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
Affiliations
- Michinobu Ohno
- Division of Surgery, Department of Surgical Specialties, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan
- Toshiko Takezoe
- Division of Surgery, Department of Surgical Specialties, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan
- Toshihiko Watanabe
- Division of Surgery, Department of Surgical Specialties, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan
- Kazunori Tahara
- Division of Surgery, Department of Surgical Specialties, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan
- Tomoro Hishiki
- Division of Surgery, Department of Surgical Specialties, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan
- Akihiro Fujino
- Division of Surgery, Department of Surgical Specialties, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan
- Motomi Matsuo
- Division of Pulmonology, Department of Medical Subspecialties, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan
- Masataka Higuchi
- Division of Pulmonology, Department of Medical Subspecialties, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan
- Kazuteru Kawasaki
- Division of Pulmonology, Department of Medical Subspecialties, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan
- Yoko Shioda
- Division of Solid Tumor, Children's Cancer Center, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan
- Motohiro Kato
- Division of Solid Tumor, Children's Cancer Center, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan
- Chikako Kiyotani
- Division of Solid Tumor, Children's Cancer Center, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan
- Kimikazu Matsumoto
- Division of Solid Tumor, Children's Cancer Center, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan
- Emi Takakuwa
- Department of Pathology, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan
- Rie Irie
- Department of Pathology, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan
- Takako Yoshioka
- Department of Pathology, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan
- Shunsuke Kimura
- Department of Pediatrics, The University of Tokyo, Japan
- Masafumi Seki
- Department of Pediatrics, The University of Tokyo, Japan
- Junko Takita
- Department of Pediatrics, The University of Tokyo, Japan
- Yutaka Kanamori
- Division of Surgery, Department of Surgical Specialties, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan
- DOI
- https://doi.org/10.1016/j.epsc.2017.02.009
- Journal volume & issue
-
Vol. 19,
no. C
pp. 34 – 37
Abstract
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.
Keywords