Cancer in the context of disorders of growth and development. Pediatrics meets oncology meets pediatrics

被引:0
|
作者
Schneider, Dominik T. [1 ,2 ]
Blessing, Tabea [1 ]
Graf, Norbert [3 ]
Abele, Michael [4 ]
Brecht, Ines B. [4 ]
Rosenbaum, Thorsten [5 ]
机构
[1] Klinikum Univ Witten Herdecke, Klinikum Dortmund, Klin Kinder & Jugendmed, Dortmund, Germany
[2] Klinikum Univ Witten Herdecke, Klinikum Dortmund gGmbH, Klin Kinder & Jugendmed, Beurhausstr 40, D-44137 Dortmund, Germany
[3] Univ Saarland, Klin Padiatr Onkol & Hamatol, Homburg, Saar, Germany
[4] Univ Klinikum Ribingen, Klin Kinder & Jugendmed, Abt Allgemeine Padiatrie Hamatol & Onkol, Tubingen, Germany
[5] Sana Kliniken Duisburg, Klin Kinder & Jugendmed, Duisburg, Germany
关键词
Cancer predisposition disease; Screening; Beckwith-Wiedemann syndrome; Neurofibromatosis; 1; DICER1; NEUROFIBROMATOSIS TYPE-1; DICER1; MUTATIONS; DIAGNOSIS; MANAGEMENT; CHILDREN;
D O I
10.1007/s00112-022-01540-6
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
Growth and development of the human body and its organs are finely tuned processes that are continuously regulated by numerous genes. Genetically determined defects of this fine regulation can result in growth disorders, e.g. in small or large stature syndromes or in organ malformations. Particularly in cases of germline defects, they can also lead to an increased risk for the development of cancer. In total, more than 50 cancer predisposition syndromes have been identified so far. This review article exemplarily presents three cancer predisposition syndromes relevant to pediatrics, neurofibromatosis type 1, Beckwith-Wiedemann syndrome, and DICER1 syndrome. Screening of these patients is of crucial importance and should be adapted to the expected tumor spectrum. The aim is to detect the development of malignant tumors at an early stage, with better prognosis and less treatment requirements. In addition, follow-up must take into account the possible development of secondary tumors or other secondary diseases. Knowledge of the oncological and nononcological aspects of these cancer predisposition syndromes is therefore a prerequisite for an appropriate and comprehensive care of these patients and thus relevant for pediatric oncologists, pediatric specialists as well as general pediatricians.
引用
收藏
页码:730 / 737
页数:8
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