Long-term survival of a sacro-coccygeal myxopapillary ependymoma with extra-neural metastases: case report and review of
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LETTER TO THE EDITOR
Long-term survival of a sacro-coccygeal myxopapillary ependymoma with extra-neural metastases: case report and review of the literature Francesco Bruno 1
&
Alessia Pellerino 1 & Luca Bertero 2 & Riccardo Soffietti 1 & Roberta Rudà 1
Received: 10 November 2019 / Accepted: 20 January 2020 # Fondazione Società Italiana di Neurologia 2020
Myxopapillary ependymomas (MPEs) are grade I tumours, accounting for 9–13% of all ependymomas [1]. Even if they typically emerge from conus medullaris and cauda equina, extra-CNS MPEs have also been described, the sacrococcygeal region being the most common location [2]. Although they predominantly show a benign course, MPEs may recur locally and metastasize, via both CSF (to other CNS sites) and blood (to extra-CNS organs), even in early stages of the disease [2]. Tendency to recurrence and spreading is strongly related to incomplete resection and capsule violation [3]; therefore, treatment should be based on surgery (as large as possible), and radio-/chemotherapy in case of partial resection and/or recurrence [4]. Here, we present a unique case of long-surviving myxopapillary ependymoma of the sacro-coccygeal region with lung and skin metastases treated with multiple lines of chemotherapy. A 32-year-old woman was diagnosed with a sacro-coccygeal tumour in 1995. She underwent a subtotal resection with a diagnosis of ‘grade I WHO myxopapillary ependymoma’. Surgery was followed by conformal radiotherapy (55 Gy) due to the presence of residual tumour. Thus, the tumour remained stable for 20 years. In February 2015, the patient presented with back pain: the MRI showed a local recurrence, with no involvement
Presented at the 13th Meeting of the European Association of NeuroOncology (EANO), hosted in Stockholm in October 2018. * Francesco Bruno [email protected] 1
Neuro-oncology Unit, Department of Neurosciences, University & City of Health & Science Hospital, via Cherasco 15, 10121 Turin, Italy
2
Pathology Unit, Department of Medical Sciences, University & City of Health & Science Hospital, Turin, Italy
of brain or spinal meninges, and the biopsy confirmed the diagnosis of MPE. However, a major surgical excision was not feasible due to the tumour’s extension (Fig. 1). Meantime, some lung nodular lesions were seen in a staging CT scan, but both fluodeoxyglucose (FDG) whole-body PET and bronchial washing were negative. For this reason, and due to depth and small size of the lesions, biopsy of lung nodules was not carried out. In May 2015, the patient came to our Department of Neuro-Oncology to receive medical treatment. Based on our experience in recurrent ependymomas [5], we chose to employ temozolomide (200 mg/m2 5 days per months): 13 cycles were administered from May 2015 to May 2016, yielding relief of the back pain and stabilization of the sacrococcygeal and lung lesions. Chemotherapy did not cause any haematological adverse events and was well tolerated. In September 2016, the tumour relapsed in the sacrococcygeal region. At the same time, the patient de
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