Primary alveolar rhabdomyosarcoma of the diaphragm requiring proximal gastrectomy

Yuki Mizuno, Naoki Shimojima, Atsushi Makimoto, Yuichi Yokokawa, Kazuaki Miyaguni, Ayano Tsukizaki, Makoto Hashimoto, Miki Ishikawa, Hideo Ishihama, Hirofumi Tomita, Akihiro Shimotakahara, Yuki Yuza, Kentaro Matsuoka, Hirofumi Kawakubo, Seiichi Hirobe

Research output: Contribution to journalArticlepeer-review

1 Citation (Scopus)


Primary rhabdomyosarcoma (RMS) of the diaphragm, especially alveolar RMS, is extremely rare. Because of its rarity, no treatment for it has yet been established, but previous studies have reported long-term survival in recipients of a combination therapy with local control, including a total resection and/or radiation therapy concomitant with multiagent chemotherapy. We experienced a case of primary alveolar rhabdomyosarcoma of the diaphragm surrounding the gastric cardia in a 15-year-old, female patient. After administering chemotherapy consisting of vincristine, actinomycin D, and cyclophosphamide (VAC) to shrink the tumor, tumor resection was performed with a proximal gastrectomy and partial resection of the diaphragm, pericardium, and left lung. After receiving postoperative adjuvant chemotherapy with VAC and radiotherapy at a total of 41.4 Gy, the patient has been relapse-free for 18 months. The tumor-induced stenosis at the hilum was corrected, allowing oral intake and markedly improving the patient's nutritional status. Tumor resection with a proximal gastrectomy for primary diaphragmatic RMS surrounding the lower esophagus is recommended both for its efficacy in local control and for allowing the preservation of postoperative gastrointestinal function.

Original languageEnglish
Article number102206
JournalJournal of Pediatric Surgery Case Reports
Publication statusPublished - 2022 Mar


  • Alveolar rhabdomyosarcoma
  • Diaphragm
  • Proximal gastrectomy

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Surgery


Dive into the research topics of 'Primary alveolar rhabdomyosarcoma of the diaphragm requiring proximal gastrectomy'. Together they form a unique fingerprint.

Cite this