The distinctive biology of cancer in adolescents and young adults

Bleyer, Archie; Barr, Ronald D.; Hayes‐Lattin, Brandon; Thomas, David M.; Ellis, Chad A.; Anderson, Barry

doi:10.1038/nrc2349

Cited by 573 publications

(489 citation statements)

References 48 publications

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“…17 Moreover, our patient had both a glioblastoma and colon cancer. Indeed, a germline c.584T>C (p.Ile195Thr) mutation of the p53 gene was detected in our patient and this Thr195-type p53 displayed loss of tumor suppressor activity in the regulation of transactivation and growth suppression.…”

Section: Resultsmentioning

confidence: 99%

“…Our patient belonged to the age group termed ''adolescent and young adult'' (AYA), and malignant tumors in AYA have recently been reviewed by Bleyer et al 17 Some proportion of the malignant tumors in AYA are attributable to germline mutations of certain genes, such as tumor suppressor genes and oncogenes. 17 Moreover, our patient had both a glioblastoma and colon cancer.…”

Section: Resultsmentioning

confidence: 99%

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Identification and characterization of a novel germline p53 mutation in a patient with glioblastoma and colon cancer

Yamada

Shinmura

Yamamura

et al. 2009

Intl Journal of Cancer

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Germline mutations in the p53 tumor suppressor gene have been identified in patients with Li-Fraumeni syndrome (LFS) and patients with Li-Fraumeni-like syndrome (LFL). However, to date, germline p53 mutations in patients not fulfilling the criteria of LFS or LFL have been reported only very rarely. In our study, a novel germline c.584T>C (p.Ile195Thr) mutation of the p53 gene was found in a 21-year-old male with a glioblastoma and colon cancer. He had no family history of cancer within second-degree relatives, and loss of the wild-type p53 allele and overexpression of p53 protein were observed in both tumors. Functional analyses revealed transactivation and growth suppressive function activities of the Thr195-type p53 to be impaired. These results suggest germline p53 mutations to possibly be responsible for a subset of young adult patient with multiple malignant tumors, even those not meeting the clinical criteria for LFS or LFL. ' 2009 UICC

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Identification and characterization of a novel germline p53 mutation in a patient with glioblastoma and colon cancer

Yamada

Shinmura

Yamamura

et al. 2009

Intl Journal of Cancer

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“…As Hodgkin lymphoma is one of the few cancers with a peak in incidence at ages 15–39 years, this cancer is of special interest in adolescent and young adult oncology 2. Hodgkin lymphoma diagnosed during adolescence or young adulthood is usually of the nodular sclerosis subtype, in contrast to cases diagnosed at older ages,3 and the biology of cancer in adolescents and young adults in general has been suggested to be different from that of cancers in children and older people 4, 5. Studies of patients in this specific age range are, therefore, important 6, 7…”

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confidence: 99%

Long-term hospitalisation rates among 5-year survivors of Hodgkin lymphoma in adolescence or young adulthood: A nationwide cohort study

et al. 2017

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In the present study, we report on the full range of physical diseases acquired by survivors of Hodgkin lymphoma diagnosed in adolescence or young adulthood. In a Danish nationwide population‐based cohort study, 1,768 five‐year survivors of Hodgkin lymphoma diagnosed at ages 15–39 years during 1943–2004 and 228,447 comparison subjects matched to survivors on age and year of birth were included. Hospital discharge diagnoses and bed‐days during 1977–2010 were obtained from the Danish Patient Register for 145 specific disease categories gathered in 14 main diagnostic groups. The analysis was conducted separately on three subcohorts of survivors, that is, survivors diagnosed 1943–1976 for whom we had no information on rehospitalisation for Hodgkin lymphoma and survivors diagnosed 1977–2004, split into a subcohort with no expected relapses and a subcohort for whom a rehospitalisation for Hodgkin lymphoma indicated a relapse. The overall standardised hospitalisation rate ratios (RRs) were 2.0 [95% confidence interval (CI), 1.9–2.1], 1.5 (1.4–1.6) and 2.9 (2.6–3.1) respectively, and the corresponding RRs for bed‐days were 3.5 (3.4–3.5), 1.8 (1.8–1.9) and 10.4 (10.3–10.6). Highest RRs were seen for nonmalignant haematological conditions (RR: 2.6; 3.1 and 9.7), malignant neoplasms (RR: 3.2; 2.5 and 4.7) and all infections combined (RR: 2.5; 2.2 and 5.3). Survivors of Hodgkin lymphoma in adolescence or young adulthood are at increased risk for a wide range of diseases that require hospitalisation. The risk depends on calendar period of treatment and on whether the survivors were rehospitalised for Hodgkin lymphoma, and thus likely had a relapse.

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“…Ewing sarcoma, the second most frequent bone cancer, is a rare aggressive tumor which occurs primarily in children, adolescents and young adults and is a member of the family of primitive neuroectodermal tumors 1–3. There is a tendency for Ewing sarcoma to be a more deadly disease in young adults than in younger patients.…”

mentioning

confidence: 99%

“…There is a tendency for Ewing sarcoma to be a more deadly disease in young adults than in younger patients. Different chromosomal abnormalities are found in patients more than 15 years of age than in younger patients and correlate with disease outcome 14. Ewing sarcoma is one of the small round blue cell tumors of the bone, characterized by strong membrane staining for CD99, and occurs primarily in Causasians 5.…”

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confidence: 99%

Characteristics of human Ewing/PNET sarcoma models

Teicher¹,

Rouleau²,

Kruger³

et al. 2011

Annals of Saudi Medicine

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Ewing/PNET (peripheral neuroepithelioma) tumors are rare aggressive bone sarcomas occurring in young people. Rare-disease clinical trials can require global collaborations and many years. In vivo models that as accurately as possible reflect the clinical disease are helpful in selecting therapeutics with the most promise of positive clinical impact. Human Ewing/PNET sarcoma cell lines developed over the past 45 years are described. Several of these have undergone genetic analysis and have been confirmed to be those of Ewing/PNET sarcoma. The A673 Ewing sarcoma line has proven to be particularly useful in understanding the biology of this disease in the mouse. The chromosomal translocation producing the EWS/FLI1 fusion transcript characterizes clinical Ewing sarcoma. Cell lines that express this genetic profile are confirmed to be those of Ewing sarcoma. The A673 Ewing sarcoma line grows in culture and as a xenograft in immunodeficient mice. The A673 model has been used to study Ewing sarcoma angiogenesis and response to antiangiogenic agents. Many Ewing sarcoma clinical specimens express the cell surface protein endosialin. Several Ewing sarcoma cell lines, including the A673 line, also express cell surface endosialin when grown as subcutaneous tumor nodules and as disseminated disease; thus the A673 is a useful model for the study of endosialin biology and endosialin-directed therapies. With the advent of tools that allow characterization of clinical disease to facilitate optimal treatment, it becomes imperative, especially for rare tumors, to develop preclinical models reflecting disease subsets. Ewing PNET sarcomas are a rare disease where models are available.

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The distinctive biology of cancer in adolescents and young adults

Cited by 573 publications

References 48 publications

Identification and characterization of a novel germline p53 mutation in a patient with glioblastoma and colon cancer

Identification and characterization of a novel germline p53 mutation in a patient with glioblastoma and colon cancer

Long-term hospitalisation rates among 5-year survivors of Hodgkin lymphoma in adolescence or young adulthood: A nationwide cohort study

Characteristics of human Ewing/PNET sarcoma models

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