Cauda equina ependymomas: surgical treatment and long-term outcomes in a series of 125 patients

Abstract

OBJECTIVE Cauda equina ependymoma (CEE) is a rare tumor for which little information is available on the oncological and clinical outcomes of patients. In this study the authors aimed to address functional, oncological, and quality-of-life (QOL) outcomes in a large series of consecutive patients operated on at their institution during the past 20 years. METHODS The records of 125 patients who underwent surgery between January 1998 and September 2018 were reviewed. Analyzed variables included demographic, clinical, radiological, surgical, and histopathological features. Neurological outcomes were graded according to the McCormick and Kesselring scales. The QOL at follow-up was evaluated by administering the EQ-5DL questionnaire. RESULTS On admission, 84% of patients had a McCormick grade of I and 76.8% had a Kesselring score of 0. At follow-up (clinical 8.13 years; radiological 5.87 years) most scores were unchanged. Sacral level involvement (p = 0.029) and tumor size (p = 0.002) were predictors of poor functional outcome at discharge. Tumor size (p = 0.019) and repeated surgery (p < 0.001) were predictors of poor outcome. A preoperative McCormick grade ≥ III and Kesselring grade ≥ 2 were associated with worse outcomes (p = 0.035 and p = 0.002, respectively). Myxopapillary ependymoma (MPE) was more frequent than grade II ependymoma (EII). The overall rate of gross-total resection (GTR) was 91.2% and rates were significantly higher for patients with EII (98%) than for those with MPE (84%) (p = 0.0074). On multivariate analysis, the only factor associated with GTR was the presence of a capsule (p = 0.011). Seventeen patients (13.7%) had recurrences (13 MPE, 4 EII; 76.4% vs 23.6%; p = 0.032). The extent of resection was the only factor associated with recurrence (p = 0.0023) and number of surgeries (p = 0.006). Differences in progression-free survival (PFS) were seen depending on the extent of resection at first operation (p < 0.001), subarachnoid seeding (p = 0.041), piecemeal resection (p = 0.004), and number of spine levels involved (3 [p = 0.016], 4 [p = 0.011], or ≥ 5 [p = 0.013]). At follow-up a higher proportion of EII than MPE patients were disease free (94.7% vs 77.7%; p = 0.007). The QOL results were inferior in almost all areas compared to a control group of subjects from the Italian general population. A McCormick grade ≥ 3 and repeated surgeries were associated with a worse QOL (p = 0.006 and p = 0.017). CONCLUSIONS An early diagnosis of CEE is important because larger tumors are associated with recurrences and worse functional neurological outcomes. Surgery should be performed with the aim of achieving an en bloc GTR. The histological subtype was not directly associated with recurrences, but some of the features more commonly encountered in MPEs were. The outcomes are in most cases favorable, but the mean QOL perception is inferior to that of the general population.

Convolutional neural network-based magnetic resonance image differentiation of filum terminale ependymomas from schwannomas

“…Compared with the 2016 CNS WHO classification, MPE is now regarded as CNS WHO grade 2 rather than 1 because the likelihood of recurrence and mean interval of recurrence [ 7 , 33 – 37 ] are now considered similar to those of SPE (WHO II). We found compelling evidence that en bloc GTR significantly reduces the recurrence of FTEs, regardless of the SPE or MPE subtype, based on a 20-year case study [ 10 ]. Although large-scale prospective studies have not been conducted, there is substantial retrospective literature evidence indicating that GTR via an en bloc technique significantly decreases recurrence and prevents repeated surgeries [ 38 – 40 ].…”

“…En bloc resection of the tumour and its surrounding tissue as a single piece is more ideal and leads to an improved prognosis [ 8 , 9 ]. A 20-year study [ 10 ] suggested that en bloc gross total resection (GTR) should be the goal during surgery for cauda equina ependymomas. This approach can reduce recurrence, but there is no significant association between histological subtypes.…”

Spinal Myxopapillary Ependymoma: A Rare Case and Review of Management Strategies

“…Gross total resection (GTR) aims to completely remove the tumor while preserving healthy spinal tissue and is associated with complete resolution of symptoms and lower rates of recurrence [ 15 ]. Studies suggest that well-encapsulated MPEs have a higher success rate of being resected without severe neurological complications [ 9 , 16 ]. However, postoperative neurological deficits, such as permanent bladder and bowel dysfunction and sensory loss, may occur if the tumor's capsule is weak and allows infiltration and penetration by the surrounding nerve roots [ 9 , 17 ].…”

Extra-Neural Metastases of Late Recurrent Myxopapillary Ependymoma to Left Lumbar Paravertebral Muscles: Case Report and Review of the Literature

“…A few common localizations are the pre- or post-sacral regions, in the so-called extraspinal variant. They generally arise in the 4th decade of life and only 8–20% occur in children [ 15 , 16 ]. The World Health Organization (WHO) Classification of Tumor of the Central Nervous System stated that myxopapillary ependymoma typically lacks histopathological signs of malignancy, classifying them as Grade II [ 7 ].…”

“…Histopathological features and genetic and epigenetic features confirm the diagnosis of myxopapillary ependymoma and support the ependymal origin of muscular lesion. Treatment gold standard for symptomatic ependymoma metastasis is complete surgical removal with or without postoperative radiotherapy depending on the extent of disease and the extent of resection [ 16 , 39 ]. Considering all the above, the decision of our spinal and oncologic board was further bolstered by the need of histologically characterize and confirm the nature of the lesion.…”