Filum terminale ependymomas. Analysis of a serie of 20 consecutive cases
Servicio de Neurocirugía, Hospital Clínico Universitario de Santiago de Compostela, Departamento de Cirugía, Universidad de Santiago de Compostela. Neurocirugia (Asturias, Spain)
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to analyze the clinical, radiological and surgical outcome of a series of filum terminale ependymomas. Patients and methods. This retrospective study involved 20 patients with 21 ependymomas of the filum terminale encountered during a 21 year period (1988- 2008). All patients were diagnosed using MRI and surgically treated.
the male: female ratio was 1:1.5, and the mean age at diagnosis was 44.8 years (range 15-64). First symptom included radicular pain (12 cases) and lumbar pain in the other 8 cases, with average symptom duration of 8.7 years (range 0-6-32). All patients underwent open biopsy, seventeen tumours received gross-total resection and 4 received subtotal resection. Histologically, 20 tumours were myxopapillary ependymomas (grade I) and 1 case a grade II ependymoma. The mean follow-up period was 8 years (range 1-18 years).
filum terminale ependimomas are slow growing tumours of the cauda equina with a high incidence in young adults. The most common presentation is with low back pain long time evolution. Although ependymomas of the filum terminale are thought to be benign, local recurrence is not uncommon.
Available from: Pablo M. Munarriz
- "The cervical region is the most common level of true intramedullary occurrence; however, 40% occurs within filum terminale. A variety of histological ependymoma subtypes may be encountered, the myxopapillary being the most common within cauda equina or filum terminale. Typical presentations for these tumors include pain, lower-extremities paresthesias, lower-extremities weakness, or bladder dysfunction. "
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ABSTRACT: Ependymomas are the most common intramedullary tumors in adults and are the most common in mid-adult years. The presence of synchronous ependymomas in different sites of the spine is not common and it is even more infrequent to find hemorrhage from a spinal ependymoma as a cause of neurological deterioration.
A 32-year-old man presented with back pain and progressive paraparesia. Magnetic resonance (MR) showed two intradural extramedullary lesions on spinal canal with signs of acute hemorrhage. The patient underwent emergent surgical decompression and resection. Pathology revealed myxopapillary ependymomas.
To our knowledge, we report the first case of a patient with acute neurological deterioration as a consequence of synchronous bleeding of two spinal ependymomas located at different levels in the spinal cord. This study illustrates the importance of recognizing the rare, but known occurrence of acute neurological deterioration after spontaneous hemorrhage in spinal ependymomas.
Available from: thejns.org
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ABSTRACT: Primary spinal cord ependymomas (EPNs) are rare in children, comprising classical WHO Grade II and III tumors and Grade I myxopapillary ependymomas (MEPNs). Despite their benign histology, recurrences and neural-axis dissemination have been reported in up to 33% MEPNs in the pediatric population. Treatment options beyond resection are limited, and little is known about their tumorigenesis. The purpose of this study was to explore the tumor biology and outcomes in a consecutive series of pediatric patients treated at a single institution.
The authors performed a retrospective clinicopathological review of 19 patients at a tertiary referral children's hospital for resection of a spinal cord ependymoma. The population included 8 patients with a pathological diagnosis of MEPN and 11 patients with a pathological diagnosis of spinal EPN (10 cases were Grade II and 1 case was Grade III). The upregulation of the following genes HOXB13, NEFL, PDGFRα, EGFR, EPHB3, AQP1, and JAGGED 1 was studied by immunohistochemistry from archived paraffin-embedded tumor samples of the entire cohort to compare the expression in MEPN versus EPN.
Gross-total resection was achieved in 75% of patients presenting with MEPNs and in 100% of those with EPNs. The average follow-up period was 79 months for the MEPN subset and 53 months for Grade II/III EPNs. Overall survival for both subsets was 100%. However, event-free survival was only 50% for patients with MEPNs. Of note, in all cases involving MEPNs that recurred, the patients had undergone gross-total resection on initial surgery. In contrast, there were no tumor recurrences in patients with EPNs. Immunohistochemistry revealed no significant differences in protein expression between the two tumor types with the exception of EPHB3, which demonstrates a tendency to be positive in MEPNs (6 reactive tumors of 9) rather than in EPN (2 reactive tumors of 10).
The authors' experience shows that, following a gross-total resection, MEPNs are more likely to recur than their higher-grade counterpart, EPNs. This supports the recommendation for close long-term radiological follow-up of pediatric patients with MEPNs to monitor for recurrence, despite the tumor's low-grade histological feature. No significant difference in the protein expression of HOXB13, NEFL, PDGFRα, EGFR, EPHB3, AQP1, and JAGGED 1 was present in this selected cohort of pediatric patients.
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ABSTRACT: Primary filum terminale ependymoma (PFTE) is a unique type of ependymomas and locates on extramedullary site. However, the clinical features and prognostic factors of PFTE are still unknown due to its rarity.
This study aimed to evaluate the clinical features, outcomes, and prognostic factors of PFTE in the largest series of cases.
Thirty-eight patients were included in this study. Gross total removal (GTR) of the tumors was achieved in 33(87%) patients. Five (13%) patients had subtotal resection (STR). For the residual tumors, postoperative radiotherapy increased the interval between the first surgery and tumor regrowth (P = 0.063). Six patients had local recurrence/progression. Univariate analysis identified STR(P = 0.001), unencapsulated tumor (P = 0.018), tumor involving more than two vertebral columns (P = 0.005), and tumor invading sacral canal(P < 0.001) as predictors of tumor recurrence. In addition, 36 (95%) patients had stable or improved neurological status directly after surgery. Klekamp-Samii score was better correlated with the symptoms than McCormick scale.
Extent of surgical removal, tumor size, tumor location, and the integrity of tumor capsule are the prognostic factors of PFTEs, and the intrasacral PFTEs always have a poor prognosis.
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