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Massimo Miscusi, Eliana Gilioli, Franco Faccioli, and Albino Bricolo

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Nicolò Marchesini, Nicola Tommasi, Franco Faccioli, Giampietro Pinna, and Francesco Sala

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.

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Mario Ganau, Andrea Talacchi, Paolo C. Cecchi, Claudio Ghimenton, Massimo Gerosa, and Franco Faccioli

Object

The ventriculus terminalis, an embryological remnant consisting of the ependymal-lined space of the conus medullaris, can occasionally become symptomatic after cystic dilation. In the existing literature, consisting of 32 cases, the preferred type of management (conservative vs surgical) is still debated. The object of this study was to report the surgical results in a consecutive series of 10 adult patients with cystic dilation of the ventriculus terminalis (CDVT), to match them with data retrieved from the relevant literature, and specifically to validate a new recent clinical classification.

Methods

The authors reported 13 new cases of CDVT treated in the Department of Neurosurgery at University Hospital in Verona, Italy. Treatment modalities and clinical and radiological outcomes, both early and at follow-up, were analyzed and compared with a preoperative classification of clinical presentation, as established by de Moura Batista and colleagues (2008).

Results

Surgical treatment seemed to guarantee the resolution of CDVT. Dorsolumbar laminotomy, myelotomy, and cystic drainage were performed in 10 patients. Patients with Type I symptoms (nonspecific complaints) often presented with comorbidities (herniated disc or facet hypertrophy) confusing their clinical status. The surgical treatment of patients with Type I symptoms promoted good results only if the diagnosis of CDVT was definitive and symptoms had rapidly evolved. In patients with Type II (focal neurological deficits) and III (sphincter disturbances) symptoms, surgical treatment sustained improvement even at the late follow-up.

Conclusions

While confirming the usefulness of de Moura Batista and colleagues' classification in its impact on prognosis, the authors propose a revision of the classification with subgroups Type Ia (nonspecific symptoms without clear relation to CDVT), which is best treated conservatively, and Type Ib (rapid onset and invalidating unspecific complaints without comorbidities), which may benefit from surgical evacuation.