| Summary: | Background: Papillary intralymphatic angioendothelioma (PLIA) is an extremely rare lymphatic vascular neoplasm characterized by low-grade vascular malignancy that mostly occurs in teenagers and young adults. Case presentation and literature review: A 23-year-old man presented with a large mass in the right cheek that had been progressively increasing in size for 10 years. Contrast-enhanced computed tomography showed a large well-defined tumor (8 cm × 5 cm × 4 cm) in the buccal space and pterygopalatine fossa region. He underwent en bloc resection through the intraoral approach. The definite pathological diagnosis was PLIA. After 2 months, he developed local recurrence, but he refused treatment and was lost to follow-up. A systematic review revealed that among 45 patients with PLIA reported in the literature, 26 were followed up for more than 6 months. Of these patients, 2 (7.7%) developed distant metastasis, and 1 (3.8%) developed local recurrence. Conclusion: PLIA has a favorable overall prognosis, but radical resection through the appropriate approach is the most important key to prevent local recurrence.
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