Novel Insights from Clinical Practice Pediatr Neurosurg 2013;49:311–315 DOI: 10.1159/000366452
Received: June 15, 2014 Accepted after revision: July 29, 2014 Published online: November 1, 2014
Spinal Clear Cell Meningioma in a 3-Year-Old: A Case Report James A. Balogun a William Halliday c Eric Bouffet b Abhaya V. Kulkarni a Divisions of a Neurosurgery, b Neuro-Oncology and c Pathology, The Hospital for Sick Children, Toronto, Ont., Canada
Established Facts • Meningiomas are less common in children. • Clear cell meningioma (CCM) is a rare, aggressive variant of meningioma with limited reports in the literature. • There is no consensus on the use of adjuvant radiotherapy.
Novel Insights • Close clinical and radiological imaging is warranted even when gross total resection is achieved in intraspinal CCM. Reoperation should be attempted in recurrence and adjuvant radiotherapy in incomplete resection. • We review the literature on this disease entity.
Key Words Clear cell meningioma · Spine · Aggressive variant · Early recurrence
Abstract Clear cell meningioma (CCM) is an aggressive meningioma variant with a tendency to early recurrence posing a challenge to its treatment. Although spinal meningiomas are uncommon in children, this rare entity has been described as the most common variant of spinal meningiomas in the pediatric age group. We present the case of a 3-year-old with a confirmed lumbar spine CCM and discuss the problems encountered in the management of this disease.
Introduction
Clear cell meningioma (CCM) is a distinct variant of meningioma with unique histological features first described by Scheithauer [1]. It is the most common variant of pediatric spinal meningiomas [2–6]. The management of this tumor poses a great challenge due to its high recurrence rate within a short period despite gross total tumor excision [2–4, 7–10]. The role of adjuvant radiotherapy remains controversial [3, 11, 12]. We add to the literature on this rare tumor by reporting a case of a 3-year-old boy with this unusual pathology.
© 2014 S. Karger AG, Basel © 2014 S. Karger AG, Basel 1016–2291/14/0495–0311$39.50/0 E-Mail [email protected] www.karger.com/pne
Abhaya V. Kulkarni, MD, PhD Division of Neurosurgery, The Hospital for Sick Children, Suite 1503 555 University Avenue Toronto, ON M5G 1X8 (Canada) E-Mail Abhaya.kulkarni @ sickkids.ca
Case Report A 3-year-old boy with a history of speech delay and behavioral problems presented with inability to walk and lower back pain over the previous 6 months. The child had not yet achieved urinary or bowel continence. Examination revealed that he was paraparetic with grade 3 power in most muscle groups of the lower extremities. The patellar and ankle reflexes were absent. The magnetic resonance imaging (MRI; fig. 1a, b) revealed an enhancing soft tissue mass within the lumbar intraspinal canal extending from L2 to L5 with secondary canal widening and posterior remodeling of the lumbar vertebrae. No other spinal or cranial lesions were found on screening. He subsequently had a T12–L5 laminoplasty. The dura was opened and the tumor exposed along its entire craniocaudal length. The cauda equina was observed to be draped over the tumor. Microsurgical excision of the tumor was carried out with intraoperative neuromonitoring. After an initial debulking with the ultrasonic device, a number of nerve roots entangled within the tumor were observed and the left L4 nerve root had to be sacrificed to achieve gross total tumor resection. The histology of the tumor revealed clear cells arranged in lobules and sheets. There was a variable amount of collagen between cells and this appeared to progress to regions with very few tumor cells but lots of intermingled collagen. To a variable degree the tumor cells stained for glycogen. Immunohistochemical studies showed membranous activity for epithelial membrane antigen. The MIB index was 5–10%. Electron microscopy detailed the tumor cell cytoplasmic glycogen, intermediate filaments and cell-tocell junctions (desmosomes). This was representative of a CCM (fig. 2a–c). The immediate postoperative MRI (fig. 3a) confirmed complete tumor excision. The child progressively improved neurologically, eventually becoming self-ambulant and voiding voluntarily. Although he had been scheduled for 3-monthly MRI follow-up examinations, these were delayed due to intercurrent respiratory illness and social issues. He re-presented 9 months after the surgery with occasional falls and clumsiness when walking. Power in most muscle groups was grade 4+ except in the left ankle dorsiflexors, which were grade 1. MRI (fig. 3b) showed a recurrence of the tumor as two homogeneously enhancing masses at the L1–L2 levels. Brain MRI did not show evidence of dissemination. The patient underwent repeat surgical exploration. Only a subtotal excision of the tumor (fig. 3c) could be achieved due to traversing of the cauda equina through the main bulk of the tumor associated with slowing on the somatosensory evoked potentials and motor evoked potentials. Postoperatively, the neurological function remained unchanged. Pathology confirmed recurrence of the CCM. The patient was sent to the radiation oncology service for adjuvant radiotherapy.
Discussion
Meningiomas are uncommon in children and constitute 2% of all meningiomas [13]. Childhood spinal meningiomas form between 4 and 10% of the meningiomas in the pediatric population [14]. Scheithauer [1] described 312
Pediatr Neurosurg 2013;49:311–315 DOI: 10.1159/000366452
a
b
Fig. 1. Preoperative T1 sagittal image without contrast (a) and with contrast (b) showing heterogeneous enhancement.
the histological features of a rare variant of meningioma called CCM, which is classified by the WHO grading as a grade II variant. This entity has been described as the most common subtype in pediatric spinal meningiomas [2–6]. It is reported to occur more in the lumbar region affecting the cauda equina [2–4, 11] compared with other variants of meningiomas that occur in the thoracic and cervical regions [13–16]. The average age in children from case reports with literature review is about 11 years [11], with the youngest reported patient being a 2-monthold child [2]. There is no definitive sex predilection though there are reports of a female predominance [2, 11, 17]. CCM does not have distinguishing clinical features and the imaging characteristics are similar to those of other spinal meningiomas. The unique characteristics of CCM are continually being defined; however, there is a consensus on its more aggressive behavior with a tendency to early recurrence, which can occur as early as 5 months after resection but may also be delayed as much as 5 years with occasional multifocal occurrence despite its benign histological characteristics [3, 4, 7–10]. Liu et al. [2] in their review documented a local recurrence rate of 63% despite gross total tumor excision, with 25% of patients having multiple recurrences at the time of follow-up. Children are noted to have higher recurrence rates and shorter mean time to recurrence [9, 18]. It is intriguing that no factor has been identified that accounts for the high rate of recurBalogun /Halliday /Bouffet /Kulkarni
Color version available online
a
50 μm
50 μm
b
c
Fig. 2. a Masson’s trichrome stain shows the variable amount of collagen between tumor cells, which demonstrate a ‘clear cell’ histology. b Immunohistochemical studies for epithelial membrane antigen show the tumor cells to be reactive. c An electron microscopic picture
shows the tumor cell cytoplasm to contain glycogen and intermediate filaments. The tumor cells have cell-to-cell junctions, and two desmosomes are demonstrated in the center of the picture.
Fig. 3. a T1-weighted image with contrast after gross tumor resection showing surgical changes. b Contrast-enhanced T1 image of the recurrent tumor. c After subtotal resection of the recurrent tumor.
a
b
c
rence in CCMs. There has been a suggestion of an association between tumor recurrence and a high MIB-1 [4], but this is not firmly established [6, 18]. Our patient had an MIB-1 index of 5–10%, but we have no certainty regarding its role in the recurrence. Surgical excision remains the treatment of choice with the aim of achieving a gross total resection. It is also generally agreed upon that adjuvant radiotherapy is an optional treatment modality following incomplete resection. There is, however, no consensus on the role of radiotherapy following gross total tumor excision in this
aggressive tumor with a high propensity for recurrence even after gross tumor resection [2, 19]. The decision is more difficult because of the long-term effect of radiation in children as well as the fact that there have been reports of tumor recurrence despite radiotherapy in some cases [3, 12]. Colen et al. [11] suggest that adjuvant radiotherapy following gross tumor excision is beneficial even with a moderately high MIB-1. The other option, which was utilized in our patient, is close postoperative surveillance with neuroimaging of the whole neuraxis for the early detection of local recurrence and distant metastases. Recur-
Spinal Clear Cell Meningioma in a 3-Year-Old
Pediatr Neurosurg 2013;49:311–315 DOI: 10.1159/000366452
313
rences should be surgically excised with adjuvant radiation in the case of incomplete resection. The recognition of this aggressive meningioma variant is important, prompting a high index of suspicion from the patient’s clinical characteristics and imaging. When confirmed by histology, there should be close postoperative surveillance and adequate parental counseling.
Though Vural et al. [19] did not detect any genomic alteration in their reported case, it may be worthwhile to further probe for peculiar mutations and pathways by genomic sequencing that can be targeted for possible therapeutic intervention, as is being explored in other meningioma variants [20] (table 1) .
Table 1. Summary of the case reports of pediatric spinal CCM
First author [Ref.], Age/sex year
Location
EOR
Adjuvant treatment
Ki67
Time to recurrence/ location
Zorludemir [4], 1995
17 years/F
L4 – L5
GTR
none
NA
no recurrence
9 years/F
L3 –L5
GTR
none
NA
6 months/same location
Matsui [10], 1998
9 years/F
T11 –T12, PR L2, L4 –L 5
none
12%
no recurrence
Cances [21], 1998
10 years/F
L1 –L4
GTR
none
NA
5 months/L1 – L2
GTR and RT
Park [3], 2000
14 months/F
T12–L2
GTR
none
3%+
8 months/cauda equina
GTR
Heth [5], 2000
7 years/F
L4 –L5
GTR
none
NA
no recurrence
Jallo [22], 2001
8 years/F
L3 –L4
GTR
none
NA
6 months/L3 – L5
GTR and RT
C3 – C5*
PR
none
NA
10 weeks, 3 months, 2 years/same location
PR/GTR/GTR, RT and chemotherapy
3 years
5 years
22 months/F
Treatment at recurrence
Follow-up 3 years
GTR
2 years 1 year 8 months NA 13 months 11 months
Carra [23, 24], 2001, 2003
22 months/M T11–L4
GTR
none
NA
5 years/vermis to C2
GTR
Yu [25], 2002
14 months/F
T12–L2
GTR
none
3%
8 months, 7 months/ same location
GTR/GTR and NA RT
Oviedo [17], 2005
7 years/M
L2 – L3
GTR
none
10%
no recurrence
Liu [2], 2005
2 years/M
T10–L1
GTR
none
NA
5 years/T12
Vural [19], 2007
4 years/F
C1 – C2
GTR
none
NA
no recurrence
6 months
Colen [11], 2009
13 years/F
L4 – L5
GTR
RT
5 – 7%
no recurrence
2 years
L2 – L5
GTR
none
5 – 10% 9 months/L1– L2
Present report
3 years/M
1 year NA
NA
PR and RT
* Recurred three times, the initial two recurrences were in the same location but the third recurrence was in the cerebellopontine angle. + The Ki67 increased from 3 to 20% at recurrence. EOR = Extent of resection; GTR = gross total resection; PR = partial resection; RT = radiotherapy; NA = not available.
References
314
1 Scheithauer BW: Tumors of the meninges: proposed modifications of the World Health Organization classification. Acta Neuropathol (Berl) 1990;80:343–354. 2 Liu PI, Liu GC, Tsai KB, Lin CL, Hsu JS: Intraspinal clear-cell meningioma: case report and review of literature. Surg Neurol 2005;63: 285–288.
Pediatr Neurosurg 2013;49:311–315 DOI: 10.1159/000366452
3 Park HC, Sohn MJ, Kim EY, Han HS, Park HS: Spinal clear cell meningioma presented with progressive paraparesis in infancy. Childs Nerv Syst 2000;16:607–610. 4 Zorludemir S, Scheithauer BW, Hirose T, Van Houten C, Miller G, Meyer FB: Clear cell meningioma: a clinicopathologic study of a potentially aggressive variant of meningioma. Am J Surg Pathol 1995;19:493–505.
Balogun /Halliday /Bouffet /Kulkarni
5 Heth JA, Kirby P, Menezes AH: Intraspinal familial clear cell meningioma in a mother and child. J Neurosurg 2000;93:317–321. 6 Jain D, Sharma MC, Sarkar C, Suri V, Garg A, Singh M, et al: Clear cell meningioma, an uncommon variant of meningioma: a clinicopathologic study of nine cases. J Neurooncol 2007;81:315–321. 7 Dubois A, Sevely A, Boetto S, Delisle MB, Manelfe C: Clear-cell meningioma of the cauda equina. Neuroradiology 1998;40:743–747. 8 Murakami T, Imoto K, Takebayashi T, Yamashita T: Clear cell meningioma of cauda equina in a 10-year-old child (case report). Tumor Res Exp Clin 2001;36:33–38. 9 Nakajima H, Uchida K, Kobayashi S, Takamura T, Yayama T, Baba H: Microsurgical excision of multiple clear cell meningiomas of the cauda equina: a case report. Minim Invasive Neurosurg 2009;52:32–35. 10 Matsui H, Kanamori M, Abe Y, Sakai T, Wakaki K: Multifocal clear cell meningioma in the spine: a case report. Neurosurg Rev 1998;21:171–173. 11 Colen CB, Rayes M, McClendon J Jr, Rabah R, Ham SD: Pediatric spinal clear cell meningioma: case report. J Neurosurg Pediatr 2009; 3: 57–60.
Spinal Clear Cell Meningioma in a 3-Year-Old
12 Epstein NE, Drexler S, Schneider J: Clear cell meningioma of the cauda equina in an adult: case report and literature review. J Spinal Disord Tech 2005;18:539–543. 13 Deen HG Jr, Scheithauer BW, Ebersold MJ: Clinical and pathological study of meningiomas of the first two decades of life. J Neurosurg 1982;56:317–322. 14 Wang XQ, Zeng XW, Zhang BY, Dou YF, Wu JS, Jiang CC, et al: Spinal meningioma in childhood: clinical features and treatment. Childs Nerv Syst 2012;28:129–136. 15 Brusius CV, Pereira Filho AA, Barnewitz JP, Putten AC, Pitta Pinheiro C, Aesse FF: Intradural spinal meningioma in a 20-month-old female. Pediatr Neurosurg 2008;44:247–252. 16 Fujii K, Sugita K, Honda T, Ishiwada N, Murakami M, Niimi H: Intradural spinal meningioma in a 5-year-old female. Brain Dev 1995; 17:349–351. 17 Oviedo A, Pang D, Zovickian J, Smith M: Clear cell meningioma: case report and review of the literature. Pediatr Dev Pathol 2005;8:386–390. 18 Kobayashi Y, Nakamura M, Tsuji O, Iwanami A, Ishii K, Watanabe K, et al: Nondura-based clear cell meningioma of the cauda equina in an adult. J Orthop Sci 2013;18:861–865. 19 Vural M, Arslantas A, Ciftci E, Artan S, Atasoy MA: An unusual case of cervical clear-cell meningioma in pediatric age. Childs Nerv Syst 2007;23:225–229.
20 Brastianos PK, Horowitz PM, Santagata S, Jones RT, McKenna A, Getz G, et al: Genomic sequencing of meningiomas identifies oncogenic SMO and AKT1 mutations. Nat Genet 2013;45:285–289. 21 Cances C, Chaix Y, Karsenty C, Boetto S, Sevely A, Delisle MB, Carriere JP: Clear cell meningioma: recurrent intraspinal tumor in a child (in French). Arch Pediatr 1998;5:758– 762. 22 Jallo GI, Kothbauer KF, Silvera VM, Epstein FJ: Intraspinal clear cell meningioma: diagnosis and management: report of two cases. Neurosurgery 2001;48:218–221; discussion 221–222. 23 Carra S, Drigo P, Gardiman M, Perilongo G, Rigobello L: Clear-cell meningioma in a 22-month-old male: a case report and literature review. Pediatr Neurosurg 2001;34:264– 267. 24 Carra S, Drigo P, Gardiman M, Perilongo G, Rigobello L: Clear cell meningioma in a 22-month-old male: update after five years. Pediatr Neurosurg 2003;38:162–163. 25 Yu KB, Lim MK, Kim HJ, Suh CH, Park HC, Kim EY, Han HS: Clear-cell meningioma: CT and MR imaging findings in two cases involving the spinal canal and cerebellopontine angle. Korean J Radiol 2002;3:125–129.
Pediatr Neurosurg 2013;49:311–315 DOI: 10.1159/000366452
315
Copyright: S. Karger AG, Basel 2014. Reproduced with the permission of S. Karger AG, Basel. Further reproduction or distribution (electronic or otherwise) is prohibited without permission from the copyright holder.
Received: June 15, 2014 Accepted after revision: July 29, 2014 Published online: November 1, 2014
Spinal Clear Cell Meningioma in a 3-Year-Old: A Case Report James A. Balogun a William Halliday c Eric Bouffet b Abhaya V. Kulkarni a Divisions of a Neurosurgery, b Neuro-Oncology and c Pathology, The Hospital for Sick Children, Toronto, Ont., Canada
Established Facts • Meningiomas are less common in children. • Clear cell meningioma (CCM) is a rare, aggressive variant of meningioma with limited reports in the literature. • There is no consensus on the use of adjuvant radiotherapy.
Novel Insights • Close clinical and radiological imaging is warranted even when gross total resection is achieved in intraspinal CCM. Reoperation should be attempted in recurrence and adjuvant radiotherapy in incomplete resection. • We review the literature on this disease entity.
Key Words Clear cell meningioma · Spine · Aggressive variant · Early recurrence
Abstract Clear cell meningioma (CCM) is an aggressive meningioma variant with a tendency to early recurrence posing a challenge to its treatment. Although spinal meningiomas are uncommon in children, this rare entity has been described as the most common variant of spinal meningiomas in the pediatric age group. We present the case of a 3-year-old with a confirmed lumbar spine CCM and discuss the problems encountered in the management of this disease.
Introduction
Clear cell meningioma (CCM) is a distinct variant of meningioma with unique histological features first described by Scheithauer [1]. It is the most common variant of pediatric spinal meningiomas [2–6]. The management of this tumor poses a great challenge due to its high recurrence rate within a short period despite gross total tumor excision [2–4, 7–10]. The role of adjuvant radiotherapy remains controversial [3, 11, 12]. We add to the literature on this rare tumor by reporting a case of a 3-year-old boy with this unusual pathology.
© 2014 S. Karger AG, Basel © 2014 S. Karger AG, Basel 1016–2291/14/0495–0311$39.50/0 E-Mail [email protected] www.karger.com/pne
Abhaya V. Kulkarni, MD, PhD Division of Neurosurgery, The Hospital for Sick Children, Suite 1503 555 University Avenue Toronto, ON M5G 1X8 (Canada) E-Mail Abhaya.kulkarni @ sickkids.ca
Case Report A 3-year-old boy with a history of speech delay and behavioral problems presented with inability to walk and lower back pain over the previous 6 months. The child had not yet achieved urinary or bowel continence. Examination revealed that he was paraparetic with grade 3 power in most muscle groups of the lower extremities. The patellar and ankle reflexes were absent. The magnetic resonance imaging (MRI; fig. 1a, b) revealed an enhancing soft tissue mass within the lumbar intraspinal canal extending from L2 to L5 with secondary canal widening and posterior remodeling of the lumbar vertebrae. No other spinal or cranial lesions were found on screening. He subsequently had a T12–L5 laminoplasty. The dura was opened and the tumor exposed along its entire craniocaudal length. The cauda equina was observed to be draped over the tumor. Microsurgical excision of the tumor was carried out with intraoperative neuromonitoring. After an initial debulking with the ultrasonic device, a number of nerve roots entangled within the tumor were observed and the left L4 nerve root had to be sacrificed to achieve gross total tumor resection. The histology of the tumor revealed clear cells arranged in lobules and sheets. There was a variable amount of collagen between cells and this appeared to progress to regions with very few tumor cells but lots of intermingled collagen. To a variable degree the tumor cells stained for glycogen. Immunohistochemical studies showed membranous activity for epithelial membrane antigen. The MIB index was 5–10%. Electron microscopy detailed the tumor cell cytoplasmic glycogen, intermediate filaments and cell-tocell junctions (desmosomes). This was representative of a CCM (fig. 2a–c). The immediate postoperative MRI (fig. 3a) confirmed complete tumor excision. The child progressively improved neurologically, eventually becoming self-ambulant and voiding voluntarily. Although he had been scheduled for 3-monthly MRI follow-up examinations, these were delayed due to intercurrent respiratory illness and social issues. He re-presented 9 months after the surgery with occasional falls and clumsiness when walking. Power in most muscle groups was grade 4+ except in the left ankle dorsiflexors, which were grade 1. MRI (fig. 3b) showed a recurrence of the tumor as two homogeneously enhancing masses at the L1–L2 levels. Brain MRI did not show evidence of dissemination. The patient underwent repeat surgical exploration. Only a subtotal excision of the tumor (fig. 3c) could be achieved due to traversing of the cauda equina through the main bulk of the tumor associated with slowing on the somatosensory evoked potentials and motor evoked potentials. Postoperatively, the neurological function remained unchanged. Pathology confirmed recurrence of the CCM. The patient was sent to the radiation oncology service for adjuvant radiotherapy.
Discussion
Meningiomas are uncommon in children and constitute 2% of all meningiomas [13]. Childhood spinal meningiomas form between 4 and 10% of the meningiomas in the pediatric population [14]. Scheithauer [1] described 312
Pediatr Neurosurg 2013;49:311–315 DOI: 10.1159/000366452
a
b
Fig. 1. Preoperative T1 sagittal image without contrast (a) and with contrast (b) showing heterogeneous enhancement.
the histological features of a rare variant of meningioma called CCM, which is classified by the WHO grading as a grade II variant. This entity has been described as the most common subtype in pediatric spinal meningiomas [2–6]. It is reported to occur more in the lumbar region affecting the cauda equina [2–4, 11] compared with other variants of meningiomas that occur in the thoracic and cervical regions [13–16]. The average age in children from case reports with literature review is about 11 years [11], with the youngest reported patient being a 2-monthold child [2]. There is no definitive sex predilection though there are reports of a female predominance [2, 11, 17]. CCM does not have distinguishing clinical features and the imaging characteristics are similar to those of other spinal meningiomas. The unique characteristics of CCM are continually being defined; however, there is a consensus on its more aggressive behavior with a tendency to early recurrence, which can occur as early as 5 months after resection but may also be delayed as much as 5 years with occasional multifocal occurrence despite its benign histological characteristics [3, 4, 7–10]. Liu et al. [2] in their review documented a local recurrence rate of 63% despite gross total tumor excision, with 25% of patients having multiple recurrences at the time of follow-up. Children are noted to have higher recurrence rates and shorter mean time to recurrence [9, 18]. It is intriguing that no factor has been identified that accounts for the high rate of recurBalogun /Halliday /Bouffet /Kulkarni
Color version available online
a
50 μm
50 μm
b
c
Fig. 2. a Masson’s trichrome stain shows the variable amount of collagen between tumor cells, which demonstrate a ‘clear cell’ histology. b Immunohistochemical studies for epithelial membrane antigen show the tumor cells to be reactive. c An electron microscopic picture
shows the tumor cell cytoplasm to contain glycogen and intermediate filaments. The tumor cells have cell-to-cell junctions, and two desmosomes are demonstrated in the center of the picture.
Fig. 3. a T1-weighted image with contrast after gross tumor resection showing surgical changes. b Contrast-enhanced T1 image of the recurrent tumor. c After subtotal resection of the recurrent tumor.
a
b
c
rence in CCMs. There has been a suggestion of an association between tumor recurrence and a high MIB-1 [4], but this is not firmly established [6, 18]. Our patient had an MIB-1 index of 5–10%, but we have no certainty regarding its role in the recurrence. Surgical excision remains the treatment of choice with the aim of achieving a gross total resection. It is also generally agreed upon that adjuvant radiotherapy is an optional treatment modality following incomplete resection. There is, however, no consensus on the role of radiotherapy following gross total tumor excision in this
aggressive tumor with a high propensity for recurrence even after gross tumor resection [2, 19]. The decision is more difficult because of the long-term effect of radiation in children as well as the fact that there have been reports of tumor recurrence despite radiotherapy in some cases [3, 12]. Colen et al. [11] suggest that adjuvant radiotherapy following gross tumor excision is beneficial even with a moderately high MIB-1. The other option, which was utilized in our patient, is close postoperative surveillance with neuroimaging of the whole neuraxis for the early detection of local recurrence and distant metastases. Recur-
Spinal Clear Cell Meningioma in a 3-Year-Old
Pediatr Neurosurg 2013;49:311–315 DOI: 10.1159/000366452
313
rences should be surgically excised with adjuvant radiation in the case of incomplete resection. The recognition of this aggressive meningioma variant is important, prompting a high index of suspicion from the patient’s clinical characteristics and imaging. When confirmed by histology, there should be close postoperative surveillance and adequate parental counseling.
Though Vural et al. [19] did not detect any genomic alteration in their reported case, it may be worthwhile to further probe for peculiar mutations and pathways by genomic sequencing that can be targeted for possible therapeutic intervention, as is being explored in other meningioma variants [20] (table 1) .
Table 1. Summary of the case reports of pediatric spinal CCM
First author [Ref.], Age/sex year
Location
EOR
Adjuvant treatment
Ki67
Time to recurrence/ location
Zorludemir [4], 1995
17 years/F
L4 – L5
GTR
none
NA
no recurrence
9 years/F
L3 –L5
GTR
none
NA
6 months/same location
Matsui [10], 1998
9 years/F
T11 –T12, PR L2, L4 –L 5
none
12%
no recurrence
Cances [21], 1998
10 years/F
L1 –L4
GTR
none
NA
5 months/L1 – L2
GTR and RT
Park [3], 2000
14 months/F
T12–L2
GTR
none
3%+
8 months/cauda equina
GTR
Heth [5], 2000
7 years/F
L4 –L5
GTR
none
NA
no recurrence
Jallo [22], 2001
8 years/F
L3 –L4
GTR
none
NA
6 months/L3 – L5
GTR and RT
C3 – C5*
PR
none
NA
10 weeks, 3 months, 2 years/same location
PR/GTR/GTR, RT and chemotherapy
3 years
5 years
22 months/F
Treatment at recurrence
Follow-up 3 years
GTR
2 years 1 year 8 months NA 13 months 11 months
Carra [23, 24], 2001, 2003
22 months/M T11–L4
GTR
none
NA
5 years/vermis to C2
GTR
Yu [25], 2002
14 months/F
T12–L2
GTR
none
3%
8 months, 7 months/ same location
GTR/GTR and NA RT
Oviedo [17], 2005
7 years/M
L2 – L3
GTR
none
10%
no recurrence
Liu [2], 2005
2 years/M
T10–L1
GTR
none
NA
5 years/T12
Vural [19], 2007
4 years/F
C1 – C2
GTR
none
NA
no recurrence
6 months
Colen [11], 2009
13 years/F
L4 – L5
GTR
RT
5 – 7%
no recurrence
2 years
L2 – L5
GTR
none
5 – 10% 9 months/L1– L2
Present report
3 years/M
1 year NA
NA
PR and RT
* Recurred three times, the initial two recurrences were in the same location but the third recurrence was in the cerebellopontine angle. + The Ki67 increased from 3 to 20% at recurrence. EOR = Extent of resection; GTR = gross total resection; PR = partial resection; RT = radiotherapy; NA = not available.
References
314
1 Scheithauer BW: Tumors of the meninges: proposed modifications of the World Health Organization classification. Acta Neuropathol (Berl) 1990;80:343–354. 2 Liu PI, Liu GC, Tsai KB, Lin CL, Hsu JS: Intraspinal clear-cell meningioma: case report and review of literature. Surg Neurol 2005;63: 285–288.
Pediatr Neurosurg 2013;49:311–315 DOI: 10.1159/000366452
3 Park HC, Sohn MJ, Kim EY, Han HS, Park HS: Spinal clear cell meningioma presented with progressive paraparesis in infancy. Childs Nerv Syst 2000;16:607–610. 4 Zorludemir S, Scheithauer BW, Hirose T, Van Houten C, Miller G, Meyer FB: Clear cell meningioma: a clinicopathologic study of a potentially aggressive variant of meningioma. Am J Surg Pathol 1995;19:493–505.
Balogun /Halliday /Bouffet /Kulkarni
5 Heth JA, Kirby P, Menezes AH: Intraspinal familial clear cell meningioma in a mother and child. J Neurosurg 2000;93:317–321. 6 Jain D, Sharma MC, Sarkar C, Suri V, Garg A, Singh M, et al: Clear cell meningioma, an uncommon variant of meningioma: a clinicopathologic study of nine cases. J Neurooncol 2007;81:315–321. 7 Dubois A, Sevely A, Boetto S, Delisle MB, Manelfe C: Clear-cell meningioma of the cauda equina. Neuroradiology 1998;40:743–747. 8 Murakami T, Imoto K, Takebayashi T, Yamashita T: Clear cell meningioma of cauda equina in a 10-year-old child (case report). Tumor Res Exp Clin 2001;36:33–38. 9 Nakajima H, Uchida K, Kobayashi S, Takamura T, Yayama T, Baba H: Microsurgical excision of multiple clear cell meningiomas of the cauda equina: a case report. Minim Invasive Neurosurg 2009;52:32–35. 10 Matsui H, Kanamori M, Abe Y, Sakai T, Wakaki K: Multifocal clear cell meningioma in the spine: a case report. Neurosurg Rev 1998;21:171–173. 11 Colen CB, Rayes M, McClendon J Jr, Rabah R, Ham SD: Pediatric spinal clear cell meningioma: case report. J Neurosurg Pediatr 2009; 3: 57–60.
Spinal Clear Cell Meningioma in a 3-Year-Old
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Pediatr Neurosurg 2013;49:311–315 DOI: 10.1159/000366452
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Copyright: S. Karger AG, Basel 2014. Reproduced with the permission of S. Karger AG, Basel. Further reproduction or distribution (electronic or otherwise) is prohibited without permission from the copyright holder.
