Spinal cord subependymoma

Spinal cord subependymoma

spinal cord subependymoma (SCSE) is a benign, non-invasive, slow-growing, WHO Grade I spinal cord tumor 1), first reported by Boykin et al. in 19542).

Epidemiology

Their most common site of occurrence is the fourth ventriclefollowed by the lateral ventricles. Spinal cord subependymomas typically manifest as cervical and cervicothoracic intramedullary or, rarely, extramedullary mass lesions.

Pathology

Histologically, there are hypocellular areas with occasional clusters of cells and frequent microcystic changes, calcifications, and hemorrhage. Radiologically, subependymomas generally manifest as eccentric well circumscribed nodular lesions with mild-to-moderate enhancement.

Clinical features

They often present clinically with pain and neurologic symptoms, including motor, sensory, urinary, and sexual dysfunction.

Diagnosis

Toi et al., made an important discovery of what seems to be a characteristic appearance for spinal subependymoma on sagittal MRI. Swelling of the spinal cord is extremely steep, providing unusually large fusiform dilatation resembling a bamboo leaf. They termed this characteristic MRI appearance as the “bamboo leaf sign.” This characteristic was apparent in 76.2% of cases of spinal subependymoma for which MRI findings were reported. Conclusion. The bamboo leaf sign on spinal MRI is useful for differentiating between subependymoma and other intramedullary tumors. Neurosurgeons encountering the bamboo leaf sign on spinal MRI should consider the possibility of subependymoma 3).

Differential diagnosis

It is not easily differentiable from a spinal cord ependymoma with radiological findings.

Treatment

Spinal cord subependymomas are not dissected easily from the spinal cord. Considering the rather indolent nature of spinal cord subependymomas, subtotal removal without the risk of neurological deficit is another option 4).

Outcome

Surgical findings and outcomes differ from those of an ependymoma, including a high risk of neurological deficit in the event of a poor dissection plane from the spinal cord with a low rate of recurrence.

Case series

Mikula et al., present a series of spinal cord subependymomas with a detailed description of the clinical, radiological and pathological features, and characterization by chromosomal microarray analysis. Briefly, the four patients included two men and two women, between the ages of 22 and 48 years. The most common presenting symptoms were neck and arm pain with upper extremity weakness. By imaging, the tumors were found to involve multiple spinal levels, including cervical/ cervico-thoracic (three patients) and thoracic (one patient), were all eccentric, and had minimal to no post-contrast enhancement. Two patients underwent gross total resection, one had a sub-total resection, and one underwent biopsy alone with a decompressive laminectomy. Follow up ranged from 6 months to 22 years. One patient (case 4) had recurrence 15 years following gross total resection and chromosomal microarray analysis revealed deletions on the long arm of chromosome 6. Our limited series suggests that spinal cord subependymomas can rarely recur, even following gross total resection, suggesting a possible role for long-term surveillance for these rare tumors5).


Yuh et al., retrospectively reviewed the medical records of ten spinal cord subependymoma patients (M : F=4 : 6; median 38 years; range, 21-77) from four institutions.

The most common symptoms were sensory changes and/or pain in eight patients, followed by motor weakness in six. The median duration of symptoms was 9.5 months. Preoperative radiological diagnosis was ependymoma in seven and astrocytoma in three. The tumors were located eccentrically in six and were not enhanced in six. Gross total resection of the tumor was achieved in five patients, whereas subtotal or partial resection was inevitable in the other five patients due to a poor dissection plane. Adjuvant radiotherapy was performed in two patients. Neurological deterioration occurred in two patients; transient weakness in one after subtotal resection and permanent weakness after gross total resection in the other. Recurrence or regrowth of the tumor was not observed during the median 31.5 months follow-up period (range, 8-89).

Spinal cord subependymoma should be considered when the tumor is located eccentrically and is not dissected easily from the spinal cord. Considering the rather indolent nature of spinal cord subependymomas, subtotal removal without the risk of neurological deficit is another option 6).

Case reports

A 51-year-old man presented with a 2-year history of progressive muscle weakness in the right lower extremity. Sagittal magnetic resonance imaging (MRI) showed spinal cord expansion at the Th7-12 vertebral level. Surgical resection was performed and the tumor was found to involve predominantly subpial growth. Histological diagnosis was subependymoma, classified as Grade I according to criteria of World Health Organization. They made an important discovery of what seems to be a characteristic appearance for spinal subependymoma on sagittal MRI. Swelling of the spinal cord is extremely steep, providing unusually large fusiform dilatation resembling a bamboo leaf. They termed this characteristic MRI appearance as the “bamboo leaf sign.” This characteristic was apparent in 76.2% of cases of spinal subependymoma for which MRI findings were reported. Conclusion. The bamboo leaf sign on spinal MRI is useful for differentiating between subependymoma and other intramedullary tumors. Neurosurgeons encountering the bamboo leaf sign on spinal MRI should consider the possibility of subependymoma 7).


A case report of a single patient in whom a subependymoma was resected from the cervical spinal cord with return to normal functioning.

Clinical examination, magnetic resonance imaging evaluation, surgical resection, and histological analysis were performed for diagnosis and treatment of this patient.

The patient experiencing myelopathy symptoms underwent a surgical resection of cervical spinal cord subependymoma that resulted in return to normal function.

Subependymoma should be included in the differential diagnosis of atypical presentations for myelopathy, as discrete surgical resection can result in good outcome 8).


A 53 year old man with a progressive paraparesis, paraesthesias of the lower limbs and sphincter disturbance. The tumour was partly removed, without progression 5 years after surgery 9).

References

1)

Im SH, Paek SH, Choi YL, Chi JG, Kim DG, Jung HW, Cho BK. Clinicopathological study of seven cases of symptomatic supratentorial subependymoma. J Neurooncol. 2003 Jan;61(1):57-67. PubMed PMID: 12587796.
2)

BOYKIN FC, COWEN D, IANNUCCI CA, WOLF A. Subependymal glomerate astrocytomas. J Neuropathol Exp Neurol. 1954 Jan;13(1):30-49. PubMed PMID: 13118373.
3) , 7)

Toi H, Ogawa Y, Kinoshita K, Hirai S, Takai H, Hara K, Matsushita N, Matsubara S, Uno M. Bamboo Leaf Sign as a Sensitive Magnetic Resonance Imaging Finding in Spinal Subependymoma: Case Report and Literature Review. Case Rep Neurol Med. 2016;2016:9108641. doi: 10.1155/2016/9108641. Epub 2016 Dec 15. PubMed PMID: 28074165; PubMed Central PMCID: PMC5198089.
4) , 6)

Yuh WT, Chung CK, Park SH, Kim KJ, Lee SH, Kim KT. Spinal Cord Subependymoma Surgery : A Multi-Institutional Experience. J Korean Neurosurg Soc. 2018 Mar;61(2):233-242. doi: 10.3340/jkns.2017.0405.001. Epub 2018 Feb 28. PubMed PMID: 29526067; PubMed Central PMCID: PMC5853201.
5)

Mikula AL, Paolini MA, Sukov WR, Clarke MJ, Raghunathan A. Subependymoma involving multiple spinal cord levels: A clinicopathological case series with chromosomal microarray analysis. Neuropathology. 2019 Mar 11. doi: 10.1111/neup.12543. [Epub ahead of print] PubMed PMID: 30856298.
8)

Cure LM, Hancock CR, Barrocas AM, Sternau LL, C Hirzel A. Interesting case of subependymoma of the spinal cord. Spine J. 2014 May 1;14(5):e9-12. doi: 10.1016/j.spinee.2013.10.056. Epub 2013 Nov 20. PubMed PMID: 24269267.
9)

Dario A, Fachinetti P, Cerati M, Dorizzi A. Subependymoma of the spinal cord: case report and review of the literature. J Clin Neurosci. 2001 Jan;8(1):48-50. PubMed PMID: 11148079.

Spinal meningioma treatment

Spinal meningioma treatment

Radical resection of spinal meningiomas can be performed with good functional results. Extensive tumor calcification, especially in elderly patients proved to harbor an increased risk for surgical morbidity 1).


Onken et al., reported on their surgical experience that involves two institutions in which 207 patients underwent surgery for spinal meningiomas (sMNGs) . Special focus was placed on patients with sMNGs localized anterior to the denticulate ligament (aMNGs) that were treated via a unilateral posterior approach (ULPA).

The duration of surgery, extent of resection, and outcomes are comparable between aMNGs and posterior to the denticulate ligament (pMNGs) when removed via a ULPA. Thus, ULPA represents a safe route to achieve a gross-total resection, even in cases of aMNG 2).


Posterior approaches provide adequate exposure to safely remove ventrally located spinal meningioma. Posterior exposures with lateral bone resection, denticulate ligament division, provide also adequate exposure for safe removal 3).

Technique

After dural opening, a plane can be developed between the arachnoid and the tumor. The tumor is then internally debulked using suction, an ultrasonic surgical aspirator, microscissors, or laser.

After debulking, in the majority of cases the tumor can be rolled away from the spinal cord and toward its dural attachment.

The tumor is then removed from its dural attachment.

Dura with remaining tumor can be coagulated using bipolar cauterization or resected.

In the majority of cases, the dural attachment was cauterized rather than resected. The dural attachment was always cauterized in cases involving an anterior dural attachment. Additionally, in most cases the dura was closed primarily, compared with suturing in a graft, which was performed far less frequently

Another option was separation of the dura into an outer and inner layer and to resect the tumor with the inner layer, leaving the outer layer available for closure 4).

Videos

References

1)

Sandalcioglu IE, Hunold A, Müller O, Bassiouni H, Stolke D, Asgari S. Spinal meningiomas: critical review of 131 surgically treated patients. Eur Spine J. 2008 Aug;17(8):1035-41. doi: 10.1007/s00586-008-0685-y. Epub 2008 May 15. PubMed PMID: 18481118; PubMed Central PMCID: PMC2518757.
2)

Onken J, Obermüller K, Staub-Bartelt F, Meyer B, Vajkoczy P, Wostrack M. Surgical management of spinal meningiomas: focus on unilateral posterior approach and anterior localization. J Neurosurg Spine. 2018 Dec 1:1-6. doi: 10.3171/2018.8.SPINE18198. [Epub ahead of print] PubMed PMID: 30544344.
3)

Notani N, Miyazaki M, Kanezaki S, Ishihara T, Kawano M, Tsumura H. Surgical management of ventrally located spinal meningiomas via posterior approach. Eur J Orthop Surg Traumatol. 2017 Feb;27(2):181-186. doi: 10.1007/s00590-016-1860-1. PubMed PMID: 27671472.
4)

Gottfried ON, Gluf W, Quinones-Hinojosa A, Kan P, Schmidt MH. Spinal meningiomas: surgical management and outcome. Neurosurg Focus. 2003 Jun 15;14(6):e2. Review. PubMed PMID: 15669787.

Intramedullary Schwannoma

Intramedullary Schwannoma

James Watson Kernohan has been recognized as the first neurosurgeon to report an intramedullary schwannoma case in 1952, though Wilder Penfield had already described an intramedullary lesion with schwannomas characteristics in 1932 1).

Epidemiology

Intramedullary (IM) schwannomas are rare entities representing the 0.3-1% of intramedullary tumors and 1.1% of spinal schwannomas. Beside many theories proposed, their rare occurrence might be related to the absence of Schwann cells into the spinal cord.

To date, approximately around 60 cases of intramedullary schwannoma in patients without neurofibromatosis have been reported in the English literature, of which only eight were children 2).

The male:female ratio for intramedullory schwannomas is 3:1 with a mean age of 40 years old. They are usually single lesions affecting the cervical spinal cord (63%), the thoracic spinal cord (26%), and the lumber spinal cord (11%). They have a slow growth pattern and because of this, the average interval between first symptom and diagnosis is 28.2 months (from 6 months through 20 years) 3).

see Thoracic intramedullary schwannoma.

Clinical features

The most described clinical manifestation is the pyramidal syndrome followed by sensitivity complaints and sphincter dysfunction. There are reports of muscular fasciculations as the first symptom 4).

Diagnosis

A tumor located in the dorsolateral spinal cord, causing expansion of the cord, with hypointense areas on T2-weighted and obviously enhancement, should arise suspicion of an intramedullary schwannoma 5).

http://www.asianjns.org/viewimage.asp?img=AsianJNeurosurg_2015_10_1_42_151509_f2.JPG

Outcome

Intramedullary schwannoma may recur after resection 6).

Case series

8 patients with pathologically confirmed intramedullary schwannomas were reviewed.

There were 6 male and 2 female patients (mean age, 49 years). Tumors were located in the cervical cord (2), the thoracic cord (5), and the cervicothoracic cord (1). Most were in the dorsolateral spinal cord and limited to one side. Expansion of the cord was observed. The majority were hypointense on T1-weighted images and hypo-hyperintense on T2-weighted images. Peritumoral edema was easily found. Gadolinium enhancement was obviously. No recurrence was seen during the follow-up period.

A tumor located in the dorsolateral spinal cord, causing expansion of the cord, with hypointense areas on T2-weighted and obviously enhancement, should arise suspicion of an intramedullary schwannoma 7).

Case reports

Dhake and Chatterjee described two cases of thoracic intramedullary schwannomas that recurred after primary excision. A 10 years old boy presented with weakness of both lower limbs. Magnetic resonance imaging showed a D10 to D12 intramedullary lesion, which was excised near totally and confirmed to be a schwannoma on histopathological examination. The tumour recurred twice after that and was re operated both times. Another 57 years old lady presented with weakness of both lower limbs and a history of being operated in the past for D9-D10 intramedullary lesion. She was re-operated with total removal of the lesion confirmed to be schwannoma on histopathological examination. Intramedullary schwannoma may recur after resection. 8).


A 8 year-old female affected by a progressive paraparesis. Neuroradiological investigations showed an oval-shaped mass at the level of T10-T11. The patient underwent surgery, performed under neurophysiological monitoring. The patient was operated on with a complete removal of the lesion. The postoperative course was uneventful.

The clinical, neuroradiological, and intraoperative findings are presented, along with a review of the literature. Despite the number of lesions potentially compressing the spinal cord, IM schwannoma is rare but should be taken into account in the differential diagnosis 9).


A rare case of thoracic intramedullary tumour in a 28 year old male patient who presented with progressive weakness of both the lower limbs and decreased sensation below D2 dermatomal level. Magnetic resonance imaging revealed an intramedullary lesion from D1 toD7. D1 to D7 laminoplasty and near total excision of the mass done. Total removal was difficult because of the infiltrative nature of the tumour 10).

References

1)

Penfield W. Notes on operative technic in neurosurgery. Ann surg. 1946;124:383–5.
2) , 6) , 8)

Dhake RP, Chatterjee S. Recurrent thoracic intramedullary schwannoma: report of two cases with long term follow up. Br J Neurosurg. 2019 Mar 5:1-4. doi: 10.1080/02688697.2019.1566516. [Epub ahead of print] PubMed PMID: 30836024.
3)

Lopez J, Diaz DR, Medina YC, Jeronimo HS, Mendez AZ, Vazquez AG, et al. Schwannoma intramedullary cervical. Arch neurocien. 2004;9:55–8.
4) , 10)

Nayak R, Chaudhuri A, Chattopadhyay A, Ghosh SN. Thoracic intramedullary schwannoma: A case report and review of literature. Asian J Neurosurg. 2015 Apr-Jun;10(2):126-8. doi: 10.4103/1793-5482.145155. PubMed PMID: 25972946; PubMed Central PMCID: PMC4421952.
5) , 7)

Gao L, Sun B, Han F, Jin Y, Zhang J. Magnetic Resonance Imaging Features of Intramedullary Schwannomas. J Comput Assist Tomogr. 2017 Jan;41(1):137-140. doi: 10.1097/RCT.0000000000000493. PubMed PMID: 27680415.
9)

Landi A, Grasso G, Gregori F, Iacopino G, Ruggeri A, Delfini R. Isolated Pediatric Intramedullary Schwannoma. Case report and review of the literature. World Neurosurg. 2018 May 9. pii: S1878-8750(18)30940-9. doi: 10.1016/j.wneu.2018.04.220. [Epub ahead of print] PubMed PMID: 29753078.
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