Carcinoma ex pleomorphic adenoma: A clinicopathologic review

Background. Carcinoma ex pleomorphic adenoma (CXPA) is an aggressive, poorl y understood salivary gland malignancy. Misdiagnosis is common, because the residual mixed tumor component may be small, and various carcinoma subtype s are possible. Methods. We retrospectively reviewed the medical records of 73 patients wit h major salivary gland CXPA treated at our institution from 1960 to 1994. O f the 73 patients, 66 had primary tumors and 7 had recurrent tumors; 47 wer e men, and 26 were women; the mean age was 61 years. Results. Adenocarcinoma (31 cases) and salivary duct carcinoma (24 cases) w ere the most common malignant subtypes, All patients were treated surgicall y, and 32 also had radiation therapy. Of 66 patients with primary tumors, 2 3% had local recurrence. Metastasis (either initial or delayed) occurred re gionally in 56%. and distantly in 44%. Thirty-six patients (55%) died of th e disease, At 3 years, overall survival was 39% and at 5 years, 30%. Conclusions. Important prognostic factors include tumor size, grade, and cl inical and pathologic stage. Patients with minimally invasive tumors (<5 mm ) should do well with appropriate surgical treatment. (C) 2001 John Wiley & Sons, Inc.