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Rare Case of Giant Supratentorial Dermoid Cyst
Journal Pre-proof A rare case of a giant supratentorial dermoid cyst – case report Louis Obled, MD, Iulia Peciu-Florianu, MD, Romain Perbet, MD, Quentin VannodMichel, MD, Nicolas Reyns, MD PhD PII:
S1878-8750(19)33035-9
DOI:
https://doi.org/10.1016/j.wneu.2019.12.007
Reference:
WNEU 13854
To appear in:
World Neurosurgery
Received Date: 25 September 2019 Revised Date:
1 December 2019
Accepted Date: 2 December 2019
Please cite this article as: Obled L, Peciu-Florianu I, Perbet R, Vannod-Michel Q, Reyns N, A rare case of a giant supratentorial dermoid cyst – case report, World Neurosurgery (2020), doi: https:// doi.org/10.1016/j.wneu.2019.12.007. 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 Elsevier Inc. All rights reserved.
A rare case of a giant supratentorial dermoid cyst – case report
Authors: Louis Obled1,MD, Iulia Peciu-Florianu1,MD, Romain Perbet2,MD, Quentin VannodMichel3,MD, Nicolas Reyns1, MD PhD
Affiliations: 1
Service of Neurosurgery, Department of Neurosciences, CHU Lille, France
2
Service of Pathologic Anatomy, Pathology Institute, CHU Lille, France
3
Service of Neuroradiology, Department of Radiology, CHU Lille, France
Corresponding author: Iulia Peciu-Florianu 1, rue Emile Laine 59000, Lille, France [email protected]
Keywords: dermoid cyst, supratentorial, convexity
Short title: Rare giant convexity dermoid cyst
CRediT author statement: Obled: Conceptualization, Writing – Original Draft; Peciu-Florianu: Conceptualization; Writing – Original Draft; Writing – Review&Editing, Visualization; Perbet: Resources, Writing – Original Draft; Vannod-Michel: Resources, Writing – Original Draft; Reyns: Supervision
Abstract
Background: Dermoid cysts are benign, congenital rare lesions, frequently occurring in or near the midline. Rare localizations and variable radiologic findings have been described but remain exceptional. Case description: The authors present a rare case of a giant temporo-parietal dermoid cyst in an adult female, paucisymptomatic. No sign of rupture was identified, with a clinical presentation suggesting a slow-growing lesion. Gross total excision was performed with a favorable clinical and radiological outcome. Conclusion: Rare localizations previously published in the literature are discussed for dermoid cysts. To our best knowledge, this is the first giant dermoid cyst presenting with this localization.
Background:
Dermoid cysts are uncommon benign extra-axial lesions, occurring in 0.5-1% of all intracranial primary lesions, from entrapped ectoderm during embryogenesis, within the developing neural tube1. Usual localization remains in or near midline, specifically in the sellar, frontal and posterior fossa regions2, 3. Rare localizations have been described, at the Sylvian fissure level3, 4 or infratentorial, prepontine or in the interpeduncular fossa5. Usual management involves maximal resection or gross total resection if safely possible. Low recurrence rates have been described6, with favorable outcomes and very frequent symptomatic improvement. Clinical exacerbation can often be seen after cyst rupture, with aseptic meningitis and symptoms depending on lesion location7, 8.
Case description:
A 54-year old female presented with a three-week history of headache, asthenia and a left visual field defect. Her medical history was notable for pulmonary embolism secondary to a factor V Leiden mutation.
On neurological examination no motor or sensitive deficit was found. Only a homolateral left hemianopsia was noted. The patient was right-handed, without any anosognosia. A dressing apraxia could be identified during daily activities.
A non-enhanced CT scan was performed (fig. 1), showing a hypodense lesion, with a large bone attachment to the mastoid. Capsular calcifications were also identified, in a digitiform pattern.
A supplementary cerebral MRI (fig. 2) was then performed, revealing a large multilobulated and well defined extra-axial lesion, starting at the temporal base and displacing the frontal and parietal lobes. The lesion appeared heterogenous on T2-FLAIR sequence with mild enhancement of the capsule, without hyperperfusion. Fatty elements in high signal on T1 sequence were described.
A surgical excision was planned, using a large fronto-temporo-parietal craniotomy. The lesion was resected using a piecemeal technique, with careful dissection of its capsule from the surrounding brain parenchyma. Calcifications of the cystic wall were observed as seen on the CT-scan towards the temporo-mastoid region, in a digitiform pattern. Visually, the lesion presented as a sebaceous cyst, containing hair follicles and calcifications (Fig. 3).
Postoperatively, the patient was observed in the neuro-ICU for the first 24 hours then transferred to the neurosurgical floor, without any postoperative neurological deficit. The preoperative homolateral left hemianopsia seemed stable. She was discharged home day 5 postoperative.
Histopathology confirmed the dermoid cyst, detailing the characteristic pilosebaceous glands with associated hair follicles and trabecular bone tissue (Fig. 4).
The patient benefitted from a clinical and radiological follow-up in our outpatient clinic.
A control cerebral MRI was obtained at three months postoperative, showing no residual or recurrent lesion (Fig. 5). Respecting our center’s protocol, a yearly clinical and radiological follow-up was scheduled.
Discussion
Dermoid cysts are benign, slow-growing lesions, arising most frequently in the midline suprasellar region, posterior fossa or the spine5, 9. Lateralized lesions are rarely described but can occur, with various clinical presentations. The described case shows that large volumes can be reached for this non-midline, slow-growing lesion, before any clinical signs can be identified.
As previously published, acute signs are usually related to dermoid cyst rupture, either spontaneously10 or post-traumatic11, 12, with symptoms of chemical meningitis due to the dissemination of cystic components into the subarachnoid space or even the ventricles. In our patient, no signs of rupture were identified. Adjacent brain parenchyma compression was responsible for the homolateral left hemianopsia and dressing apraxia. Surgical excision was offered and performed in a semi-urgent setting, taking into account the large tumor volume and important mass effect.
Imaging techniques are paramount in the differential diagnosis for unusual localizations of dermoid cysts. CT confirmed a hypodense lesion due to fat components with associated calcifications, while cerebral MRI showed a high T1 signal with heterogenous T2-FLAIR hypersignal. These aspects have been consistently reported for unruptured dermoid cysts9, 13, 14
, indicating a highly precise diagnosis even for unusual localizations. In our case, even
with suggestive imaging features, final diagnosis was confirmed by histopathology, after gross total excision.
Conclusion:
There is little literature concerning non-midline dermoid cyst localizations. A rare case of supratentorial dermoid cyst is described, with detailed presentation of preoperative imaging
and surgical management. Gross total excision remains the gold standard, allowing a rapid decompression of the brain parenchyma, with complete clinical recovery and discharge to the patient’s home.
1. 2.
3.
4. 5.
6. 7. 8.
9. 10. 11.
12. 13. 14.
Khalid S, Ruge J. Considerations in the management of congenital cranial dermoid cysts. J Neurosurg Pediatr. 2017;20:30-34 Liu JK, Gottfried ON, Salzman KL, Schmidt RH, Couldwell WT. Ruptured intracranial dermoid cysts: Clinical, radiographic, and surgical features. Neurosurgery. 2008;62:377-384; discussion 384 Garces J, Mathkour M, Beard B, Sulaiman OA, Ware ML. Insular and sylvian fissure dermoid cyst with giant cell reactivity: Case report and review of literature. World Neurosurg. 2016;93:491 e491-495 Anand D, Aggarwal S, Soin D, Garg R. Sylvian fissure dermoid cyst - a rare case. J Clin Diagn Res. 2014;8:RD01-02 Chung LK, Lagman C, Duong C, Nagasawa DT, Tucker AM, Yong WH, et al. Dermoid cyst of the prepontine cistern and meckel's cave: Illustrative case and systematic review. J Neurol Surg B Skull Base. 2018;79:139-150 Park SK, Cho KG. Recurrent intracranial dermoid cyst after subtotal removal of traumatic rupture. Clin Neurol Neurosurg. 2012;114:421-424 Stendel R, Pietila TA, Lehmann K, Kurth R, Suess O, Brock M. Ruptured intracranial dermoid cysts. Surg Neurol. 2002;57:391-398; discussion 398 Shashidhar A, Sadashiva N, Prabhuraj AR, Narasingha Rao K, Tiwari S, Saini J, et al. Ruptured intracranial dermoid cysts: A retrospective institutional review. J Clin Neurosci. 2019;67:172-177 Jackow J, Tse G, Martin A, Sasiadek M, Romanowski C. Ruptured intracranial dermoid cysts: A pictorial review. Pol J Radiol. 2018;83:e465-e470 El-Bahy K, Kotb A, Galal A, El-Hakim A. Ruptured intracranial dermoid cysts. Acta Neurochir (Wien). 2006;148:457-462 Esquenazi Y, Kerr K, Bhattacharjee MB, Tandon N. Traumatic rupture of an intracranial dermoid cyst: Case report and literature review. Surg Neurol Int. 2013;4:80 Kim IY, Jung S, Jung TY, Kang SS, Kim TS. Traumatic rupture of an intracranial dermoid cyst. J Clin Neurosci. 2008;15:469-471 Osborn AG, Preece MT. Intracranial cysts: Radiologic-pathologic correlation and imaging approach. Radiology. 2006;239:650-664 Orakcioglu B, Halatsch ME, Fortunati M, Unterberg A, Yonekawa Y. Intracranial dermoid cysts: Variations of radiological and clinical features. Acta Neurochir (Wien). 2008;150:1227-1234; discussion 1234
FIGURES
Figure 1: Cerebral CT-scan demonstrating a large hypodense lesion in the coronal plane (A and B), with capsular calcifications appearing from the internal lamina of the mastoid (C)
Figure 2: Cerebral axial MRI showing a large fronto-temporo-parietal convexity lesion, extraaxial, heterogenous on T2-FLAIR (A), with high signal on T1 and mild enhancement (B and C). No hyperperfusion was noted (D).
Figure 3: Intraoperative views, during intracapsular excision (A) and parenchymal decompression (B)
Figure 4: Histopathology demonstrating a cystic tumor, composed of a range of somatic tissues: skin, skin appendages (A), and bone. There were no criteria for malignancy or immaturity.
Figure 5: Postoperative cerebral axial MRI, confirming absence of recurrence, with improvement of global mass effect on the brain parenchyma
ABBREVIATIONS
CT – computer tomography
MRI – magnetic resonance imaging
Conflict of interest
I, Iulia Peciu-Florianu, corresponding author, certify that there are NO conflict of interests for any of the authors of the manuscript “A rare case of a giant supratentorial dermoid cyst – case report”
S1878-8750(19)33035-9
DOI:
https://doi.org/10.1016/j.wneu.2019.12.007
Reference:
WNEU 13854
To appear in:
World Neurosurgery
Received Date: 25 September 2019 Revised Date:
1 December 2019
Accepted Date: 2 December 2019
Please cite this article as: Obled L, Peciu-Florianu I, Perbet R, Vannod-Michel Q, Reyns N, A rare case of a giant supratentorial dermoid cyst – case report, World Neurosurgery (2020), doi: https:// doi.org/10.1016/j.wneu.2019.12.007. 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 Elsevier Inc. All rights reserved.
A rare case of a giant supratentorial dermoid cyst – case report
Authors: Louis Obled1,MD, Iulia Peciu-Florianu1,MD, Romain Perbet2,MD, Quentin VannodMichel3,MD, Nicolas Reyns1, MD PhD
Affiliations: 1
Service of Neurosurgery, Department of Neurosciences, CHU Lille, France
2
Service of Pathologic Anatomy, Pathology Institute, CHU Lille, France
3
Service of Neuroradiology, Department of Radiology, CHU Lille, France
Corresponding author: Iulia Peciu-Florianu 1, rue Emile Laine 59000, Lille, France [email protected]
Keywords: dermoid cyst, supratentorial, convexity
Short title: Rare giant convexity dermoid cyst
CRediT author statement: Obled: Conceptualization, Writing – Original Draft; Peciu-Florianu: Conceptualization; Writing – Original Draft; Writing – Review&Editing, Visualization; Perbet: Resources, Writing – Original Draft; Vannod-Michel: Resources, Writing – Original Draft; Reyns: Supervision
Abstract
Background: Dermoid cysts are benign, congenital rare lesions, frequently occurring in or near the midline. Rare localizations and variable radiologic findings have been described but remain exceptional. Case description: The authors present a rare case of a giant temporo-parietal dermoid cyst in an adult female, paucisymptomatic. No sign of rupture was identified, with a clinical presentation suggesting a slow-growing lesion. Gross total excision was performed with a favorable clinical and radiological outcome. Conclusion: Rare localizations previously published in the literature are discussed for dermoid cysts. To our best knowledge, this is the first giant dermoid cyst presenting with this localization.
Background:
Dermoid cysts are uncommon benign extra-axial lesions, occurring in 0.5-1% of all intracranial primary lesions, from entrapped ectoderm during embryogenesis, within the developing neural tube1. Usual localization remains in or near midline, specifically in the sellar, frontal and posterior fossa regions2, 3. Rare localizations have been described, at the Sylvian fissure level3, 4 or infratentorial, prepontine or in the interpeduncular fossa5. Usual management involves maximal resection or gross total resection if safely possible. Low recurrence rates have been described6, with favorable outcomes and very frequent symptomatic improvement. Clinical exacerbation can often be seen after cyst rupture, with aseptic meningitis and symptoms depending on lesion location7, 8.
Case description:
A 54-year old female presented with a three-week history of headache, asthenia and a left visual field defect. Her medical history was notable for pulmonary embolism secondary to a factor V Leiden mutation.
On neurological examination no motor or sensitive deficit was found. Only a homolateral left hemianopsia was noted. The patient was right-handed, without any anosognosia. A dressing apraxia could be identified during daily activities.
A non-enhanced CT scan was performed (fig. 1), showing a hypodense lesion, with a large bone attachment to the mastoid. Capsular calcifications were also identified, in a digitiform pattern.
A supplementary cerebral MRI (fig. 2) was then performed, revealing a large multilobulated and well defined extra-axial lesion, starting at the temporal base and displacing the frontal and parietal lobes. The lesion appeared heterogenous on T2-FLAIR sequence with mild enhancement of the capsule, without hyperperfusion. Fatty elements in high signal on T1 sequence were described.
A surgical excision was planned, using a large fronto-temporo-parietal craniotomy. The lesion was resected using a piecemeal technique, with careful dissection of its capsule from the surrounding brain parenchyma. Calcifications of the cystic wall were observed as seen on the CT-scan towards the temporo-mastoid region, in a digitiform pattern. Visually, the lesion presented as a sebaceous cyst, containing hair follicles and calcifications (Fig. 3).
Postoperatively, the patient was observed in the neuro-ICU for the first 24 hours then transferred to the neurosurgical floor, without any postoperative neurological deficit. The preoperative homolateral left hemianopsia seemed stable. She was discharged home day 5 postoperative.
Histopathology confirmed the dermoid cyst, detailing the characteristic pilosebaceous glands with associated hair follicles and trabecular bone tissue (Fig. 4).
The patient benefitted from a clinical and radiological follow-up in our outpatient clinic.
A control cerebral MRI was obtained at three months postoperative, showing no residual or recurrent lesion (Fig. 5). Respecting our center’s protocol, a yearly clinical and radiological follow-up was scheduled.
Discussion
Dermoid cysts are benign, slow-growing lesions, arising most frequently in the midline suprasellar region, posterior fossa or the spine5, 9. Lateralized lesions are rarely described but can occur, with various clinical presentations. The described case shows that large volumes can be reached for this non-midline, slow-growing lesion, before any clinical signs can be identified.
As previously published, acute signs are usually related to dermoid cyst rupture, either spontaneously10 or post-traumatic11, 12, with symptoms of chemical meningitis due to the dissemination of cystic components into the subarachnoid space or even the ventricles. In our patient, no signs of rupture were identified. Adjacent brain parenchyma compression was responsible for the homolateral left hemianopsia and dressing apraxia. Surgical excision was offered and performed in a semi-urgent setting, taking into account the large tumor volume and important mass effect.
Imaging techniques are paramount in the differential diagnosis for unusual localizations of dermoid cysts. CT confirmed a hypodense lesion due to fat components with associated calcifications, while cerebral MRI showed a high T1 signal with heterogenous T2-FLAIR hypersignal. These aspects have been consistently reported for unruptured dermoid cysts9, 13, 14
, indicating a highly precise diagnosis even for unusual localizations. In our case, even
with suggestive imaging features, final diagnosis was confirmed by histopathology, after gross total excision.
Conclusion:
There is little literature concerning non-midline dermoid cyst localizations. A rare case of supratentorial dermoid cyst is described, with detailed presentation of preoperative imaging
and surgical management. Gross total excision remains the gold standard, allowing a rapid decompression of the brain parenchyma, with complete clinical recovery and discharge to the patient’s home.
1. 2.
3.
4. 5.
6. 7. 8.
9. 10. 11.
12. 13. 14.
Khalid S, Ruge J. Considerations in the management of congenital cranial dermoid cysts. J Neurosurg Pediatr. 2017;20:30-34 Liu JK, Gottfried ON, Salzman KL, Schmidt RH, Couldwell WT. Ruptured intracranial dermoid cysts: Clinical, radiographic, and surgical features. Neurosurgery. 2008;62:377-384; discussion 384 Garces J, Mathkour M, Beard B, Sulaiman OA, Ware ML. Insular and sylvian fissure dermoid cyst with giant cell reactivity: Case report and review of literature. World Neurosurg. 2016;93:491 e491-495 Anand D, Aggarwal S, Soin D, Garg R. Sylvian fissure dermoid cyst - a rare case. J Clin Diagn Res. 2014;8:RD01-02 Chung LK, Lagman C, Duong C, Nagasawa DT, Tucker AM, Yong WH, et al. Dermoid cyst of the prepontine cistern and meckel's cave: Illustrative case and systematic review. J Neurol Surg B Skull Base. 2018;79:139-150 Park SK, Cho KG. Recurrent intracranial dermoid cyst after subtotal removal of traumatic rupture. Clin Neurol Neurosurg. 2012;114:421-424 Stendel R, Pietila TA, Lehmann K, Kurth R, Suess O, Brock M. Ruptured intracranial dermoid cysts. Surg Neurol. 2002;57:391-398; discussion 398 Shashidhar A, Sadashiva N, Prabhuraj AR, Narasingha Rao K, Tiwari S, Saini J, et al. Ruptured intracranial dermoid cysts: A retrospective institutional review. J Clin Neurosci. 2019;67:172-177 Jackow J, Tse G, Martin A, Sasiadek M, Romanowski C. Ruptured intracranial dermoid cysts: A pictorial review. Pol J Radiol. 2018;83:e465-e470 El-Bahy K, Kotb A, Galal A, El-Hakim A. Ruptured intracranial dermoid cysts. Acta Neurochir (Wien). 2006;148:457-462 Esquenazi Y, Kerr K, Bhattacharjee MB, Tandon N. Traumatic rupture of an intracranial dermoid cyst: Case report and literature review. Surg Neurol Int. 2013;4:80 Kim IY, Jung S, Jung TY, Kang SS, Kim TS. Traumatic rupture of an intracranial dermoid cyst. J Clin Neurosci. 2008;15:469-471 Osborn AG, Preece MT. Intracranial cysts: Radiologic-pathologic correlation and imaging approach. Radiology. 2006;239:650-664 Orakcioglu B, Halatsch ME, Fortunati M, Unterberg A, Yonekawa Y. Intracranial dermoid cysts: Variations of radiological and clinical features. Acta Neurochir (Wien). 2008;150:1227-1234; discussion 1234
FIGURES
Figure 1: Cerebral CT-scan demonstrating a large hypodense lesion in the coronal plane (A and B), with capsular calcifications appearing from the internal lamina of the mastoid (C)
Figure 2: Cerebral axial MRI showing a large fronto-temporo-parietal convexity lesion, extraaxial, heterogenous on T2-FLAIR (A), with high signal on T1 and mild enhancement (B and C). No hyperperfusion was noted (D).
Figure 3: Intraoperative views, during intracapsular excision (A) and parenchymal decompression (B)
Figure 4: Histopathology demonstrating a cystic tumor, composed of a range of somatic tissues: skin, skin appendages (A), and bone. There were no criteria for malignancy or immaturity.
Figure 5: Postoperative cerebral axial MRI, confirming absence of recurrence, with improvement of global mass effect on the brain parenchyma
ABBREVIATIONS
CT – computer tomography
MRI – magnetic resonance imaging
Conflict of interest
I, Iulia Peciu-Florianu, corresponding author, certify that there are NO conflict of interests for any of the authors of the manuscript “A rare case of a giant supratentorial dermoid cyst – case report”