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胰腺滤泡树突状细胞肉瘤:一例报告

Pancreatic follicular dendritic cell sarcoma: a case report.

作者信息

Lou Jing, Xia Runyu, Li Guoli, Su Jun, Zheng Hong

机构信息

Department of Pathology, The Affiliated Hospital of Zunyi Medical University, Zunyi City, Guizhou Province, People's Republic of China.

出版信息

World J Surg Oncol. 2023 Oct 13;21(1):327. doi: 10.1186/s12957-023-03213-4.

Abstract

BACKGROUND

Follicular dendritic cell sarcoma (FDCS) is a rare, low-to-moderate-grade malignant tumor, which occurs in the dendritic cells of the germinal center. Pancreatic FDCS (PFDCS) is extremely rare, with only a few reported cases. Presently, the etiology and pathogenesis of pancreatic FDCS are still unclear, and the clinical symptoms and signs as well as the laboratory diagnosis lack specificity. Although PFDCS presents better histological and morphological characteristics and a distinct immunophenotype, it can be easily missed and/or misdiagnosed if it occurs outside the node. Lymph node FDCS are easier to diagnose because of the rarity of fusiform cell tumors in lymph nodes.

CASE DEMONSTRATION

Herein, we reported a 67-year-old female patient with upper-left abdominal pain without obvious cause and was admitted for treatment. A computed tomography (CT) scan revealed a cystic solid mass in the pancreatic tail toward the greater curvature of the stomach, with an obvious enhancement of the cyst wall in enhanced scanning. Subsequently, the patient underwent surgical resection and the resected sample was sent for pathological biopsy. According to the results, the pathology was consistent with the histological morphology and immunohistochemical characteristics of FDCS, and the Epstein-Barr virus (EBV)-encoded RNA was negative for in situ hybridization. Three months post-resection, the patient returned to the hospital for chemotherapy. This case report is aimed to improve the clinical recognition of FDCS.

CONCLUSION

Pancreatic FDCS is a rare disease. Herein, we have reported a case of pancreatic FDCS and analyzed its clinical and pathological features and differential diagnosis to improve the understanding of FDCS.

摘要

背景

滤泡性树突状细胞肉瘤(FDCS)是一种罕见的低至中度恶性肿瘤,发生于生发中心的树突状细胞。胰腺FDCS(PFDCS)极为罕见,仅有少数病例报道。目前,PFDCS的病因和发病机制仍不清楚,其临床症状、体征及实验室诊断均缺乏特异性。尽管PFDCS具有较好的组织学和形态学特征以及独特的免疫表型,但如果发生在淋巴结外则很容易被漏诊和/或误诊。淋巴结FDCS因淋巴结中梭形细胞瘤罕见而更容易诊断。

病例展示

在此,我们报告了一名67岁女性患者,因左上腹无明显诱因疼痛入院治疗。计算机断层扫描(CT)显示胰尾向胃大弯处有一囊实性肿块,增强扫描时囊壁明显强化。随后,患者接受了手术切除,并将切除的样本送去做病理活检。根据结果,病理与FDCS的组织形态学和免疫组化特征一致,原位杂交检测爱泼斯坦-巴尔病毒(EBV)编码RNA为阴性。切除术后三个月,患者返回医院进行化疗。本病例报告旨在提高对FDCS的临床认识。

结论

胰腺FDCS是一种罕见疾病。在此,我们报告了一例胰腺FDCS病例,并分析了其临床和病理特征及鉴别诊断,以增进对FDCS的了解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/75be/10571425/fb4522388ae0/12957_2023_3213_Fig1_HTML.jpg

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