Familial vs sporadic papillary thyroid carcinoma: a matched-case comparative study showing similar clinical/prognostic behaviour

Abstract

Objective: Familial non-medullary thyroid cancer has been proposed as an aggressive clinical entity. Our aim in this study is to investigate potential distinguishing features as well as the biological and clinical aggressiveness of familial vs sporadic papillary thyroid carcinoma (PTC). We assessed clinicopathological characteristics, outcome measures and DNA ploidy. Design: A matched-case comparative study. Methods: A series of patients with familial PTC (nZ107) and two subgroups, one with three or more affected elements (nZ32) and another including index cases only (nZ61), were compared with patients with sporadic PTC (nZ107), matched by age, gender, pTNM disease extension and approximate follow-up duration. Histological variant, extrathyroidal extension, vascular invasion, tumour multifocality and bilateral growth were evaluated. Ploidy pattern was analysed in available samples by DNA flow cytometry. The probabilities of disease-free survival (DFS) and overall survival (OS) were estimated according to the Kaplan-Meier (K-M) method. Results: No patient with familial PTC died of disease during follow-up (median, 72 months), contrarily to five patients (4.7%) (PZ0.06) with sporadic PTC (median, 90 months). There was a significantly higher tumour multifocality in familial PTC (index cases subgroup) vs sporadic PTC (PZ0.035), and a trend, in the familial PTC cohort with three or more affected elements, to show extrathyroidal extension (PZ0.054) more frequently. No difference was observed in DNA ploidy status. The K-M analyses showed no significant differences between both entities in relation to DFS or OS. Conclusion: Apart from multifocality, familial PTC appears to have similar clinical/prognostic behaviour when compared with sporadic forms of the disease.

A Clinical Framework to Facilitate Risk Stratification When Considering an Active Surveillance Alternative to Immediate Biopsy and Surgery in Papillary Microcarcinoma

“…Compared with ideal patients, patients classified as being appropriate candidates have a higher risk of disease progression (e.g., middle-aged patients) (15), child-bearing potential with or without immediate plans for pregnancy (14), or have specific characteristics that will make it more difficult to follow with observation (e.g., less than high-quality neck ultrasonography, potential of tumor multifocally in patients with a strong family history of thyroid cancer) (21), subcapsular location not adjacent to critical structures (e.g., trachea or recurrent laryngeal nerve), a potentially more aggressive molecular phenotype, or ill-defined nodule margins (22). While a disease progression rate of approximately 10% is expected in this cohort, treatment offered at the time of disease progression will still be very effective and associated with excellent clinical outcomes when these patients are followed carefully by an experienced management team.…”

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.…”