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Pediatric ganglioneuroma, although benign, is typically treated by surgical resection. However, it is often asymptomatic and an incidental finding. We present an incidental multifocal ganglioneuroma diagnosed within the appendix and the subsequent treatment for this lesion. This intraoperative discovery led to the diagnosis of PTEN (phosphatase and tensin homolog) hamartoma syndrome which had otherwise gone undiagnosed

Multifocal appendiceal ganglioneuroma as the presenting symptom in a patient with PTEN hamartoma syndrome