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Abstract Details

Pineal parenchymal tumors of intermediate differentiation treated with ventricular radiation and temozolomide
Neuro-oncology
P1 - Poster Session 1 (5:30 PM-6:30 PM)
7-017
To describe a single-institution experience treated PPTID with ventricular radiation, focal radiation boosts, and temozolomide
Pineal parenchymal tumors of intermediate differentiation (PPTID), first defined by the World Health Organization in 2000, represent a very rare class of central nervous system malignancy.    They have an intermediate grade between WHO grade I pineocytomas and WHO Grade IV pineoblastomas.  Pineocytomas have a low recurrence rate and are typically treated with surgery alone.  Pineoblastomas behave much more aggressively and have a propensity for craniospinal spread.  Therefore, treatment includes multimodality therapy with myeloablative chemotherapy and craniospinal irradiation.  However, there is no consensus on the optimal treatment of PPTID.  While these tumors have an approximately 25% risk of leptomeningeal spread, craniospinal irradiation carries significant associated morbidity. At our institution we have treated PPTID patients with ventricle radiation followed by stereotactic  boosts to the pineal area and resection bed with the goal of reducing the risk of CNS dissemination and local recurrence while limiting toxicity.  We additionally treat with concurrent temozolomide using a modified Stupp protocol.  Here, we present our institutional experience treating 3 PPTID patients. 
Case series

Of our three patients treated with  one suffered recurrent disease and died 4 years after initial diagnosis.  The other two patients show no evidence of disease recurrence 2 and 6 years after initial diagnosis and show no significant treatment-related toxicity. 

These cases show that postoperative ventricular radiation plus focal boosts combined with temozolomide may be a viable treatment strategy in PPTID.

Authors/Disclosures
Justin T. Low, MD, PhD (Duke University School of Medicine)
PRESENTER
Dr. Low has nothing to disclose.
No disclosure on file
Katherine B. Peters, MD, PhD, FAAN (Duke University Medical Center) Dr. Peters has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Servier. Dr. Peters has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sapience. Dr. Peters has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for ONO Pharmaceutical. Dr. Peters has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Telix. Dr. Peters has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for AnHeart. Dr. Peters has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Rigel. Dr. Peters has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Nuvation. The institution of Dr. Peters has received research support from Biomimetix. The institution of Dr. Peters has received research support from Servier. The institution of Dr. Peters has received research support from Varian. The institution of Dr. Peters has received research support from Sapience. The institution of Dr. Peters has received research support from Ono Pharmaceuticals/Deciphera. The institution of Dr. Peters has received research support from Nuvation. The institution of Dr. Peters has received research support from Novocure. The institution of Dr. Peters has received research support from Curis.