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Long term outcomes following surgical resection of myxopapillary ependymomas

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Abstract

Myxopapillary ependymomas, a specific tumor variant of spinal cord ependymomas, occur most commonly in the lumbosacral region. During the study period, 1,013 patients underwent surgery for spinal cord tumors. Fifty-two of the patients had a myxopapillary ependymoma. Forty-eight of these patients underwent surgery at our institutions. There were four patients who came for consultations only. Fourteen pediatric patients were diagnosed with myxopapillary ependymoma. The overall average age at which a patient was diagnosed was 31.8 years. The average age a child was diagnosed was 12.6 years. The adult mean age was 38.7 years. The clinical presentation was of a slow, indolent course, with average symptom duration of 20.8 months. Overall, the pediatric patients had a much more aggressive clinical course with a much higher rate of local recurrence and dissemination of the tumor within the neural axis (64% versus 32%). The median time to disease recurrence was 88 months for the entire group. The overall survival after 11.5 years of follow-up was 94%. The optimal management of patients harboring myxopapillary ependymomas remains somewhat controversial. Excellent outcomes may be obtained, however, with the use of aggressive surgical techniques. No clear benefit for adjunctive chemotherapy, and radiation therapy was demonstrated.

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Authors and Affiliations

  1. Division of Neurosurgery, Duke University School of Medicine, Durham, NC, USA

    Carlos A. Bagley

  2. Department of Medicine, Yale University School of Medicine, New Haven, CT, USA

    Sean Wilson

  3. Division of Neurosurgery, Kantonsspital Luzern, Lucerne, Switzerland

    Karl F. Kothbauer

  4. Department of Neurosurgery, Temple University school of Medicine, Philadelphia, PA, USA

    Markus J. Bookland

  5. Department of Neurosurgery, Institute for Neurology and Neurosurgery, Beth Israel Medical Center, New York, NY, USA

    Fred Epstein

  6. Department of Neurosurgery, Johns Hopkins University School of Medicine, Harvey 811, Johns Hopkins Hospital, 600 North Wolfe Street, Baltimore, MD, 21287-7713, USA

    George I. Jallo

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  1. Carlos A. Bagley
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  2. Sean Wilson
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Comments

Kazuhiro Hongo, Matsumoto, Japan

This is a paper on the long-term outcomes following surgical resection of myxopapillary ependymomas with extensive analysis of 48 surgical cases. The authors meticulously analyzed the cases and discussed. They stressed the importance of maximum surgical resection by mentioning that excellent outcomes may be obtained with the use of aggressive surgical techniques and also that no clear benefit for adjunctive chemotherapy and radiation therapy was demonstrated.

The myxopapillary ependymoma is histologically benign; however, it may involve the conus medullaris and nerve roots may penetrate the tumor, as the authors mentioned. Meticulous removal under high magnification is required to obtain maximum resection with maintaining the surrounding neural structures.

Ignacio J. Previgliano, Buenos Aires, Argentina

This is an important paper regarding myxopapillary ependymomas not only for the number of cases but also for the exhaustive literature review. The authors are also very honest showing a detailed clinical summary of all their patients, including complications.

I found three important messages:

1. Although infrequent, these tumors are aggressive and recurrent regardless the treatment, as is demonstrated by the 40% of recurrence or dissemination in this study and a nearby percentage in the literature review.

2. Gross surgical resection is still the better approach to achieve good outcomes and less recurrence or metastases. This seems to be true for children and adults, with a clear trend of symptoms free time in children.

3. There is no strong evidence to use adjunctive therapy after gross resections. Even though in the Kaplan Meier survival curve (Fig. 3), there is no significance; a clear trend for recurrence in the nonradiated group is shown. Probably a greater sample will help in addressing the question.

Nevertheless, the authors’ commentary on the selection bias of radiotherapy-treated patients should be bore in mind.

I think that this kind of tumors are a challenge for the spinal cord surgeons and that surgery is one of those in which technical skill, respectful management of nerve roots, and aggressive resection can make a difference on patient functional outcome and survival.

Fred Epstein is deceased.

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Bagley, C.A., Wilson, S., Kothbauer, K.F. et al. Long term outcomes following surgical resection of myxopapillary ependymomas. Neurosurg Rev 32, 321–334 (2009). https://doi.org/10.1007/s10143-009-0190-8

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