An aggressive gastric CIC-DUX4 sarcoma surgically resected with multivisceral organs: a case report

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2024年10月23日

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Mizuki Koba,
Atsushi Takeno,
Hiroko Hasegawa,
Ryotaro Sakamori,
Rei Higashiura,
Masaaki Yamamoto,
Shinji Tokuyama,
Reishi Toshiyama,
Kenji Kawai,
Yusuke Takahashi,
Kenji Sakai,
Naoki Hama,
Kunihito Gotoh,
Takeshi Kato,
Yumiko Hirose,
Kiyoshi Mori,
Masayuki Mano,
Motohiro Hirao

摘要

Background

Capicua transcriptional repressor-double homeobox 4 sarcoma (CDS) is a rare and aggressive malignant soft tissue tumor that typically arises within the soft tissues. We report an exceptionally rare case of a gastric CDS successfully resected despite its extensive invasion into surrounding organs.

Case presentation

A 48-year-old male presented with a progressively enlarging abdominal mass. Upper gastrointestinal endoscopy revealed a large ulcerative tumor on the posterior gastric wall. Biopsy results initially suggested a neuroendocrine cell carcinoma. Contrast-enhanced computed tomography showed a 20 cm tumor protruding from the posterior stomach wall, directly invading the pancreas and colon. We performed a multivisceral resection (stomach, pancreatic tail, spleen, and transverse colon) achieving an R0 resection. Pathological examination of the permanent specimen revealed small round cells with high nuclear-to-cytoplasmic ratios. Immunohistochemical staining confirmed the diagnosis of CDS. The patient recovered well and was discharged on postoperative day 33.

Conclusions

This case report describes the first detailed account of a surgically resected aggressive CDS originating from the stomach.