Paediatric Intracranial Anaplastic Ependymoma: The Role of Multiple Surgical Resections for Disease Relapse in Maintaining Quality of Life and Prolonged Survival

Abstract

Introduction: Ependymoma is the third most common intracranial glioma in children. The treatment of choice for these tumours remains gross total resection followed by radiotherapy. There are two principal histological subtypes, namely classic (∼70%) and anaplastic (∼30%) ependymoma. Case: We present the case of a 12-year-old girl with an anaplastic ependymoma of the left temporal lobe. She underwent initial image-guided resection following biopsy. A postoperative MRI showed a macroscopic resection. She subsequently relapsed and indeed had 11 local and distant relapses managed by 12 separate craniotomies and tumour resection, 4 courses of radiotherapy and chemotherapy. Conclusion: For patients with multiple relapses, surgery should be considered primarily to re-resect any symptomatic lesion. This case demonstrates that multiple tumour resections can be undertaken with limited morbidity for the patient and with maintenance of quality of life. Repeated focal irradiation can also be used to control the disease with limited morbidity.

Pediatric Ependymoma

“…114 Next line treatment is often repeat surgical resection with a goal of maximal resection, which can offer a limited chance for cure and, more likely, at least a longer overall survival with an improvement in quality of life. 115 Several series also have reported a benefit with reirradiation ranging from local hypofractionated to full dose craniospinal radiation. 116 –119…”

MRI Features of Intracranial Anaplastic Ependymomas: A Comparison of Supratentorial and Infratentorial Lesions

“…In our series, we observed a similar higher prevalence in male compared with female patients. Compared with infratentorial neoplasms, supratentorial ependymomas exhibit an increased occurrence ratio among WHO grade III tumors and commonly occur in the brain parenchyma (6,9,14). In the present cases, most SAEs (91.7%) are extraventricular in origin.…”

“…Given that studies of anaplastic ependymoma are limited by its low incidence, the accuracy of preoperative diagnosis is very low. Several previous reports of anaplastic ependymomas have described their imaging features ( 2 , 6 – 8 ), and most of these studies were case reports. Similar to grade II ependymomas, intracranial anaplastic ependymomas are also located infratentorially or supratentorially.…”

Current and Emerging Methods of Management of Ependymoma

“…As complete surgical resection has such a significant value in the long-term prognosis of ependymoma, it is not surprising that the value of 'second-look' surgery has been evaluated by a number of groups (13,(34)(35)(36)(37)(38). This is particularly relevant if tumour has been inadvertently overlooked or the resection was discontinued as a result of blood loss.…”

“…Indeed, multiple surgical resections can be carried out in certain patients, whilst limiting cumulative morbidity (36). This aggressive surgical strategy was seen in the seminal St. Jude study referred to above (13), in which 43% of patients underwent multiple resections.…”