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Multicentric extramedullary myxopapillary ependymomas: Two case reports and literature review.

Landriel F, Ajler P, Tedesco N, Bendersky D, Vecchi E - Surg Neurol Int (2012)

Bottom Line: Magnetic resonance imaging (MRI) showed multiple intradural extramedullary lesions with homogeneous enhancement after gadolinium injection at C7, D2, D4, D5, D8, D10, D11, L1, L3, L5, S1, and S2.Complete tumor resection of the approached tumors was archived in both cases.Patient's neurologic outcome was good and no residual tumor was present at MRI control at 10 years in case 1 and 12 months in case 2.

View Article: PubMed Central - PubMed

Affiliation: Neurosurgical Department of the Hospital Italiano de Buenos Aires. J.D. Perón, CH - Buenos Aires - Argentina.

ABSTRACT

Background: Ependymoma has been described typically as an intramedullary tumor derived from ependymal cells. Intradural extramedullary presentation is rarely described and almost always as a unique lesion. Myxopapillary ependymoma is a histological variant that distinguishes from the ordinary type of ependymoma because of its generally better prognosis. We present two cases of multicentric extramedullary myxopapillary ependymomas.

Case description: Case 1 was a 30-year-old man with progressive paresthesia and paresis in the lower limbs, urinary sphincter disturbances, gait instability, ataxia, and chronic low back pain with multiple intradural extramedullary lesions at C2-C3, D2-D4-D5, and D12-L1. Case 2 was a 32-year-old man, presented with low back pain and mild paresthesia in the right lower limb. Magnetic resonance imaging (MRI) showed multiple intradural extramedullary lesions with homogeneous enhancement after gadolinium injection at C7, D2, D4, D5, D8, D10, D11, L1, L3, L5, S1, and S2. Complete tumor resection of the approached tumors was archived in both cases. Histological studies confirm myxopapillary ependymomas. Patient's neurologic outcome was good and no residual tumor was present at MRI control at 10 years in case 1 and 12 months in case 2.

Conclusions: We report the first two cases of multicentric extramedullary myxopapillary ependymomas, this etiology must be taken into account in the differential diagnosis of intradural extramedullary tumors.

No MeSH data available.


Related in: MedlinePlus

(a and b) Demonstrate D2 anteromedullary enhanced tumor on T1 sequence. (c and d) Show D12-L1 lesion posterior located in the spinal canal displacing anteriorly the conus medullaris. (e and f) 10-year postoperative control MRI showing no recurrence on T1. (g and h) Shows no recurrence in T2. (i, j, k and l) demonstrate no tumor at D12-L2 level in 10-year postoperative control
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Figure 1: (a and b) Demonstrate D2 anteromedullary enhanced tumor on T1 sequence. (c and d) Show D12-L1 lesion posterior located in the spinal canal displacing anteriorly the conus medullaris. (e and f) 10-year postoperative control MRI showing no recurrence on T1. (g and h) Shows no recurrence in T2. (i, j, k and l) demonstrate no tumor at D12-L2 level in 10-year postoperative control

Mentions: A 30-year-old man was referred to our neurosurgical department presenting progressive paresthesia and paraparesis, urinary sphincter disturbances, gait instability, ataxia, and chronic low back pain. Neurological examination showed bilateral hypoesthesia at D2 and perineal region was not associated with radicular pain and hypereflexia in lower-limbs. Spinal magnetic resonance imaging (MRI) revealed multiple intradural extramedullary lesions, isointense on T1, hyperintense on T2, and fluid attenuated inversion recovery (FLAIR) sequences with intense contrast enhancement, at C2-C3, D2-D4-D5, and D12-L1 [Figure 1 shows no recurrence in T2. I, J, K and L, demonstrate no tumor at D12-L2 level in 10-year postoperative control”]. Cranial MRI did not reveal any abnormality. The D12-L1 tumor was located posteriorly in the spinal canal displacing anteriorly the conus medullaris and was removed through a D12-L2 laminectomy in first place. The D2 antero-medullary mass compress and displace posteriorly the spinal cord. After D1-D3 laminectomy and midline durotomy the left dental ligament was cut and retracted to improve surgical field. The tumor has soft consistence and a fleshy-gray appearance and was completely encapsulated by a thin fibrous capsule with a well delimitated cleavage plane. A microvascular attachment to the spinal cord was identified, coagulated, and divided. The tumor was debulked and completely resected using microsurgical techniques. Histological results inform a myxopapillary ependymoma. Patient's neurologic condition improved after surgery and he was discharged with a mild lower limb paresis and D2 left radicular pain, which resolved with rehabilitation 6 months after treatment. Adjuvant radiotherapy was performed and he had no tumor progression in 10 years follow up.


Multicentric extramedullary myxopapillary ependymomas: Two case reports and literature review.

Landriel F, Ajler P, Tedesco N, Bendersky D, Vecchi E - Surg Neurol Int (2012)

(a and b) Demonstrate D2 anteromedullary enhanced tumor on T1 sequence. (c and d) Show D12-L1 lesion posterior located in the spinal canal displacing anteriorly the conus medullaris. (e and f) 10-year postoperative control MRI showing no recurrence on T1. (g and h) Shows no recurrence in T2. (i, j, k and l) demonstrate no tumor at D12-L2 level in 10-year postoperative control
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3475877&req=5

Figure 1: (a and b) Demonstrate D2 anteromedullary enhanced tumor on T1 sequence. (c and d) Show D12-L1 lesion posterior located in the spinal canal displacing anteriorly the conus medullaris. (e and f) 10-year postoperative control MRI showing no recurrence on T1. (g and h) Shows no recurrence in T2. (i, j, k and l) demonstrate no tumor at D12-L2 level in 10-year postoperative control
Mentions: A 30-year-old man was referred to our neurosurgical department presenting progressive paresthesia and paraparesis, urinary sphincter disturbances, gait instability, ataxia, and chronic low back pain. Neurological examination showed bilateral hypoesthesia at D2 and perineal region was not associated with radicular pain and hypereflexia in lower-limbs. Spinal magnetic resonance imaging (MRI) revealed multiple intradural extramedullary lesions, isointense on T1, hyperintense on T2, and fluid attenuated inversion recovery (FLAIR) sequences with intense contrast enhancement, at C2-C3, D2-D4-D5, and D12-L1 [Figure 1 shows no recurrence in T2. I, J, K and L, demonstrate no tumor at D12-L2 level in 10-year postoperative control”]. Cranial MRI did not reveal any abnormality. The D12-L1 tumor was located posteriorly in the spinal canal displacing anteriorly the conus medullaris and was removed through a D12-L2 laminectomy in first place. The D2 antero-medullary mass compress and displace posteriorly the spinal cord. After D1-D3 laminectomy and midline durotomy the left dental ligament was cut and retracted to improve surgical field. The tumor has soft consistence and a fleshy-gray appearance and was completely encapsulated by a thin fibrous capsule with a well delimitated cleavage plane. A microvascular attachment to the spinal cord was identified, coagulated, and divided. The tumor was debulked and completely resected using microsurgical techniques. Histological results inform a myxopapillary ependymoma. Patient's neurologic condition improved after surgery and he was discharged with a mild lower limb paresis and D2 left radicular pain, which resolved with rehabilitation 6 months after treatment. Adjuvant radiotherapy was performed and he had no tumor progression in 10 years follow up.

Bottom Line: Magnetic resonance imaging (MRI) showed multiple intradural extramedullary lesions with homogeneous enhancement after gadolinium injection at C7, D2, D4, D5, D8, D10, D11, L1, L3, L5, S1, and S2.Complete tumor resection of the approached tumors was archived in both cases.Patient's neurologic outcome was good and no residual tumor was present at MRI control at 10 years in case 1 and 12 months in case 2.

View Article: PubMed Central - PubMed

Affiliation: Neurosurgical Department of the Hospital Italiano de Buenos Aires. J.D. Perón, CH - Buenos Aires - Argentina.

ABSTRACT

Background: Ependymoma has been described typically as an intramedullary tumor derived from ependymal cells. Intradural extramedullary presentation is rarely described and almost always as a unique lesion. Myxopapillary ependymoma is a histological variant that distinguishes from the ordinary type of ependymoma because of its generally better prognosis. We present two cases of multicentric extramedullary myxopapillary ependymomas.

Case description: Case 1 was a 30-year-old man with progressive paresthesia and paresis in the lower limbs, urinary sphincter disturbances, gait instability, ataxia, and chronic low back pain with multiple intradural extramedullary lesions at C2-C3, D2-D4-D5, and D12-L1. Case 2 was a 32-year-old man, presented with low back pain and mild paresthesia in the right lower limb. Magnetic resonance imaging (MRI) showed multiple intradural extramedullary lesions with homogeneous enhancement after gadolinium injection at C7, D2, D4, D5, D8, D10, D11, L1, L3, L5, S1, and S2. Complete tumor resection of the approached tumors was archived in both cases. Histological studies confirm myxopapillary ependymomas. Patient's neurologic outcome was good and no residual tumor was present at MRI control at 10 years in case 1 and 12 months in case 2.

Conclusions: We report the first two cases of multicentric extramedullary myxopapillary ependymomas, this etiology must be taken into account in the differential diagnosis of intradural extramedullary tumors.

No MeSH data available.


Related in: MedlinePlus