Chang Yun-Chen, Chau Ivy Yenwen, Yeh Yi-Chen, Chau Gar-Yang
School of Medicine, National Yang-Ming University, Taipei, Taiwan Poznan University of Medical Science Medical Faculty II, Poznan, Poland Division of General Surgery, Department of Surgery Department of Pathology, Taipei Veterans General Hospital, Taipei, Taiwan.
Medicine (Baltimore). 2017 Aug;96(31):e7261. doi: 10.1097/MD.0000000000007261.
Follicular dendritic cell sarcoma (FDCS) is a rare neoplasia composed of spindle or oval cells with follicular dendritic cell differentiation, usually occurring in lymphoid tissue. In this report, we present a case of FDCS of the small intestine with liver metastasis.
A 19-year-old female presented with recent onset of left upper abdominal pain. Abdominal computed tomography scan showed a large tumor mass in the liver lateral segment with compression to the pancreas upper part, and a smaller mass in the terminal ileum, respectively. High serum levels of amylase and lipase were noted. Resection of the tumors was performed. Microscopically, both tumors consisted of ovoid to spindle-shaped nuclei cells admixed with some lymphocytes arranged in fascicles, whorls, storiform arrays. Immunohistochemistry demonstrated that the tumor cells were positive for follicular dendritic cell markers, including CD21, CD23, and CD35. Epstein-Barr virus encoding small RNA (EBER; Inform EBER probe; Ventana Medical Systems, Tucson, AZ) in situ hybridization was negative.
According to the clinicopathological features, diagnosis of FDCS of intestinal origin was made.
Resection of tumors located in the liver and at the small intestine was performed. After the operation, patient received adjuvant vinblastin chemotherapy.
There was no evidence of recurrence at 8-month follow-up.
It was unusual for FDCS of intestinal origin with liver metastasis and expressing with high serum levels of pancreatic enzymes.
滤泡性树突状细胞肉瘤（FDCS）是一种罕见的肿瘤，由具有滤泡性树突状细胞分化的梭形或椭圆形细胞组成，通常发生于淋巴组织。在本报告中，我们呈现了一例发生小肠且伴有肝转移的FDCS病例。
一名19岁女性因近期出现左上腹疼痛就诊。腹部计算机断层扫描显示肝脏外侧段有一个大肿瘤块压迫胰腺上部，回肠末端有一个较小肿块。血清淀粉酶和脂肪酶水平升高。对肿瘤进行了切除。显微镜下，两个肿瘤均由卵圆形至梭形核细胞组成，混有一些呈束状、漩涡状、席纹状排列的淋巴细胞。免疫组化显示肿瘤细胞对滤泡性树突状细胞标志物呈阳性，包括CD21、CD23和CD35。爱泼斯坦-巴尔病毒编码小RNA（EBER；Inform EBER探针；Ventana Medical Systems，图森，亚利桑那州）原位杂交为阴性。
根据临床病理特征，诊断为肠道来源的FDCS。
对位于肝脏和小肠的肿瘤进行了切除。术后患者接受了长春碱辅助化疗。
8个月随访时无复发迹象。
肠道来源的FDCS伴有肝转移且血清胰酶水平升高实属罕见。
