A germline DICER1 gene mutation is observed in a high percentage of patients with PPB, providing highly significant prognostic and treatment indications for future care [ 4 ]. p53 mutations have been associated with Li–Fraumeni syndrome and numerous neoplasms, including sarcomas, brain tumours, adrenocortical carcinoma and breast cancer, but have not been directly associated with PPB [ 5 ].
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