Myxopapillary ependymomas in children: imaging, treatment and outcomes

Abstract

Myxopapillary ependymomas (MPEs) are rare spinal tumors in children. The natural history and clinical course of pediatric MPEs are largely unknown and the indication for adjuvant therapy remains to be clarified. We performed an IRB-approved, retrospective review of children with MPEs treated at the Dana-Farber/Boston Children's Cancer and Blood Disorder Center between 1982 and 2013. Eighteen children (age range 8-21 years, median age 14 years) met inclusion criteria. We reviewed the histopathology, magnetic resonance imaging, tumor location and stage, surgical management, adjuvant therapy, and clinical outcomes. The median follow-up duration was 9.4 years (range 1-30 years). Children most commonly presented with pain, scoliosis, and urinary symptoms. All primary tumors were located in the lower thoracic or lumbar spine. Nine children (50%) had leptomeningeal tumor seeding at presentation, most commonly located within the distal thecal sac. A gross-total resection was achieved in nine children (50%). Three children were treated with irradiation following initial surgery. No child received adjuvant chemotherapy at diagnosis. The 10-year event-free survival (EFS) was 26% ± 14.8. Children with disseminated disease trended towards inferior EFS compared to those with localized disease (10-year EFS 12.7% ± 12 vs. 57 ± 25%, p value 0.07). The 10-year overall survival was 100%. The efficacy of adjuvant irradiation could not be assessed due to the small sample size. Although children with MPEs frequently present with disseminated tumor and/or develop recurrent or progressive disease, their overall survival is excellent. Treatment should aim to minimize both tumor- and therapy-related morbidity.

EANO guidelines for the diagnosis and treatment of ependymal tumors

“…MPE is very rare in children. Although patients frequently present with disseminated tumor and/or develop recurrent or progressive disease following treatments, 115 the OS at 5 and 10 years in the SEER database is estimated at 97% and 95%, respectively. 116 A recent series from Johns Hopkins Hospital 117 indicated a significant reduction in local failure for patients receiving radiotherapy following STR or GTR.…”

Concomitant Double Tumors of Myxopapillary Ependymoma Presented at Cauda Equina-Filum Terminale in Adult Patient

“…MPE is iso-intense on T1- and hyper-intense on T2-weighted MRI with heterogenous imaging properties on T2-weighted scans. These heterogeneities correspond to hemorrhages and calcifications present in the tumor 3 12) .…”

Invasive myxopapillary ependymoma of the lumbar spine: A case report

“…Both a complete neurological examination and MRI are equally important to precisely delineate the disease[3,13]. Ependymomas may present with lumbar or radicular pain, weakness and urinary symptoms[14]. However, many patients have a long history of nonspecific complaints prior to the clinical presentation, owing to a slow growth of the myxo-papillary ependymoma.…”

“…The treatment aim is to minimize both tumour and therapy-related morbidity. Usually, it encompasses different modalities[9,14]. It generally involves surgical treatment with or without adjuvant radiotherapy, which is most commonly used in patients with subtotal resection of intradural ependymomas, local recurrence or CNS dissemination.…”