Prognosis and predictive factors

In general, the recommended therapy is wide local excision with contiguous lymph node dissection. Adjuvant radiation therapy is recommended for widely invasive tumours. If the carcinomatous component is low-grade and/or minimally invasive and if the tumour is adequately excised, then adjuvant radiation therapy may not be necessary. Patients with non-invasive or minimally invasive Ca-ex-PA typically have an excellent prognosis, similar to benign pleomorphic adenoma. Metastatic spread is exceptional {726}. Invasive Ca-ex-PAs, as a group, are extremely aggressive malignancies with approximately 23-50% of patients developing one or more recurrences {786, 898,1491,1533}. The metastatic rate varies with each series; up to 70% of patients develop local or distant metastasis {877,898,1491}. Metastatic sites in order of frequency are lung, bone (especially spine), abdomen and central nervous system {786,2592}. Ca-ex-PA with capsular penetration of more than 1.5 mm is associated with a poor prognosis; survival rates at 5, 10, 15, and 20 years range from 25-65%, 18-50%, 10-35%, and 0-38%, respectively {786,877,1491, 1533,2592,2624}. Therefore, it is important to designate those Ca-ex-PA that are confined within the capsule and those invading through the capsule as non-invasive or invasive, respectively, and to differentiate within the latter group between widely invasive and minimally invasive tumours.

One study showed that no patient with less than 8 mm invasion from the capsule died from the tumour, whereas all patients with invasion greater than 8 mm beyond the capsule ultimately died of disease {2624}. The local recurrence rate (LRR) in this latter series also correlated with extent of invasion; a LRR of 70.5% was found for tumours with invasion beyond 6 mm from the capsule, as compared to a LRR of 16.6% for tumours with invasion of less than 6 mm. In another study consisting of four patients with 5 mm of invasion beyond the tumour capsule, two died of disease and two were alive and well {1491}. The two patients with less than 5 mm of invasion (2 and 3 mm) were alive and well with no evidence of disease. Also, all four patients with intracapsular carcinoma were alive and well without evidence of disease progression.

The improved prognosis for minimally invasive tumours has been confirmed by Brandwein et al who observed recurrence free for periods ranging from 1-4 years (mean 2.5 years) {252}. Tumour size and grade are also significant prognosticators in the more widely invasive Ca-ex-PAs. The five-year survival rates have been correlated with his-tologic subtype of the carcinoma component: there was a 30% survival rate for undifferentiated carcinomas, 50% for myoepithelial carcinomas, 62% for ductal carcinomas and 96% survival rate for terminal duct carcinomas {2624}. In addition, 63% of patients with high-grade carcinomatous components died of the disease, while patients with lower grade carcinomatous elements did not {1491}.

Carcinoma ex pleomorphic adenoma 243

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