Does Familial Non‐Medullary Thyroid Cancer Adversely Affect Survival?

Triponez, Frédéric; Wong, Mariwil G.; Sturgeon, Cord; Caron, Nadine R.; Ginzinger, David G.; Segal, Mark R.; Kebebew, Electron; Duh, Quan Yang; Clark, Orlo H.

doi:10.1007/s00268-005-0398-x

Cited by 76 publications

(87 citation statements)

References 30 publications

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“…The low prevalence of FNMTC, together with small sampling series and short follow-up studies, has limited consistent information on patient survival as well. Some studies have reported distinct clinical behaviours (3,16,17,18,19,20,21), but others have claimed that there are no differences between the two entities (1,22,23,24,25). Our present data seem to support the latter view, as we did not observe, overall, statistical differences in clinicopathological features, DNA ploidy status and outcome measures between familial PTC (whole series) and sporadic PTC.…”

Section: Discussioncontrasting

confidence: 37%

“…In a retrospective matched-case control study of FNMTC, the majority being PTCs, Maxwell et al (24) reported that familial cases tend to have, but not significantly, improved DFS and disease-specific survival. In a classical study (34), based on probability calculation analysis, Charkes pointed out that up to 60% of familial PTCs diagnosed in families with only two affected members might, actually, represent a fortuitous aggregation of sporadic cases, suggesting that the disease may be more accurately diagnosed in families with three or more affected members (17,21). This fact could, therefore, also contribute to misestimate the aggressiveness of familial PTC.…”

Section: Discussionmentioning

confidence: 99%

“…Although some studies have reported a less favourable prognosis for FNMTC (3,16,17,18,19,20,21), others did not verify this (1,22,23,24,25), and therefore, the biological and clinical behaviour of this familial disease, as compared with the much more frequent sporadic forms, remains a matter of controversy. Furthermore, data on molecular biomarkers, e.g.…”

Section: Introductionmentioning

confidence: 97%

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

Pinto

Silva²,

Henrique

et al. 2014

European Journal of Endocrinology

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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.

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Section: Discussioncontrasting

confidence: 37%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 97%

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

Pinto

Silva²,

Henrique

et al. 2014

European Journal of Endocrinology

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“…Observou que após tratamento, o carcinoma não afetou significativamente a sobrevida de pacientes quando comparados com membros de sua família não acometidos ou com a população geral dos Estados Unidos. Contudo, aqueles que faziam parte de famílias com três ou mais membros afetados apresentavam uma sobrevida mais curta quando comparados com famílias com somente dois membros acometidos (14).…”

Section: Característicasunclassified

Carcinoma não medular familiar da tiróide

Santos

Melo

Assumpção

2007

Arq Bras Endocrinol Metab

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RESUMOO carcinoma diferenciado da tiróide, papilífero ou folicular, origina-se da célula folicular tiroideana, sendo a neoplasia maligna mais freqüente desta glândula. Desde 1955 têm sido relatados casos de agrupamento familiar deste carcinoma, e atualmente estima-se que 4,2% de todos os carcinomas diferenciados da tiróide tenham origem familiar. Esses casos costumam ser mais agressivos, incidem em idade mais precoce, são multifocais e apresentam maior taxa de recorrência. Parecem ser transmitidos por herança autossômica dominante com penetrância variável, mas os genes exatos responsáveis pela doença ainda não foram totalmente identificados. Os pacientes devem ser tratados com tiroidectomia total e freqüentemente também com esvaziamento linfonodal cervical, seguidos de ablação com iodo radioativo e terapia supressiva do TSH com levotiroxina. Alguns autores recomendam rastreamento de familiares de primeiro grau dos pacientes afetados através da ultrassonografia cervical, com objetivo de realizar diagnóstico precoce, possibilitando melhores resultados terapêuticos. Nonmedullary thyroid carcinoma, originating from thyroid epithelial cells, is the most frequent thyroid malignant neoplasia. Since 1955, there has been increasing evidence that this cancer may have a familial predisposition. It is now established that around 4.2% of all nonmedullary thyroid carcinomas occurs on the background of familial predisposition. These cases are often more aggressive, due to early onset, multifocality and a higher percentual of recurrences. An autossomal dominant inheritance pattern appears likely in most families, although the exact genes responsible for this syndrome have not yet been identified. Patients affected by this cancer should be treated with total thyroidectomy routinely and, in most cases, lymph node dissection, followed by iodine ablation and TSH suppressive therapy with levothyroxine. Some authors also recommend that first-degree relatives of patients with nonmedullary thyroid cancer (especially women) should be submitted to neck ultrasound for thyroid cancer screening, aiming early diagnosis for better treatment results. A GRANDE MAIORIA DAS NEOPLASIAS malignas tiroideanas tem origem na célula folicular, sendo chamada de carcinomas diferenciados da tiróide (CDT). Histologicamente, podem ser papilíferos ou foliculares. Caracterizam-se por serem esporádicos, entretanto diversos trabalhos têm demonstrado um risco aumentado de 3,21 a 10,3 vezes de CDT em familiares de pacientes afetados por essa neoplasia (1-3). Em 1955, Robinson e Orr relataram casos de câncer da tiróide não medular em gêmeos monozigóti-

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“…Unfortunately, prophylactic thyroidectomy, directed by genetic testing, currently can not be recommended to remove the thyroid gland in patients who are genetically predisposed to developing FPTC. (48) reported that patients with three or more members with FPTC were more likely to die from their thyroid cancer than those with just two documented patients with thyroid cancer or patients without thyroid cancer. Index cases had a higher death rate than subsequent family members (48).…”

Section: Familial Non-medullary Thyroid Cancermentioning

confidence: 99%

Controversies in familial thyroid cancer 2014

Clark¹

2014

UCD

Self Cite

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Controversies in familial thyroid cancer 2014Thyroid cancer is the sixth most common cancer in women, and the majority of patients with thyroid cancer has sporadic disease. However, about 25% of patients with medullary thyroid cancer and 5% with papillary thyroid cancer have familial tumors. Currently, there are numerous controversies regarding the mode of inheritance, tumor behavior, extent of surgical resection for optimal results, coexisting thyroid pathology, risk of other cancers, and extent of postoperative treatment of patients with familial thyroid cancer. This review aimed to give insight to surgeons on this interesting topic.

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Does Familial Non‐Medullary Thyroid Cancer Adversely Affect Survival?

Abstract: Our results suggest that FNMTC may be more aggressive than sporadic thyroid cancer, particularly in families with 3 or more affected members. However, when recognized and treated appropriately, it does not significantly shorten the overall life expectancy of the affected patients.

Cited by 76 publications

References 30 publications

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

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

Carcinoma não medular familiar da tiróide

Controversies in familial thyroid cancer 2014

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