Pediatric myxopapillary ependymoma treated with subtotal resection and radiation therapy: a case report and review of the literature

Abstract

Study design: Case report and review of the literature. Objectives: Myxopapillary ependymoma (MPE) is a relatively rare glioma that develops from the spinal part of the filum terminale, usually in adulthood. While it is generally benign, MPE can disseminate intraspinally, and this malignant behavior requires a multidisciplinary response with surgery and radiotherapy. We report here a case of MPE occurring in the lumbosacral spine area of an 8-year-old boy. Setting: Japan, Tokyo. Methods: We report here a case of MPE, treated with subtotal surgical resection followed by craniospinal irradiation (CSI), in an 8-year-old boy. The patient was referred to our hospital with a 6-month history of severe pain in the lower back and legs, paralysis of the legs and dysuria. Magnetic resonance imaging images showed a large tumor that filled the entire spinal canal below L1. After subtotal resection of the tumor, the pathological findings established a diagnosis of MPE. Since the tumor had perforated its capsule, increasing the risk of intraspinal dissemination, the patient underwent radiotherapy and CSI after surgery. Results: Magnetic resonance images obtained 3 years after the surgery did not show any recurrence of MPE. Conclusion: Although tumor resection followed by CSI can be considered an effective strategy for treating a child with MPE, long-term follow-up is necessary to ensure early detection of any local recurrence or dissemination of the tumor, or of post-radiotherapy scoliosis.

Myxopapillary Ependymoma with Drop Metastasis in a Young Child

“…Seven cases of MPE in less than 15 years of age treated with cranio-spinal irradiation had been reported in the literature [6][7][8][9]. Four patients had metastatic lesions in the spinal canal at the time of presentation as in our case and three patients had localized disease.…”

An Overview of Central Nervous System Tumours

“…This aggressive behaviour is most commonly seen in children [84,85]. MPEs can be cured if all of the tumour has been removed during surgery (total resection) [86] and usually presents with an excellent prognosis.…”

Outcomes following limited‐volume proton therapy for multifocal spinal myxopapillary ependymoma

“…When treating multifocal MPE in children, the rationale for CSI is understandable and advocated by several authors. 12,24,25 However, the late toxicities associated with CSI can be severe in children, including a profound impact on growth and development extending to neurocognitive deficits, hormonal deficiencies, short stature, and other effects. 42,43 Reducing radiation exposure to normal tissue should always be a primary goal when radiotherapy is administered to children.…”

“…This reduction in dose may decrease both acute and late toxicities, including the risk for secondary malignancies. In limited series, craniospinal irradiation (CSI) has been employed in multifocal spinal MPE with success 12,24,25 . However, even with proton therapy, the long‐term effects of the whole brain component of CSI are undesirable in patients with benign tumors and excellent long‐term survival rates.…”

“…In limited series, craniospinal irradiation (CSI) has been employed in multifocal spinal MPE with success. 12,24,25 However, even with proton therapy, the long-term effects of the whole brain component of CSI are undesirable in patients with benign tumors and excellent long-term survival rates. Bearing this in mind, our institution's systematic approach has been limited-volume irradiation for multifocal spinal MPE.…”