Hereditary Cancer: Ascertainment and Management

Lynch, Henry T.; Lynch, Patrick M.; Albano, William A.; Edney, John; Organ, Claude H.; Lynch, F.

doi:10.3322/canjclin.29.4.216

Cited by 30 publications

(18 citation statements)

References 51 publications

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“…However, some other studies have not found FNMTC to be more aggressive than sporadic disease (10)(11)(12)14,17). In addition, some studies have reported that patients with FNMTC presented earlier cancer onset (16) or smaller primary tumors (30,31). In our study, FNMTC cases exhibited greater multifocality, a higher recurrence rate, and shorter recurrence-free survival than sporadic NMTC cases.…”

Section: Outcome Of Familial Nonmedullary Thyroid Carcinomacontrasting

confidence: 44%

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

Park

Ahn

Choi

et al. 2012

Thyroid

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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.

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Section: Outcome Of Familial Nonmedullary Thyroid Carcinomacontrasting

confidence: 44%

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

Park

Ahn

Choi

et al. 2012

Thyroid

View full text Add to dashboard Cite

show abstract

“…The behavior of the tumors in the siblings under study was generally characteristic of hereditary cancers as described by Lynch et al 3 Early onset and excessive bilateral involvement were apparent. Each patient was 20 years old or younger when the disease was discovered; each suffered recurrence of cancer within a few years in the remainder of the thyroid gland.…”

Section: Report Of Casesmentioning

confidence: 58%

Familial Papillary Carcinoma of the Thyroi

Phade¹,

Lawrence²,

Max³

1981

Arch Surg

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“…It has long been suspected that the breast and ovarian cancer in some families has a hereditary basis (see Lynch et al (1979) for a description of various hereditary cancer syndromes and their management recommendations over 25 years ago). The breast cancer in these families commonly occurs before menopause, often in close relatives and in several generations of the family (Thull and Vogel, 2004).…”

Section: Hereditary Breast/ovarian Cancer (Hboc) and Brca Gene Mutationsmentioning

confidence: 99%

The Effect of BRCA Gene Testing on Family Relationships: A Thematic Analysis of Qualitative Interviews

Douglas¹,

Hamilton

Grubs³

2009

Journal of Genetic Counseling

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Individuals with a personal or family history of cancer can pursue testing for mutations in BRCA1 and BRCA2, breast and ovarian cancer susceptibility genes, in order to help them make decisions about cancer risk-reducing surgeries and other management options. However, this genetic testing can also have emotional consequences, not only for the tested individual but also for his or her relatives since testing can provide risk information for them as well. Thus, this study investigated the impact of BRCA testing on family dynamics and family relationships. A qualitative research design was employed, in which a secondary analysis was conducted on interview transcripts. In the initial study, two open-ended, tape-recorded interviews were performed using grounded theory methodology with each of 12 participants approximately three years apart. All participants had tested positive for a mutation in either BRCA1 or BRCA2.Thematic analysis of interview transcripts was conducted in the current secondary analysis to characterize family relationships after BRCA testing. Three main themes were identified: 1.That the first in the family to have testing or seek genetic counseling takes on a special family role that can be difficult for them; 2. That discussions in the family, especially those associated with BRCA testing, often change after genetic testing; and 3. That individuals may feel more or less connected to certain family members after genetic testing has occurred in the family. These changes in family dynamics seem to depend on the family history of cancer, prior relationships within the family, emotional coping strategies of relatives, value placed on particular iv communication patterns, and sharing or not sharing the family's BRCA mutation. The results of this study highlight the profound changes in family life that can occur after BRCA testing.Health professionals can use the insight they gain from this study in their management of patients considering BRCA testing. This work also has public health relevance since it describes how genetic testing for susceptibility to a common disease can influence family dynamics. Such an understanding will be important as the genetic basis of common disease becomes better understood and tests for additional susceptibility genes become available. PREFACEFirst and foremost, I would like to acknowledge all of the participants in this study for sharing their experiences so openly. These individuals were truly an inspiration to me and have greatly The preliminary interviews were supported by a research grant to Dr. Rebekah J.Hamilton from Sigma Theta Tau International. INTRODUCTIONIn the 1990s, the discovery of the BRCA genes offered an explanation for the clustering of ovarian cancer and early onset breast cancer in some families. In these families, it was found that individuals who inherited a mutated copy of a BRCA gene had a lifetime risk of cancer much higher than that of the general population, while those who did not inherit a gene mutation had no elevated risk (Ford et al., ...

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Hereditary Cancer: Ascertainment and Management

Cited by 30 publications

References 51 publications

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

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

Familial Papillary Carcinoma of the Thyroi

The Effect of BRCA Gene Testing on Family Relationships: A Thematic Analysis of Qualitative Interviews

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