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Type II pleuropulmonary blastoma with DICER1 mutation

Taichi Fukuzawa, Yuki Endo, Masahiro Irie and 15 more

Journal of Pediatric Surgery Case Reports · 2022

doi:10.1016/j.epsc.2022.102468 (opens in a new tab)

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Abstract

Pleuropulmonary blastoma (PPB) is a rare tumor that arises from mesenchymal cells of the lung or pleura. PPB is classified as type I (cystic), type Ir (regressed), type II (cystic and solid), or type III (solid). Here, we report the case of a 2-year-old boy with type II PPB. The patient was treated with neoadjuvant chemotherapy, and the tumor was completely surgically resected. A DICER1 mutation was detected in the tumor specimen. The risk of recurrence is high, and long–term follow–up is required.

Abstract licence: CC BY-NC

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