Journal of Pediatric Surgery Case Reports (Apr 2017)

A female case of pleuropulmonary blastoma type 1 whose pulmonary cystic lesion was followed since neonate

  • Michinobu Ohno,
  • Toshiko Takezoe,
  • Toshihiko Watanabe,
  • Kazunori Tahara,
  • Tomoro Hishiki,
  • Akihiro Fujino,
  • Motomi Matsuo,
  • Masataka Higuchi,
  • Kazuteru Kawasaki,
  • Yoko Shioda,
  • Motohiro Kato,
  • Chikako Kiyotani,
  • Kimikazu Matsumoto,
  • Emi Takakuwa,
  • Rie Irie,
  • Takako Yoshioka,
  • Shunsuke Kimura,
  • Masafumi Seki,
  • Junko Takita,
  • Yutaka Kanamori

DOI
https://doi.org/10.1016/j.epsc.2017.02.009
Journal volume & issue
Vol. 19, no. C
pp. 34 – 37

Abstract

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Pleuropulmonary blastoma (PPB) is a rare malignant mesenchymal tumor. It is classified into 3 subgroups, and PPB type 1 is known to be a cystic lesion with good prognosis. Here, we report a case of PPB type 1 seen in a 1-year-old girl whose pulmonary cystic lesion had been followed-up as a congenital pulmonary airway malformation since neonate. The cyst had been diagnosed as congenital pulmonary airway malformation before surgery but the final diagnosis was type 1 PPB. The tumor had a somatic mutation in exon 25 of the DICER 1 gene whereas no germline mutation was found.

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