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

IF 0.7 Q4 SURGERY Surgical Case Reports Pub Date : 2024-10-23 DOI:10.1186/s40792-024-02035-0

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

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Abstract

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.

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手术切除多脏器的侵袭性胃 CIC-DUX4 肉瘤：病例报告。

背景：Capicua 转录抑制因子-双同源框 4 肉瘤（CDS）是一种罕见的侵袭性恶性软组织肿瘤，通常发生在软组织内。我们报告了一例非常罕见的胃CDS病例，尽管该病广泛侵犯周围器官，但仍被成功切除：一名 48 岁的男性因腹部肿块逐渐增大而就诊。上消化道内镜检查发现胃后壁有一个巨大的溃疡性肿瘤。活检结果初步提示为神经内分泌细胞癌。对比增强计算机断层扫描显示，20 厘米长的肿瘤从胃后壁突出，直接侵犯胰腺和结肠。我们进行了多脏器切除术（胃、胰尾、脾脏和横结肠），实现了 R0 切除。永久标本的病理检查发现了核质比高的小圆形细胞。免疫组化染色证实了 CDS 的诊断。患者恢复良好，术后第 33 天出院：本病例报告首次详细描述了胃部侵袭性 CDS 的手术切除情况。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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