The Long-Term Outcomes of the Second Generation of Familial Nonmedullary Thyroid Carcinoma Are More Aggressive than Sporadic Cases

Abstract

Background: Familial nonmedullary thyroid carcinoma (FNMTC) is frequently detected, but the prevalence or the aggressiveness of FNMTC is still unclear. We aimed to investigate the prevalence, clinical characteristics, and prognosis of FNMTC. Methods: This study included 3056 nonmedullary thyroid carcinoma (NMTC) patients who were pathologically confirmed to exhibit differentiated thyroid carcinoma from January 1962 through March 2010. The duration of follow-up was 6.2 -6.2 years. Results: The prevalence of FNMTC was 9.6%; 37.9% of the FNMTC patients exhibited a parent-offspring relationship, and 62.1% exhibited a sibling relationship. FNMTC was smaller in tumor size (1.2 -0.9 vs. 1.4 -1.1 cm) and more multifocal (33.6% vs. 27.0%) than sporadic cases. FNMTC presented higher recurrence rates (29.5% vs. 19.8%) and shorter recurrence-free survival than sporadic NMTC ( p = 0.046). When we compared sporadic NMTC with parent-offspring or sibling FNMTC separately, parent-offspring FNMTC was more multifocal (39.3% vs. 27.0%), while sibling FNMTC was more prevalent in female patients (89.6% vs. 82.5%) and presented smaller tumors (1.2 -0.8 vs. 1.4 -1.1 cm) than sporadic NMTC. The recurrence rate was higher than that of sporadic NMTC in parent-offspring FNMTC (35.6% vs. 19.8%) but not in sibling FNMTC. Among the 123 parent-offspring FNMTC cases, the second generation exhibited an earlier age at the diagnosis (38 -11 vs. 57 -11 years), more extrathyroidal invasion (57.8% vs. 29.4%), a higher recurrence rate (50.0% vs. 19.0%), and shorter recurrence-free survival ( p = 0.015) than the first generation. Conclusion: FNMTC was found to have a very high prevalence in our population. Parent-offspring FNMTC demonstrated higher recurrence than sporadic NMTC; specifically, the second generation of parent-offspring FNMTC cases exhibited more aggressive clinical characteristics than the first generation.

Long-Term Prognosis of Differentiated Thyroid Cancer with Lung Metastasis in Korea and Its Prognostic Factors

“…In Korea, the incidence of thyroid cancer is also rapidly increasing; the incidence rates per 100,000 persons adjusting for age increased from 2.1 (in 1999) to 15.4 (in 2009) in males and 10.4 (in 1999) to 79.6 (in 2009) in females (23)-rates that are higher than in any other country studied (24). Furthermore, Korean DTCs have several distinguishing characteristics: (i) family history of DTC is twofold higher than in other studies (25), (ii) PTC is more frequent (more than 90%) (26), and (iii) DTC patients had higher rates of BRAF V600E mutations (58-81%) (27)(28)(29) in Korea than in any other country (30). Environmentally, Korea is one of the iodinesufficient areas in the world (31).…”

ENDOCRINE TUMOURS: Familial nonmedullary thyroid carcinoma is a more aggressive disease: a systematic review and meta-analysis

“…Of these, 1165 studies were excluded after the first screening based on titles or abstracts. After further reviewing the full text of the remaining 53 articles, a total of 12 studies finally satisfied the eligibility criteria (11,12,13,14,15,16,18,19,20,21,33,34).…”

“…All were retrospective studies, including eight cohort studies (11,13,14,15,16,18,20,34) and four case-control studies (12,19,21,33), and all of them were hospital-based studies. The included studies were published between 2000 and 2014, and the number of patients ranged from 136 to 6015, for a total of 12 741 subjects across studies, including 1087 cases of FNMTC.…”

“…NMTC represents more than 95% of all thyroid cancer, and four histologic subtypes can be distinguished: papillary (85%), follicular (11%), Hürthle syndromes such as Cowden disease (multiple hamartoma syndrome), familial adenomatous polyposis, or Carney complex (8). The reported prevalence of FNMTC varies from 2.5 to 11.3% of all NMTC patients (5,9,10,11). Given the high prevalence of thyroid cancer in the general population, the occurrence of FNMTC may not be rare.…”

“…A number of researchers have found that FNMTC has more aggressive biological behaviors, characterized by a higher incidence of multifocality, extrathyroidal invasion, bilateral disease, and lymph node metastases, which lead to a higher recurrence rate and decreased disease-free survival (DFS) (11,12,13,14,15,16,17). Therefore, some of them recommended more aggressive initial treatment for patients with FNMTC, including total thyroidectomy (TT) and concomitant prophylactic central compartment node dissection (14,17).…”