paediatrics Brussels 17

Abstract

This study was undertaken to determine the

disease outcomes in patients treated with surgery alone

or surgery and adjuvant radiotherapy (RT) for myxo-

papillary ependymoma (MPE) of the spine. The med-

ical records of 35 patients with MPE treated at The

University of Texas M.D. Anderson Cancer Center

between December 1968 and July 2002 were reviewed.

The endpoints analyzed were progression-free survival

(PFS), overall survival, and local control. The median

age of patients was 35 years (range, 14–63 years), and

the male to female ratio was 2.5:1. In total, 21 (60%)

patients underwent a gross total resection, 13 (37%) a

subtotal resection, and 1 (3%) a biopsy only; 22 of

them (63%) also received adjuvant RT. The median

follow-up was 10.7 years. The 10-year overall survival,

PFS, and local control rates for the entire group were

97%, 62%, and 72%, respectively. Of 11 patients, 5

(45%) who had undergone gross total resection alone

had recurrence. A total of 12 (34%) patients had dis-

ease recurrence, all in the neural axis; 8 of them had

treatment failure at the primary site only, 3 in the

distant neural axis only, and 1 at the primary site and in

the distant neural axis. Patient age (> 35 years;

= 0.002) and adjuvant RT (

= 0.04) significantly

affected PFS. The long-term patient survival duration

for MPE managed with surgery and adjuvant RT is

favorable. Regardless of the extent of resection, adju-

vant RT appears to significantly reduce the rate of

tumor progression. Failures occurred exclusively in the

neural axis, mainly at the primary site.

Keywords

Myxopapillary ependymoma

Ependymoma

Spinal tumor

Surgery

Radiotherapy

Introduction

Ependymomas are the most common intraspinal

tumors [1], representing 15% of spinal cord tumors and

up to 60% of spinal cord gliomas [2, 3]. Myxopapillary

ependymomas (MPEs), first described in 1932 by

Kernohan [4], are a distinctive variant of ependymo-

mas both clinicopathologically and genetically [5, 6].

According to the World Health Organization classifi-

cation of central nervous system tumors, MPEs are

grade I tumors [7]. MPEs are usually histologically

benign, often encapsulated, and slow-growing tumors

with a long disease course. The incidence of MPE is

low; in a large series of cases of ependymomas, only

13% were found to be of the myxopapillary type [8].

Most MPEs occur in the lumbosacral/cauda equina

region. Occasionally, MPEs arising from other sites in

the spinal cord, from the intracranial region, or from

the subcutaneous soft tissues in the sacrococcygeal

region have been described [9–13].

Most spinal ependymoma series published in the

literature have included only patients with spinal

S. Akyurek

E. L. Chang (

)

T.-K. Yu

D. Little

P. K. Allen

A. Mahajan

M. H. Maor

S. Y. Woo

Department of Radiation Oncology, The University of

Texas M.D. Anderson Cancer Center, 1515 Holcombe

Blvd., # 97, Houston, TX 77030, USA

e-mail:

echang@mdanderson.org

S. Akyurek

Department of Radiation Oncology, Ankara University

School of Medicine, Ankara, Turkey

I. McCutcheon

Department of Neurosurgery, The University of Texas M.D.

Anderson Cancer Center, Houston, TX 77030, USA

J Neurooncol (2006)

DOI 10.1007/s11060-006-9169-2

123

ORIGINAL PAPER

Spinal myxopapillary ependymoma outcomes in patients treated

with surgery and radiotherapy at M.D. Anderson Cancer Center

Serap Akyurek

Eric L. Chang

Tse-Kuan Yu

Darrin Little

Pamela K. Allen

Ian McCutcheon

Anita Mahajan

Moshe H. Maor

Shiao Y. Woo

Received: 9 March 2006 / Accepted: 29 March 2006

Springer Science+Business Media B.V. 2006