Yin Wei-hua, Yu Guang-yin, Ma Ya, Rao Hui-lan, Lin Su-xia, Shao Chun-kui, Liang Qiong, Guo Na, Chen Guo-qin, Zhou Wei, Zhao Tong, Zhu Mei-gang
Department of Pathology, Peking University Shenzhen Hospital, China.
Zhonghua Bing Li Xue Za Zhi. 2010 Aug;39(8):522-7.
To study the clinicopathologic features of follicular dendritic cell sarcoma (FDCS) and its differential diagnosis.
Ten cases of FDCS were studied by light microscopy, immunohistochemistry and in-situ hybridization. The clinical features and follow-up information were analyzed.
Amongst the 10 cases of FDCS studied, the male-to-female ratio was 1:1. The mean age of the patients was 42 years. Six of them were located in cervical and peritoneal lymph nodes and four in extranodal sites (including tonsil, pelvic cavity, tail of pancreas and spleen). Histologically, the tumor cells had whorled, storiform or diffuse growth patterns. They were spindle in shape and contained syncytial eosinophilic cytoplasm, with round or oval nuclei, vesicular chromatin, distinct nucleoli and a variable number of mitotic figures. Multinucleated tumor giant cells and intranuclear pseudoinclusions were occasionally seen. There was a sprinkling of small lymphocytes and neutrophils within the tumor as well as in the perivascular region. Immunohistochemical study showed that the tumor cells were diffusely or focally positive for CD21, CD23, CD35 and D2-40, but negative for LCA, CD20, CD3, CD1a, HMB45 and CK. Some of them showed EMA, CD68 and S-100 reactivity. In-situ hybridization for Epstein-Barr virus-encoded RNA (EBER) showed positive signals in only one case (which was diagnosed as inflammatory pseudotumor-like FDCS). Of the 7 patients with follow-up information available (duration: 2 months to 39 months; mean: 14 months), 2 cases with paraneoplastic pemphigus died of pulmonary infection at 5 and 7 months respectively. The remaining 5 patients were alive and disease-free after surgical excision (+/- chemotherapy and radiotherapy).
FDCS is a rare low to intermediate-grade malignant tumor. Appropriate application of FDC markers, such as CD21, CD35 and D2-40, would be helpful for arriving at a correct diagnosis. Most cases are associated with good prognosis after surgical treatment, with or without chemotherapy and radiotherapy. Patients with paraneoplastic pemphigus carry a less favorable prognosis.
研究滤泡树突状细胞肉瘤（FDCS）的临床病理特征及其鉴别诊断。
对10例FDCS进行光镜、免疫组化和原位杂交研究，并分析其临床特征及随访信息。
在研究的10例FDCS中，男女比例为1:1。患者平均年龄42岁。其中6例位于颈部和腹膜后淋巴结，4例位于结外部位（包括扁桃体、盆腔、胰尾和脾脏）。组织学上，肿瘤细胞呈漩涡状、束状或弥漫性生长模式。细胞呈梭形，有合体性嗜酸性细胞质，核圆形或椭圆形，核染色质呈泡状，核仁明显，有数量不等的有丝分裂象。偶尔可见多核肿瘤巨细胞和核内假包涵体。肿瘤内及血管周围区域有少量小淋巴细胞和中性粒细胞散在分布。免疫组化研究显示，肿瘤细胞CD21、CD23、CD35和D2-40弥漫性或局灶性阳性，但LCA、CD20、CD3、CD1a、HMB45和CK阴性。部分病例EMA、CD68和S-100呈阳性反应。爱泼斯坦-巴尔病毒编码RNA（EBER）原位杂交仅1例呈阳性信号（该例诊断为炎症性假瘤样FDCS）。7例有随访信息的患者（随访时间：2个月至39个月，平均14个月），2例伴副肿瘤性天疱疮的患者分别于5个月和7个月死于肺部感染。其余5例患者手术切除（±化疗和放疗）后存活且无疾病复发。
FDCS是一种罕见的低至中级恶性肿瘤。合理应用FDC标志物如CD21、CD35和D2-40有助于正确诊断。大多数病例手术治疗后预后良好，无论是否联合化疗和放疗。伴副肿瘤性天疱疮的患者预后较差。
