• L Jiwang, L Zhendong, L Shuchun, H Bo, and L Yanguo.
• Department of Head and Neck Surgery, Liaoning Cancer Hospital & Institute, Shenyang, People's Republic of China;
• Acta Otorhinolaryngol Ital. 2015 Oct 1; 35 (4): 234-42.

AbstractIt is unclear whether familial non-medullary thyroid carcinoma (FNMTC) is more aggressive than sporadic carcinoma, and its prevalence is still under debate. In this study, we investigated the clinicopathologic features of familial papillary thyroid carcinoma (PTC) compared with its sporadic counterpart. We used data from our hospital between 2008 and 2014 to compare the features of 24 familial PTC with 80 sporadic PTC. The prevalence of familial PTC was 1.5%; 25% of familial PTC exhibited a parent-offspring relationship, and 75% exhibited a sibling relationship. There were significant differences in terms of Hashimoto's thyroiditis, nodular goiter, multicentricity, bilaterality, histologic variant, T stage and N stage between the familial and sporadic PTC groups (all p < 0.05). When we compared sporadic PTC with parent-offspring or sibling familial PTC separately, parent-offspring familial PTC was more Hashimoto's thyroiditis and central LNM, while sibling familial PTC was more prevalent in multifocality and bilaterality than sporadic PTC. The recurrence rate was not significantly higher than that of sporadic PTC in familial PTC. The second generation in parent-offspring familial PTC patients exhibited an earlier age at diagnosis, greater multifocality and a higher metastasis rate than the first generation. Based on our results, we conclude that familial PTC is a clinically distinct entity with an aggressive nature. Because of the frequent presence of benign nodules, multifocality, bilaterality and high rate of recurrence, total or near-total thyroidectomy with neck dissection in these patients might be recommended. To date, the optimal clinical treatment is yet to be established, but improved awareness and screening will permit earlier detection, more timely intervention and improved outcomes for patients and their families.

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