Single-center study of familial papillary thyroid cancer in China: surgical considerations

Abstract

BackgroundWhether familial papillary thyroid cancer (FPTC) is more aggressive than sporadic counterpart remains elusive, and the optimal clinical approach for FPTC is yet to be established. In this study, we investigated familial occurrence of PTC in China and reviewed our experience of its surgical treatment.MethodsThe clinical records of 248 consecutive patients with an established diagnosis of PTC who were admitted to Nanfang Hospital for thyroidectomy between January 2011 and June 2013 were analyzed in this study. Patients included 66 males and 182 females, aged 11 to 76 years.ResultsTwenty-two patients (8.9%) with a positive family history were confirmed. Patients with FPTC had a predilection for female subjects and tended to be younger than other patients, but the difference was not significant (P = 0.0514 and P = 0.168). They were more likely to present large tumors (P = 0.0024), multifocality (familial vs. sporadic: 54.50% vs. 26.50%; P < 0.006), local invasion (81.8% vs. 23.9%; P < 0.001), and malignant lymph nodes (63.6% vs. 33.6%; P = 0.005). Univariate and multivariate analyses identified that a positive family history was an independent risk factor for local invasion (OR: 5.683; 95% CI: 2.056 to 15.707; P = 0.001), malignant lymph nodes (OR: 3.005; 95% CI: 1.046 to 8.630; P = 0.041) in FPTC patients. Kaplan-Meier survival curves revealed that an aggressive surgical strategy was associated with a better relapse-free survival than conventional one (P = 0.032).ConclusionsFPTC is more likely to possess aggressive features than sporadic counterparts. Thus, screening of at-risk families is essential to aid in earlier recognition. An aggressive surgical strategy appeared to be the more effective therapy. However, sufficient detailed interrogation and long-term follow-up of the patients and their family are necessary for providing individualized recommendations for clinical management.

The clinicopathologic characteristics of familial and sporadic papillary thyroid carcinoma in Turkish patients

“…Some groups report a lower age at diagnosis in familial cases [1,13]. However, we did not report a significant difference in age between familial and sporadic cases, like many others [3,[8][9][10][11][12]. This may be related to missed screening following the diagnosis in the other family members.…”

“…Most authors agree that fPTC is more aggressive than sPTC. This aggressiveness is characterized by multicentricity, larger tumour size, ETE, vascular invasion, and lymph nodal involvement [4,[8][9][10][11][12][13][14][15][16][17]. Since there is also contradictory data, it remains controversy [1,[18][19][20][21].…”

The impact of family history on non-medullary thyroid cancer

“…In the previously mentioned Chinese study [56], patients with FNMTC had a significantly higher rate of metastases than the sporadic cohort (64% versus 34%, p=0.005). Zhang et al [57], in a more recent Chinese study reported similar findings.…”

“…Although not all groups have found this to be statistically significant due to the already high rate of multifocality in sporadic NMTC [52], the majority of series report rates of multicentricity of approximately 50% in patients with a family history versus 30–40% in sporadic cases [50, 53–56, 59, 62, 63]. Indeed, those groups with a sufficient number of cases to analyze those with 3 or more affected family members report rates of up to 68% in true papillary FNMTC [53].…”

“…However, a recent Chinese study which applied a strict definition of FNMTC reported both larger tumors and higher rates of extra thyroidal extension in familial cases (88% versus 64%, p=0.02 and 82% versus 24%, p<0.001, respectively) [56]. Similar results were reported by a group from the United States, who used the less strict definition of at least one other family member affected, and reported extra thyroidal extension in 5.4% of familial versus 0.6% of sporadic cases (p=0.007), respectively [54].…”