Biological behavior and prognosis of familial papillary thyroid carcinoma

doi:10.1016/j.surg.2008.08.004

Surgery

Volume 145, Issue 1, January 2009, Pages 100-105

https://doi.org/10.1016/j.surg.2008.08.004 Get rights and content

Background

Although the responsible genes have not yet been identified, it is known that the risk of nonmedullary thyroid carcinoma is elevated in individuals with 1st-degree relatives with nonmedullary thyroid carcinoma. However, it remains controversial whether the biological character of familial nonmedullary carcinoma (FNMTC) differs from that of sporadic carcinoma. In this study, we investigated the prevalence of familial papillary carcinoma and its biological behavior.

Methods

Between 1987 and 2004, 6,015 patients underwent initial surgical treatment for papillary carcinoma at Kuma Hospital and 273 (4.5%) were classified as having familial carcinoma. We compared the biological characteristics including prognosis between familial and sporadic papillary carcinomas.

Results

Disease-free survival and cause-specific survival rates of familial carcinoma did not differ from those of sporadic carcinoma. Familial papillary carcinoma showed multicentricity and recurrence to the thyroid more frequently than sporadic carcinoma. There were no differences in other clinicopathological parameters between the 2 groups.

Conclusion

Prognosis of patients with familial papillary carcinoma did not differ from that of those with sporadic papillary carcinoma. Although routine total thyroidectomy is recommended for familial papillary carcinoma, its therapeutic strategy can otherwise be the same as that for sporadic papillary carcinoma.

Section snippets

Patients

Between 1987 and 2004, 6,015 patients underwent initial surgical treatment for papillary carcinoma at Kuma Hospital. Of these 6,015 patients, 273 (4.5%) were classified as having familial papillary carcinoma, because they had one or more 1st-degree relatives who underwent surgical treatment for papillary or follicular carcinoma. The criteria for FNMTC in the present study were the same as those reported by Uchino et al.¹² Of these 273 patients, 255 (93.4%) belonged to families having 2 affected

Incidence of familial papillary carcinoma

We retrospectively examined the records of 6,015 patients with papillary carcinoma who underwent an initial surgical treatment in Kuma Hospital between 1987 and 2004. As indicated above, 273 patients (4.5%) were diagnosed as having familial papillary carcinoma.

Preoperative evaluation

Size, location, and multicentricity of primary carcinoma and lymph node metastasis were evaluated by ultrasonography for all patients. Of 6,015 patients in our series, 1,722 (29%) were preoperatively diagnosed as multicentric. Lymph node

Discussion

In this study, we demonstrated that DFS and CSS of patients with familial papillary carcinoma were the same as those of sporadic carcinoma patients. This is, however, discrepant with that of a previous study from Japan,¹² which reported that familial FNMTC showed a worse DFS. They analyzed patients who underwent surgical treatment between 1946 and 2000, and their follow-up periods averaged longer than 11 years. We enrolled patients who received their initial surgery after 1987, resulting in a

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El carcinoma familiar de tiroides no medular (FNMTC, familial non-medulary thyroid carcinoma) se define por la presencia de 2 o más miembros familiares de primer grado con carcinoma diferenciado de tiroides (CDT). El objetivo de este estudio es analizar las características clínicas, anatomopatológicas y pronósticas del FNMTC respecto al carcinoma esporádico (CE).
Estudio retrospectivo de los casos de CDT registrados en nuestro centro hospitalario durante el periodo 1990-2018.
Se analizó a un total de 927 pacientes, de los cuales 61 son FNMTC, con un seguimiento medio de 9,7 ± 6,5 años. La prevalencia del FNMTC es del 6,6%, presentando un estadio TNM inicial más bajo (p = 0,003) debido a la mayor proporción de tumores inferiores a 2 cm (p = 0,003), además de ser más multifocales (p = 0,034) y del subtipo histológico papilar (p = 0,022), respecto al CE. No se detectaron diferencias significativas en la edad al diagnóstico (p = 0,347), género (p = 0,406), ni en otros marcadores de agresividad (bilateralidad, extensión extratiroidea, afectación ganglionar y metástasis). La tasa de persistencia/recurrencia (p = 0,656), supervivencia libre de enfermedad (p = 0,929) y mortalidad ocasionada por el propio tumor (p = 0,666) fueron comparables. En las familias con ≥ 3 familiares afectados, los tumores son más pequeños (p = 0,005), más multifocales (p = 0,040) y bilaterales (p = 0,002), además de haber una mayor proporción de hombres (p = 0,020). Los pacientes de la segunda generación presentaron al diagnóstico el FNMTC a una edad más precoz respecto a los de la primera (p = 0,001).
El FNMTC presenta en nuestro estudio, en comparación con el CE, una estadificación TNM más baja, mayor multifocalidad y representación del subtipo papilar, con similares datos de agresividad y sin diferencias en el pronóstico.
Familial non-medullary thyroid carcinoma (FNMTC) is defined by the presence of 2 or more first-degree family members with differentiated thyroid carcinoma (DTC). The aim of this study is to compare clinicopathological features and prognosis of FNMTC and sporadic carcinoma (SC).
Retrospective study of DTC included in the hospital database during the period 1990-2018.
A total of 927 patients were analyzed, 61 of them were FNMTC, with a mean follow-up of 9.7 ± 6.5 years. The prevalence of FNMTC was 6.6%, with a lower TNM staging presentation (P = .003) consequence of a higher proportion of tumors smaller than 2 centimeters (P=.003), combined with a greater multifocality (P=.034) and papillary histologic subtype (P=.022) compared to SC. No significant differences in age at diagnosis (P=.347), gender (P=.406), neither in other aggressiveness markers (bilaterality, extrathyroidal extension, lymph node involvement and metástasis) were detected. Rate of persistence/recurrence (P=.656), disease-free survival (P=.929) and mortality caused by the tumor itself (P=.666) were comparable. Families with ≥3 affected relatives, had smaller tumors (P=.005), more multifocality (P=.040) and bilaterality (P=.002), as well as a higher proportion of males (P=.020). Second generation patients present earlier FNMTC compared to those of the first generation (P=.001).
In our study FNMTC presents a lower TNM staging, higher multifocality and papillary variant, with similar aggressiveness and prognosis compared to SC.
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Approximately 3% to 9% of all nonmedullary thyroid cancers (NMTCs) are inherited. Familial nonmedullary thyroid cancer (FNMTC) may be part of familial cancer syndromes (syndromic) or the predominant or only feature (nonsyndromic). The majority of FNMTCs are nonsyndromic (95%). Syndromic FNMTCs include familial adenomatous polyposis (FAP); PTEN hamartoma tumor syndrome; Carney complex type 1; Werner, Pendred, and DICER1 syndromes; and ataxia-telangiectasia. Although the risk of thyroid cancer is higher in syndromic FNMTC than in the general population, it is commonly a minor component with incomplete penetrance. On the other hand, nonsyndromic FNMTC has an autosomal-dominant pattern of inheritance and is associated with more aggressive disease than sporadic NMTC. In this chapter, we discuss screening for FNMTC, the clinical and pathologic features of syndromic and nonsyndromic FNMTC, and the treatment of patients with FNMTC.
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Citation Excerpt :
In particular, most studies found familial non-medullary thyroid cancer to be more frequently multifocal,94,95,97,98,100 and some studies found more frequent extrathyroidal extension95,104 and lymph node metastases.85,95,104 More frequent recurrence was also reported by some authors,85,94,95,97,98 but not by others.100–103 The more aggressive therapeutic approach advocated by some authors appears not to be justified, given the lack of consistency in the data.
The main purpose of cancer screening programmes should not be to detect all cancers, but to discover potentially fatal or clinically relevant cancers. The US Preventive Services Task Force recommends against screening for thyroid cancer in the general, asymptomatic adult population, as such screening would result in harms that outweigh any potential benefits. This recommendation does not apply to patients with symptoms or to individuals at increased risk of thyroid cancer because of a history of exposure to ionising radiation (in childhood, as radioactive fallout, or in medical treatment as low-dose radiotherapy for benign conditions or high-dose radiation for malignancy), inherited genetic syndromes associated with thyroid cancer (eg, familial adenomatous polyposis), or one or more first-degree relatives with a history of thyroid cancer. We discuss the evidence for and against screening individuals who are at high risk, and consider the different screening tools available.
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Approximately 3%–9% of all nonmedullary thyroid cancers are inherited. Familial nonmedullary thyroid cancer (FNMTC) is classified syndromic (part of a familial cancer syndrome) or as nonsyndromic (the predominant or only feature being nonmedullary thyroid cancer). Nonsyndromic FNMTC account for 95% of FNMTC cases is more aggressive than sporadic nonmedullary thyroid cancer and has a younger age at presentation when three or more first-degree relatives are affected. Although the risk of nonmedullary thyroid cancer is higher than in the general population in syndromic FNMTC, it is commonly a minor component or an infrequent manifestation of the syndrome. Syndromic FNMTC includes familial adenomatous polyposis, PTEN hamartoma tumor syndrome, Carney's complex type 1, Werner, Pendred and DICER1 syndromes, and ataxia-telangiectasia. In this chapter, we review the genetics of syndromic and nonsyndromic FNMTC and the clinicopathologic features of syndromic and nonsyndromic FNMTC.

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Original CommunicationBiological behavior and prognosis of familial papillary thyroid carcinoma

Background

Methods

Results

Conclusion

Section snippets

Patients

Incidence of familial papillary carcinoma

Preoperative evaluation

Discussion

Risk factors contributing to a poor prognosis of papillary thyroid carcinoma; validity of UICC/AJCC TNM classification and stage grouping

World J Surg

Prognostic significance of extrathyroid extension of papillary thyroid carcinoma: massive but not minimal extension affects the relapse-free survival

World J Surg

Ultrasound-detectable and anatomopathologically-detectable node metastasis in the lateral compartment as indicators of worse relapse-free survival in patients with papillary thyroid carcinoma

World J Surg

Familial nonmedullary thyroid carcinoma-the case for genetic susceptibility

J Clin Endocrinol Metab

Familial nonmedullary thyroid cancer

Thyroid

Carcinoma of the thyroid and other diseases of the thyroid in identical twins

Arch Surg

Familial occurrence of differentiated (non-medullary) thyroid cancer

Oncology

Increased risk for nonmedullary thyroid cancer in the first-degree relatives of prevalent cases of nonmedullary thyroid cancer: a hospital-based study

J Clin Endocrinol Metab

Systematic population-based assessment of cancer risk in the first-degree relatives of cancer probands

J Natl Cancer Inst

Original Communication
Biological behavior and prognosis of familial papillary thyroid carcinoma