jueves, 7 de julio de 2016

Everolimus. Xanthoastrocytoma

LG-03
COMPLETE REMISSION OF AN EXTRA-CRANIALLY DISSEMINATED PLEOMORPHIC XANTHOASTROCYTOMA (PXA) WITH EVEROLIMUS: CASE REPORT AND LITERATURE REVIEW

  1. Mohamed S. AbdelBaki1
+Author Affiliations
  1. 1Nationwide Children's Hospital and The Ohio State University, Columbus, OH USA
  2. 2Faculty of Medicine, Ain Shams University, Cairo, Egypt
BACKGROUND: Pleomorphic xanthoastrocytoma (PXA) is a rare astrocytic tumor often with favorable outcome. Gross total resection, when feasible, confers a 10 year overall survival of 70%. However, features including anaplasia, subtotal resection, metastasis, and recurrence portend a less favorable prognosis. CASE REPORT: A 5 year old male with chronic headaches and history of seizures was admitted to our institution after he was found to have a mass in the right sylvian fissure. He underwent subtotal resection and pathology confirmed PXA with anaplastic features. Over the following four years, despite being treated with multiple chemotherapy regimens and gamma knife radiation, he has had multiple tumor recurrences as well as lung and abdominal metastases. The tumor was found to be positive for a mutation in the TSC (tuberous sclerosis complex) 2 gene. Since TSC regulate mammalian target of rapamycin (mTOR), our patient was started on the (mTOR) inhibitor, everolimus. Currently, he has been successfully maintained on this single oral chemotherapy agent for more than 12 months. Imaging of the brain, chest and abdomen now show no evidence of disease. CONCLUSION: Here we report the first case in the literature of an extra-cranially disseminated PXA with TSC2 gene mutation that demonstrated complete remission of the tumor with everolimus. Further clinical trials are warranted to test the possibility of using mTOR inhibitors in the management of recurrent anaplastic PXA.
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