen magnum. Neurol India 2014;62:577-9. Source of Support: Nil, Conflict of Interest: None declared.
579
Copyright of Neurology India is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
