Oral and maxillofacial pathologyOnline only articlePrimary central carcinoma ex pleomorphic adenoma of the mandible: report of a rare case and literature review

We report a rare case of primary carcinoma ex pleomorphic adenoma (CXPA) of the mandible. A 13-year-old girl presented with a large mass, measuring about 30 cm in its greatest dimension, involving the mandible. She was referred to our department for surgery, and her postoperative course was uneventful; she is currently free from the disease 48 months after surgery. Primary CXPAs located centrally within the jawbones are rare with only 10 cases reported in the English and Chinese literature. This case illustrates 2 key facts regarding the diagnosis and therapy of CXPA. First, clinicians should be aware of this possibility and should emphasize the need for submission of so-called cystic lytic lesions for histopathologic analysis. Second, tumors should be excised en bloc with adjuvant postoperative radiotherapy when local recurrence and regional metastasis are suspected clinically.