Multiple primary cancer in Leser — Trelat syndrome (description of a clinical case)

2020 ◽  
Vol 0 (5) ◽  
pp. 65-70
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V. G. Bondar ◽  
O. V. Kayryak ◽  
V. V. Vasiliev ◽  
N. B. Gubergrits ◽  
T. L. Mozhyna
Therapy ◽  
2021 ◽  
Vol 3_2021 ◽  
pp. 100-105
Author(s):  
Semenkin A.A. Semenkin ◽  
Vorotilina L.V. Vorotilina ◽  
Sapronenko V.S. Sapronenko ◽  
Zhivilova L.A. Zhivilova ◽  
Drokina O.V. Drokina ◽  
...  

2007 ◽  
Vol 10 (4) ◽  
pp. 263 ◽  
Author(s):  
Sang Hee Jung ◽  
Seung Soo Kwak ◽  
Seong Chul Kim ◽  
Moon Ki Park ◽  
Gun Seok Lee ◽  
...  

1985 ◽  
Vol 39 (3) ◽  
pp. 385-391
Author(s):  
Hideo Kurokawa ◽  
Minoru Kajiyama ◽  
Nobuto Nomura ◽  
Takahiro Kodama ◽  
Isao Akama ◽  
...  

1999 ◽  
Vol 47 (3) ◽  
pp. 331
Author(s):  
Chang Jin Shin ◽  
Hye Jung Park ◽  
Kyeong Cheol Shin ◽  
Young Ran Shim ◽  
Jin Hong Chung ◽  
...  

2014 ◽  
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Jon Hoffman ◽  
Cyril Chapman ◽  
Kai Ren Ong ◽  
Fiona Lalloo ◽  
...  

1985 ◽  
Vol 16 (1) ◽  
pp. 13-19 ◽  
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Henry T. Lynch ◽  
David Allan Katz ◽  
Patrick Bogard ◽  
Jane F. Lynch

2019 ◽  
Author(s):  
Seung Jun Shin ◽  
Elissa B. Dodd ◽  
Fan Gao ◽  
Jasmina Bojadzieva ◽  
Jingxiao Chen ◽  
...  

AbstractLi-Fraumeni syndrome (LFS) is a rare autosomal dominant disorder associated with TP53 germline mutations and an increased lifetime risk of multiple primary cancers (MPC). Penetrance estimation of time to first and second primary cancer within LFS remains challenging due to limited data availability and the difficulty of accounting for the characteristic effects of a primary cancer on the penetrance of a second primary cancer. Using a recurrent events survival modeling approach, we estimated penetrance for both first and second primary cancer diagnosis from a pediatric sarcoma cohort (number families=189; Single primary cancer, or SPC=771; MPC=87). We then validated the risk prediction performance using an independent clinical cohort of TP53 tested individuals from MD Anderson (SPC=102; MPC=58). Our penetrance estimates are dependent on TP53 genotype, sex, and, importantly, age of diagnosis (AoD) for the first PC. We observed the later the AoD is, the shorter the gap time is for this person to be diagnosed with a second PC. We achieved an Area under the ROC curve (AUC) of 0.77 for predicting individual outcomes of MPC vs. SPC. In summary, we have provided the first set of penetrance estimates for SPC and MPC for TP53 mutation carriers, and demonstrated its accuracy for cancer risk assessment. Our open-source R package, LFSPRO, provides future risk estimates for SPC and MPC among TP53 germline mutation carriers.SignificanceOur tool can be used to support clinical counseling of LFS cancer survivors for better health management.


Author(s):  
Koji EMOTO ◽  
Yukishige YAMADA ◽  
Akihiko WATANABE ◽  
Tomoyoshi TAKAYAMA ◽  
Yoshiyuki NAKAJIMA

2021 ◽  
Author(s):  
Yitong Li ◽  
Dengjie Ouyang ◽  
Qitong Chen ◽  
Tao Hong ◽  
Wenjun Yi

Abstract Background: Radiotherapy combined with breast-conserving surgery is widely performed in patients with ductal carcinoma in situ (DCIS). This research was conducted to evaluate the use of radiotherapy to reduce the risk of multiple primary cancer (MPC) and mortality in DCIS patients.Methods: 108,416 patients first diagnosed with DCIS between 1998 and 2015 who received lumpectomy in the Surveillance, Epidemiology, and End Results (SEER) 18 database were included. Age, race, year of diagnosis, laterality, pathologic grade, surgery, radiation, estrogen receptor status, progesterone receptor status, tumor size and vital status were extracted. A comparison of lumpectomy vs. lumpectomy plus radiotherapy was performed using 1:1 propensity score-based matching.Results: Of the 108,416 patients, 39,039 patients were treated with lumpectomy alone, and 69,377 were treated with lumpectomy and radiotherapy. The adjusted hazard ratio (HR) for death was 0.801 and 0.7444 for occurrence of MPC in the lumpectomy and radiotherapy vs. lumpectomy alone groups, respectively. Conclusion: Lumpectomy plus radiotherapy is associated with a significant reduction in mortality and risk of MPC, mainly second primary BC. Younger patients, Black women with high-grade tumors were likely to benefit most. Radiotherapy reduced the risk of occurrence rather than the mortality of MPC patients to reduce the overall mortality.


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