Long-Term Clinical Outcome in Familial and Sporadic Papillary Thyroid Carcinoma

Background:The definition and the behaviour of familial papillary thyroid cancer (FPTC) compared to the sporadic form (SPTC) are still debated. Some authors believe that only families with 3 or more affected members represent an actual example of familial diseases.Objectives:The objective of the study was to analyse the clinicopathological features and the outcome of sporadic and familial PTC patients also according to the number of affected members.Methods:Among 731 patients, we identified 101 (13.8%) with familial diseases, 79 with 2 affected members (FPTC-2) and 22 with 3 or more affected members (FPTC-3) followed for a mean period of 10 years.Results:FPTC patients had more frequently bilateral tumour (p= 0.007). No difference was found between the 2 groups for the other evaluated variables. At the time of the first follow-up (1-2 years after initial therapy), FPTC patients had a higher rate of persistent disease. However, at the last follow-up, the clinical outcome was not different between sporadic and familial patients. When the comparison between SPTC and FPTC was performed, according to the number of affected members, a significant trend between the 3 groups was observed for tumour diameter (p= 0.002) and bilaterality (p= 0.003), while we did not observe a significant trend for both response to initial therapy (p= 0.15) and last clinical outcome (p= 0.22).Conclusions:Our results suggest that, although the clinicopathological features of FPTC may be more aggressive, the long-term outcome is similar between FPTC and SPTC. A possible explanation is that PTC has a favourable prognosis, even when clinical presentation is more aggressive.