P05.49 Extra-neural metastases from a sacro-coccygeal myxopapillary ependymoma: a case report

Myxopapillary ependymoma (MPE) is a subtype of ependymal tumour of grade I WHO arising in the conus medullary, cauda equina and filum terminale. The prognosis is relatively favourable, although MPE may recur and spread in the leptomeninges. Extra-neural metastases are rare, but occasionally described. Here, we report a unique case of subcutaneous and lung metastases from a MPE of the sacro-coccygeal region who underwent multiple lines of therapy. A 53 year-old woman was diagnosed with a sacro-coccygeal MPE in 1997. She underwent subtotal resection and adjuvant radiotherapy with residual tumour stabilization until 2012, when she experienced lumbar pain and the MRI showed signs of local progression. CT showed multiple lung lesions, without FDG (fluoro-deoxy-glucose) PET positivity, that were considered too small for a biopsy. Therefore, chemotherapy with temozolomide was started, based on literature data in ependymomas. A good clinical and radiologic disease control was obtained, both at the primary site and the lung level for 18 months, when temozolomide was stopped. 6 months after interrupting temozolomide, we observed a local sacro-coccygeal recurrence and the appearance of a subcutaneous nodule in the gluteus, with stable lung lesions on CT and FDG-PET. The biopsy of the cutaneous nodule confirmed the diagnosis of MPE localization. Intensified temozolomide was started with further disease progression both in the sacro-coccygeal site and lung with multiple active lesions, associated with symptomatic and drug resistant chronic cough. A CT-guided biopsy of lung nodules confirmed the diagnosis of MPE. Thus, the patient started II line chemotherapy with an association of platinum and lomustine, of which 8 cycles have been administered with radiologic stabilization of all lesions. To date, no further recurrences have been observed. We present a case of late multiple extra-neural metastatic MPE that displayed a successful stabilization following chemotherapy.