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胰腺破骨细胞样巨细胞未分化癌的评估和处理:病例报告及文献复习。

Assessment and management of undifferentiated carcinoma with osteoclastic like giant cells of the pancreas: a case report and revision of literature.

机构信息

Histopathology Unit of National Institute of Gastroenterology "S. de Bellis," Research Hospital, Via Turi 27, 70013, Castellana Grotte, Bari, Italy.

Laboratory of Experimental Immunopathology, National Institute of Gastroenterology "S. de Bellis," Research Hospital, Castellana Grotte, Bari, Italy.

出版信息

BMC Gastroenterol. 2021 Jun 2;21(1):247. doi: 10.1186/s12876-021-01779-5.

Abstract

BACKGROUND

Undifferentiated carcinoma with osteoclast-like giant cells (UCOGCs) is a rare and aggressive non endocrine pancreatic carcinoma characterized by the presence of osteoclastic giant cells mixed with mononuclear cell. Very few cases have been reported in the literature and the histogenesis is controversial as, at the time of diagnosis, the tumor is often of advanced size and stage and it is difficult to pathologically observe its relationship with the pancreatic duct.

CASE PRESENTATION

We present a case of 65-year-old male patient presenting with abdominal pain, nausea, and weight loss, which was treated with surgical resection. Histological examination revealed an undifferentiated pancreatic carcinoma with osteoclast-like giant cells. The patient underwent to a routine pylorus preserving pancreatoduodenectomy. Actually, the patient was in good performance status and disease-free five months.

CONCLUSIONS

Based on the present case and limited previous data, further researches preferably with large cohorts are necessary to clarify the pathogenesis of the neoplasm. However, as show in this case, histopathological and immunohistochemically studies are the gold standard for the diagnosis of UCPOGC. Investigation of the genomic alterations in UPOGCs could help to explain the histologic diversity of variant tumor and could provide a genetic basis for prognosis and treatment.

摘要

背景

破骨细胞样巨细胞未分化癌(UCOGC)是一种罕见且侵袭性的非内分泌胰腺癌,其特征是存在破骨细胞样巨细胞与单核细胞混合。文献中报道的病例非常少,其组织发生存在争议,因为在诊断时,肿瘤往往体积较大且分期较晚,难以从病理上观察其与胰管的关系。

病例介绍

我们报告了一例 65 岁男性患者,因腹痛、恶心和体重减轻就诊,接受了手术切除。组织学检查显示为破骨细胞样巨细胞未分化胰腺癌。患者接受了常规保留幽门的胰十二指肠切除术。实际上,患者的一般情况良好,无疾病困扰已达 5 个月。

结论

基于本病例和有限的既往数据,需要进一步开展研究,最好是采用大样本队列研究,以阐明肿瘤的发病机制。然而,如本例所示,组织病理学和免疫组织化学研究是诊断 UCPOGC 的金标准。对 UPOGC 中基因组改变的研究有助于解释肿瘤的组织学多样性,并为预后和治疗提供遗传基础。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/10d9/8170928/94ba09ea8651/12876_2021_1779_Fig1_HTML.jpg

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