INNV-17. EXTRANEURAL METASTASES OF MYXOPAPILLARY EPENDYMOMA TREATED WITH DOSE-DENSE TEMOZOLOMIDE AND LAPATINIB

Abstract

Abstract Myxopapillary ependymomas (MPEs) are recently reclassified WHO grade II tumors that usually affect the sacral spinal cord. MPEs have a high recurrence rate, especially if gross total resection (GTR) is not achieved. Recurrence tends to be local, but distant CNS metastases are not uncommon. Rarely, MPEs can metastasize to extraneural locations, including the lungs. Patient outcomes are worse with recurrence, and there is an absence of consensus for the best treatment approach for extraneural MPE metastases. The standard of care for initial MPE diagnosis is GTR, or subtotal resection (STR) with radiotherapy. Systemic treatment has shown inconclusive results regarding improvement of MPE outcomes. A recent phase II clinical trial demonstrated the efficacy of using dose-dense temozolomide (TMZ) and lapatinib for the treatment of metastatic, recurrent ependymoma. We present a case of a 35-year-old female with a history of MPE status post GTR who developed extraneural metastases 11 years after her initial resection. Sites of metastases included multiple bilateral intrapulmonary and pleural based masses with pleural effusion and pelvic mass. Her original site of disease had no evidence of recurrence and imaging of the neuro-axis was negative. Biopsy of the lung mass and pelvic mass confirmed metastatic MPE. She proceeded with dose-dense TMZ and lapatinib and had a positive radiographic response after 3 cycles of treatment. This is the first known case of extraneural metastases of MPE to demonstrate response to dose-dense TMZ and lapatinib.