Journal of Pediatric Surgery Case Reports (Dec 2022)

Type II pleuropulmonary blastoma with DICER1 mutation

  • Taichi Fukuzawa,
  • Yuki Endo,
  • Masahiro Irie,
  • Hideyuki Sasaki,
  • Hironori Kudo,
  • Megumi Nakamura,
  • Ryo Ando,
  • Ryuji Okubo,
  • Tsuyoshi Sakurai,
  • Masatoshi Hashimoto,
  • Keisuke Tada,
  • Yudai Nakajima,
  • Kosuke Sato,
  • Ryoma Endo,
  • Hidekazu Aoki,
  • Saori Katayama,
  • Tomohiro Nakano,
  • Motoshi Wada

Journal volume & issue
Vol. 87
p. 102468

Abstract

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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.

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