Background: Papillary tumor of the pineal region (PTPR) is a rare neuroectodermal tumor that was first described in 2003 and formally codified by the World Health Organization in 2007. Limited reports suggest surgical resection is the mainstay of treatment; however, the role of multimodality therapy is not well defined. We evaluated our institutional experience in the treatment of PTPR.
Methods: A retrospective review of 8 patients with pathologically confirmed PTPR diagnosed between 1999 and 2013 was performed.
Results: The median age at diagnosis was 37 years (range, 25-56 years). After a median follow-up period of 60 months (range, 10-170 months), 7 of 8 patients were still living. All patients underwent maximum safe surgical resection; 5 received adjuvant radiation (63%). Overall and progression-free survival 5 years after diagnosis were 100% and 51%, respectively. Progression-free survival 5 years after completion of adjuvant radiotherapy was 64%. Crude recurrence rates for patients receiving adjuvant radiotherapy (n = 5) and patients not receiving adjuvant radiotherapy (n = 3) were 20% and 67%, respectively. Crude recurrence rate after gross total resection (GTR) and no adjuvant radiotherapy (n = 2) was 100% versus 0% when adjuvant radiotherapy was administered after GTR (n = 2). After subtotal resection, 3 patients received adjuvant radiotherapy; 1 of these patients had out-of-field recurrence at 46 months (crude recurrence rate 33%). In all cases, salvage with radiation at the time of recurrence was effective.
Conclusions: Our institutional experience confirms a recent multicenter retrospective series showing excellent survival but high risk of local recurrence for PTPR. Our findings suggest that radiotherapy provides durable local control, particularly when administered in the adjuvant setting after GTR.
Keywords: Pineal region tumor; Radiation; Surgery.
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