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胃母细胞瘤,无 GLI1 和 EWSR1 基因断裂。

Gastroblastoma without GLI1 and EWSR1 gene breaks.

机构信息

Department of Clinical Pathology, Liuzhou Workers' Hospital, Guangxi, China.

Department of Gastrointestinal Surgery, Liuzhou Workers' Hospital, Guangxi, China.

出版信息

World J Surg Oncol. 2023 Sep 1;21(1):274. doi: 10.1186/s12957-023-03159-7.

DOI:10.1186/s12957-023-03159-7
PMID:37658451
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10472644/
Abstract

OBJECTIVE

To report a rare gastroblastoma; discuss its clinical features, histopathological morphology, diagnosis, differential diagnosis, treatment, and prognosis; and so as to improve the understanding on this disease and provide reference for its diagnosis, treatment, and prognosis.

METHODS

The diagnosis and treatment, imaging examination, pathological, and genetic data of a 19-year-old young female patient with gastroblastoma were analyzed retrospectively, and the relevant literature was reviewed and summarized.

RESULTS

The patient was found to have a "gastrointestinal stromal tumor" for 3 days by physical examination in another hospital. Abdominal CT and MRI considered "solid pseudopapilloma of pancreas" and clinically planned to perform "radical pancreatoduodenectomy." During the operation, the tumor was observed to bulge from the posterior wall of the gastric antrum, and the root was located in the gastric antrum, so it was changed to "partial gastrectomy + Ronx-y gastrojejunal anastomosis." The postoperative pathology showed that the tumor was bi-differentiated between gastric epithelium and mesenchymal. Combined with the results of IHC and the opinions of several consultation units, the diagnosis of gastric blastoma (low-grade malignancy) was supported. However, the fracture rearrangement of GLI1 and EWSR1 genes was not detected by FISH. After 19 months of follow-up, no signs of tumor recurrence and metastasis were found.

CONCLUSION

Combined with existing literature reports, gastroblastoma occurs in young people, equally in men and women, and tends to occur in the gastric antrum. The biological behavior of the tumor tends to be inert, and the prognosis of most cases is good. Postoperative pathology and IHC are reliable methods for the diagnosis of gastric blastoma, and surgical resection of the lesion is the preferred treatment.

摘要

目的

报道 1 例罕见的胃胚细胞瘤,探讨其临床特征、组织病理学形态、诊断、鉴别诊断、治疗及预后,提高对本病的认识,为其诊断、治疗及预后提供参考。

方法

回顾性分析 1 例 19 岁女性胃胚细胞瘤患者的诊治、影像学检查、病理及基因等资料,并复习相关文献进行总结。

结果

患者因体检在外院发现“胃间质瘤”3 d,腹部 CT、MRI 考虑“胰腺实性假乳头状瘤”,临床拟行“胰十二指肠根治术”。术中见肿瘤自胃窦后壁向腔外突出,根部位于胃窦,遂改行“胃部分切除+毕Ⅱ式胃肠吻合术”。术后病理示肿瘤胃上皮和间叶双向分化,结合免疫组化结果及多会诊单位意见,支持胃胚细胞瘤(低度恶性)诊断,但未检测到 FISH 断裂重排的 GLI1 和 EWSR1 基因。随访 19 个月,未见肿瘤复发转移征象。

结论

结合现有文献报道,胃胚细胞瘤好发于青年人,男女发病无明显差异,多发生于胃窦部。肿瘤生物学行为倾向惰性,多数病例预后良好。术后病理及免疫组化是诊断胃胚细胞瘤的可靠方法,手术完整切除病灶是首选治疗方案。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7fac/10472644/c10e8296b06d/12957_2023_3159_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7fac/10472644/ad5ff3cccae5/12957_2023_3159_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7fac/10472644/95053249df45/12957_2023_3159_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7fac/10472644/5d7935ae6c9e/12957_2023_3159_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7fac/10472644/c10e8296b06d/12957_2023_3159_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7fac/10472644/ad5ff3cccae5/12957_2023_3159_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7fac/10472644/95053249df45/12957_2023_3159_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7fac/10472644/5d7935ae6c9e/12957_2023_3159_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7fac/10472644/c10e8296b06d/12957_2023_3159_Fig4_HTML.jpg

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Gastroblastoma, a biphasic neoplasm of stomach: A case report.
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The SCARE Statement: Consensus-based surgical case report guidelines.SCARE 声明:基于共识的外科手术病例报告指南。
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