一例侵袭性胃CIC-DUX4肉瘤行多脏器手术切除：病例报告

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

作者信息

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

机构信息

Department of Surgery, NHO Osaka National Hospital, 2-1-14 Hoenzaka, Chuo-ku, Osaka, 540-0006, Japan.

Department of Gastroenterology and Hepatology, NHO Osaka National Hospital, Osaka, Japan.

出版信息

Surg Case Rep. 2024 Oct 23;10(1):241. doi: 10.1186/s40792-024-02035-0.

DOI:10.1186/s40792-024-02035-0

PMID:39441368

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11499562/

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.

摘要

背景

Capicua转录抑制因子-双同源盒4肉瘤（CDS）是一种罕见且侵袭性强的恶性软组织肿瘤，通常发生于软组织内。我们报告了一例极为罕见的胃CDS病例，尽管其广泛侵犯周围器官，但仍成功切除。

病例介绍

一名48岁男性因腹部肿块逐渐增大就诊。上消化道内镜检查发现胃后壁有一个巨大溃疡性肿瘤。活检结果最初提示为神经内分泌细胞癌。增强计算机断层扫描显示一个20厘米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（胃、胰尾、脾脏和横结肠），实现了R0切除。永久标本的病理检查显示核质比高的小圆形细胞。免疫组织化学染色确诊为CDS。患者恢复良好，术后第33天出院。