يعرض 1 - 10 نتائج من 206 نتيجة بحث عن '"Second Primary"', وقت الاستعلام: 0.80s تنقيح النتائج
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    المصدر: JCRPE, Vol 13, Iss 2, Pp 225-231 (2021)
    Journal of Clinical Research in Pediatric Endocrinology

    الوصف: Most cases of malignancies appear to be sporadic, but some syndromes are associated with malignancies with germline variants. Herein, a child with an unusual association of oncocytic variant adrenocortical carcinoma (ACC) and rhabdomyosarcoma (RMS) was presented. An 18-month-old-boy was admitted with virilization of the genital area, penis enlargement and erection, which had begun six months earlier. Serum total testosterone (457 ng/dL; NR

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    المصدر: Cancer Res

    الوصف: Li-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 because of limited data and the difficulty of characterizing the effects of a primary cancer on the penetrance of a second primary cancer. Using a recurrent events survival modeling approach that incorporates a family-wise likelihood to efficiently integrate the pedigree structure, we estimated the penetrance for both first and second primary cancer diagnosis from a pediatric sarcoma cohort at MD Anderson Cancer Center [MDACC, Houston, TX; number of families = 189; single primary cancer (SPC) = 771; and MPC = 87]. Validation of the risk prediction performance was performed using an independent MDACC clinical cohort of TP53 tested individuals (SPC = 102 and MPC = 58). These findings showed that an individual diagnosed at a later age was more likely to be diagnosed with a second primary cancer. In addition, TP53 mutation carriers had a HR of 1.65 (95% confidence interval, 1.1–2.5) for developing a second primary cancer versus SPC. The area under the ROC (AUC) curve for predicting individual outcomes of MPC versus SPC was 0.77. In summary, we provide the first set of penetrance estimates for first and second primary cancer for TP53 germline mutation carriers and demonstrate its accuracy for cancer risk assessment. Significance: These findings present an open-source R package LFSPRO that could be used for genetic counseling and health management of individuals with LFS as it estimates the risk of both first and second primary cancer diagnosis. See related article by Shin et al., p. 354

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    المصدر: The Journal of Clinical Endocrinology & Metabolism. 106:e2580-e2588

    الوصف: Purpose This study investigated radioactive iodine treatment (RAIT) patterns and the secondary cancer incidence among children and young adults receiving RAIT after thyroidectomy for thyroid cancer. Methods This population-based cohort study used the Health Insurance Review and Assessment database of South Korea to identify a total of 18 617 children and young adults (0–29 years) who underwent thyroidectomy for thyroid cancer between 2008 and 2018. We recorded age at surgery, sex, the interval from surgery to RAIT, the doses of RAI, the number of RAIT sessions, and secondary cancer incidence. Results A total of 9548 (51.3%) children and young adults underwent 1 or more RAIT sessions. The initial dose of RAIT was 4.35 ± 2.19 GBq. The overall RAIT frequency fell from 60.9% to 38.5%, and the frequency of high-dose RAIT (>3.7 GBq) fell from 64.2% to 36.5% during the observational period. A total of 124 cases of secondary cancer developed during 120 474 person-years of follow-up; 43 (0.5%) in the surgery cohort and 81 (0.8%) in the RAIT cohort. Thus, the RAIT cohort was at an increased risk of secondary cancer (adjusted hazard ratio 1.52 [95% confidence interval 1.03–2.24], P = 0.035). Conclusion The proportion of children and young adults receiving RAIT, and the RAI dose, fell significantly over the observational period. RAIT was associated with secondary cancers. This is of major concern in the context of child and young adult thyroid cancer survivors.

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    المصدر: Journal of Gastroenterology. 56:434-441

    الوصف: Second primary cancers have impact on survival in patients who achieved cure for the first esophageal cancer. We, therefore, assessed the risk of incidence and mortality for second primary cancer by calculating standardized incidence ratio (SIR) and standardized mortality ratio (SMR) in patients with superficial or localized esophageal cancer without lymph node metastases as the first cancer (index cancer). Data on cancer development and subsequent causes of deaths were collected from integrated database of the Osaka Cancer Registry and the Vital Statistics of Japan. Records with information on patients with index esophageal cancer diagnosed between 2004 and 2013 were extracted from the database. Then, SIR and SMR for second primary cancers that developed in other organ were calculated with the reference to the general population during the same period. All probability values are two-tailed. Of 473,784 case records, 3022 cases of patients with index esophageal cancer were identified. Significantly higher SMRs/SIRs for cancers in mouth/pharynx, larynx, pancreas, and leukemia were confirmed with the values of 10.78/16.16, 8.56/6.44, 2.33/2.31, and 3.96/4.42, respectively. Significantly, higher SIRs for stomach, lung, and skin cancers were confirmed with the values of 2.84, 2.36, and 3.38, respectively, while SMRs were not significantly higher in these cancers. Significantly higher risks for mouth/pharynx, larynx, pancreas, and leukemia as second cancers were clarified. Careful surveillance for these cancers is required for esophageal cancer patients.

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    المصدر: Nature Communications, Vol 12, Iss 1, Pp 1-11 (2021)
    Nature Communications

    الوصف: Pediatric therapy-related myeloid neoplasms (tMN) occur in children after exposure to cytotoxic therapy and have a dismal prognosis. The somatic and germline genomic alterations that drive these myeloid neoplasms in children and how they arise have yet to be comprehensively described. We use whole exome, whole genome, and/or RNA sequencing to characterize the genomic profile of 84 pediatric tMN cases (tMDS: n = 28, tAML: n = 56). Our data show that Ras/MAPK pathway mutations, alterations in RUNX1 or TP53, and KMT2A rearrangements are frequent somatic drivers, and we identify cases with aberrant MECOM expression secondary to enhancer hijacking. Unlike adults with tMN, we find no evidence of pre-existing minor tMN clones (including those with TP53 mutations), but rather the majority of cases are unrelated clones arising as a consequence of cytotoxic therapy. These studies also uncover rare cases of lineage switch disease rather than true secondary neoplasms.
    Paediatric therapy-related myeloid neoplasms (tMN) have a dismal prognosis and have not been comprehensively profiled. Here the authors characterise the molecular landscape of 84 paediatric tMN patients, and find that, unlike adult tMNs, these do not emerge from pre-existing clones and that MECOM dysregulation is frequent.

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    المصدر: Pediatric and Developmental Pathology. 24:164-168

    الوصف: Rhabdoid tumor predisposition syndrome (RTPS) is defined as the presence of a SMARCB1 or SMARCA4 genetic aberration in a patient with malignant rhabdoid tumor. Patients with RTPS are more likely to present with synchronous or metachronous rhabdoid tumors. Based on the current state of rhabdoid tumor taxonomy, these diagnoses are based largely on patient demographics, anatomic location of disease, and immunohistochemistry, despite their nearly identical histologic and immunohistochemical profiles. Thus, the true distinction between such tumors remains a diagnostic challenge. Central nervous system atypical teratoid/rhabdoid tumor (AT/RT) is a rare, aggressive, primarily pediatric malignancy with variable histologic features and a well documented association with loss of SMARCB1 expression. Epithelioid sarcoma (ES) is a rare soft tissue tumor arising in patients of all ages and characteristically staining for both mesenchymal and epithelial immunohistochemical markers while usually demonstrating loss of SMARCB1 expression. To our knowledge we herein present the first documented case of a patient with RTPS who presented with metachronous AT/RT and ES.

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    المصدر: American Journal of Gastroenterology. 116:1063-1071

    الوصف: Introduction Among survivors from first primary cancers that occurred during childhood and adolescence, their risks of developing subsequent primary digestive system cancers are not well understood. Therefore, we conducted the largest and most comprehensive analysis examining risks for diverse types of digestive system cancers after survival from a wide variety of first primary childhood and adolescent cancers. Methods We identified 41,249 patients diagnosed with first primary cancer from 1975 to 2015 before 20 years of age from 9 Surveillance, Epidemiology and End Results Program registries. Standardized incidence ratios (SIRs) and absolute excess risks (AERs) for digestive system cancers were calculated controlling for age, sex, race, and calendar year. Results Among 41,249 cancer survivors, 133 developed subsequent primary digestive system cancer, with a median digestive system cancer diagnosis age of 37 years. The SIR and AER for any digestive system cancer were highest among survivors of bone cancer, lymphoma, and neuroblastoma. Among survivors from any first primary cancer, the SIR was significantly elevated for cancer of the esophagus, stomach, small intestine, large intestine, liver, and pancreas, whereas the AER was highest for large intestine cancer. Discussion Childhood and adolescent cancer survivors diagnosed from 1975 to 2015 have significantly elevated risks of digestive system cancers compared with the US general population. Our detailed findings may contribute to surveillance recommendations of childhood and adolescent cancer survivors and promote future studies to further understand mechanisms by which having various first primary cancers lead to subsequent primary digestive system cancers.

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    المصدر: European Journal of Endocrinology. 183:471-480

    الوصف: Context: Growth hormone (GH) deficiency is a common late effect of cranial irradiation. However, concerns have been raised that GH treatment might lead to an increased risk of a second neoplasm (SN). Objective: To study the impact of GH treatment on the risk of SN in a French cohort of survivors of childhood cancer (CCS) treated before 1986. Design and setting: Cohort study and nested case–control study. Participants: Of the 2852 survivors, with a median follow-up of 26 years, 196 had received GH therapy (median delay from cancer diagnosis: 5.5 years). Main outcome measures: Occurrence of SN Results: In total, 374 survivors developed a SN, including 40 who had received GH therapy. In a multivariate analysis, GH treatment did not increase the risk of secondary non-meningioma brain tumors (RR: 0.6, 95% CI: 0.2–1.5, P = 0.3), secondary non-brain cancer (RR: 0.7, 95% CI: 0.4–1.2, P = 0.2), or meningioma (RR: 1.9, 95% CI: 0.9–4, P = 0.09). Nevertheless, we observed a slight non-significant increase in the risk of meningioma with GH duration: 1.6-fold (95% CI: 1.2–3.0) after an exposure of less than 4 years vs 2.3-fold (95% CI: 0.9–5.6) after a longer exposure (P for trend = 0.07) confirmed by the results of a case–control study. Conclusion: This study confirms the overall safety of GH use in survivors of childhood cancer, which does not increase the risk of a SN. The slight excess in the risk of meningioma in patients with long-term GH treatment is non-significant and could be due to difficulties in adjustment on cranial radiation volume/dose and/or undiagnosed meningioma predisposing conditions.

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    المصدر: Cancer Medicine
    Cancer Medicine, Vol 9, Iss 21, Pp 7954-7963 (2020)

    الوصف: Background Li‐Fraumeni Syndrome (LFS) is a rare cancer‐predisposing condition caused by germline mutations in TP53. Conventional wisdom and prior work has implied an increased risk of secondary malignancy in LFS patients treated with radiation therapy (RT); however, this risk is not well‐characterized. Here we describe the risk of subsequent malignancy and cancer‐related death in LFS patients after undergoing RT for a first or second primary cancer. Methods We reviewed a multi‐institutional hereditary cancer registry of patients with germline TP53 mutations who were treated from 2004 to 2017. We assessed the rate of subsequent malignancy and death in the patients who received RT (RT group) as part of their cancer treatment compared to those who did not (non‐RT group). Results Forty patients with LFS were identified and 14 received RT with curative intent as part of their cancer treatment. The median time to follow‐up after RT was 4.5 years. Fifty percent (7/14) of patients in the curative‐intent group developed a subsequent malignancy (median time 3.5 years) compared to 46% of patients in the non‐RT group (median time 5.0 years). Four of seven subsequent malignancies occurred within a prior radiation field and all shared histology with the primary cancer suggesting recurrence rather than new malignancy. Conclusion We found that four of14 patients treated with RT developed in‐field malignancies. All had the same histology as the primary suggesting local recurrences rather than RT‐induced malignancies. We recommend that RT should be considered as part of the treatment algorithm when clinically indicated and after multidisciplinary discussion.
    Forty patients with LFS were identified and 14 received RT with curative intent as part of their cancer treatment. We found that four of 14 patients treated with RT developed in‐field malignancies. All had the same histology as the primary suggesting local recurrences rather than RT‐induced malignancies.