Familial Papillary Thyroid Carcinoma: A Retrospective Analysis

Abstract

Background. Whether or not the familial form of papillary thyroid carcinoma is more aggressive than the sporadic form of the disease remains controversial. Methods. To explore this question and whether or not increased aggressiveness is more apparent in families with multiple affected members, we performed a chi square by trend analysis on our patients clinical and pathologic data comparing: first degree families with three or more affected members versus first degree families with two affected members versus sporadic cases of papillary thyroid carcinoma. Results. No statistically significant trends were seen for any presenting surgical pathology parameter, age at presentation, length of follow-up or gender distribution. The familial groups exhibited significant trends for higher rates of reoperation (P = 0.05) and/or requiring additional radioactive iodine therapy (P = 0.03), distant metastases (P = 0.003) and deaths (P = 0.01). These aggressive features were most apparent in certain families with three or more affected members. Conclusions. Using the chi square by trend analysis, a significant trend was seen for the familial form of papillary thyroid cancer to possess more aggressive features than the sporadic disease. Prompt recognition of the familial nature of the disease may provide earlier diagnosis and treatment in similarly affected family members.

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.…”

“…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. Of the 12 included studies, five studies were conducted in Asia (11,14,15,18,21), four in North America (13,16,19,20), two in Europe (33,34), and the remaining one included patients from Japan and USA (12). FNMTC was defined as patients with two or more first-degree relatives with NMTC in eight studies, while it was defined as occurring when at least one first-degree relative was affected with NMTC in the other four studies (13,14,15,18).…”

“…Despite the variations in the diagnosis criteria of recurrence observed in the included studies, most of the authors defined recurrence based on a variable combination of results including clinical, radiographic, and nuclear medicine imaging studies; serum Tg level; and hisopathologic findings (11,12,13,15,18,19,21,34). However, there were three articles which did not provide the information about definition of recurrence (14,16,33). The Newcastle-Ottawa scale scores for included studies Flow chart of article selection in this meta-analysis.…”

“…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).…”