Article
Outcome of two pairs of monozygotic twins with pleuropulmonary blastoma: case report.
Italian journal of pediatrics - 7 Oct 2020
Zhang Shihan, Wang Xisi, Li Sihui, Cai Siyu, Yu Tong, Fu Libing, Zhang Na, Peng Xiaoxia, Zeng Qi, Ma Xiaoli
Abstract excerpt
BACKGROUND: Pleuropulmonary blastomas (PPB) are rare aggressive paediatric lung malignancies and are among the most common DICER1-related disorders: it is estimated that 75-80% of children with a PPB have the DICER1 mutation. DICER1 mutations are responsible for familial tumour susceptibility syndrome with an increased risk of tumours. In approximately 35% of families with children manifesting PPB, further...
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