Chan J K, Fletcher C D, Nayler S J, Cooper K
Department of Pathology, Queen Elizabeth Hospital, Kowloon, Hong Kong.
Cancer. 1997 Jan 15;79(2):294-313.
The goal of this study was to characterize the clinicopathologic features of follicular dendritic cell sarcoma, a very uncommon neoplasm.
The 17 cases were collected from the consultation and surgical pathology files of the authors, including 8 previously reported cases. The histologic and immunohistochemical features and outcome were analyzed.
The patients had a median age of 40 years, with a slight female predominance. Seven patients presented with enlarged lymph nodes, and ten presented with tumor in extranodal sites. Two cases were associated with hyaline-vascular Castleman's disease. The tumors had an average greatest dimension of 6.7 cm. The most common histologic feature was a storiform or fascicular array of spindle, ovoid, or polygonal cells with oval nuclei, delicate nuclear membrane, vesicular or granular chromatin, distinct nucleoli, indistinct cell borders, and frequently fibrillary cytoplasm. There were often scattered multinucleated forms. The tumor cells sometimes formed sheets, circular whorls, follicle-like structures, trabeculae, or pseudovascular spaces. There was a sprinkling of small lymphocytes, with or without cuffing around blood vessels. The neoplastic cells were immunoreactive for CD21 (17 of 17 cases), CD35 (17 of 17 cases), desmoplakin (10 of 17 cases), epithelial membrane antigen (14 of 16 cases), S-100 protein (6 of 17 cases), and CD68 (2 of 17 cases), but not cytokeratin. Ultrastructural studies showed villous processes connected by desmosomes. Only one harbored the Epstein-Barr virus. Among 13 patients with a median follow-up of 3 years, local recurrence occurred in 6, metastasis in 6, and 3 died of disease.
Follicular dendritic cell sarcoma exhibits distinctive histologic features that permit its presumptive recognition, but a firm diagnosis requires confirmation with special studies. Because it has a significant recurrent and metastatic potential (the latter risk having been previously underestimated), it should be viewed as an intermediate grade malignancy. An intraabdominal location is associated with a particularly aggressive clinical course.
本研究的目的是描述滤泡树突状细胞肉瘤这一非常罕见肿瘤的临床病理特征。
从作者的会诊及手术病理档案中收集了17例病例，其中包括8例先前报道过的病例。对其组织学、免疫组化特征及转归进行了分析。
患者的中位年龄为40岁，女性略占优势。7例患者表现为淋巴结肿大，10例患者表现为结外部位肿瘤。2例与透明血管型Castleman病相关。肿瘤的平均最大径为6.7 cm。最常见的组织学特征是梭形、卵圆形或多边形细胞呈车辐状或束状排列，细胞核呈卵圆形，核膜薄，染色质呈泡状或颗粒状，核仁明显，细胞边界不清，胞质常呈纤维状。常有散在的多核形式。肿瘤细胞有时形成片状、圆形涡旋、滤泡样结构、小梁或假血管腔隙。有少量小淋巴细胞，血管周围可有或无淋巴细胞套。肿瘤细胞对CD21（17例中的17例）、CD35（17例中的17例）、桥粒斑蛋白（17例中的10例）、上皮膜抗原（16例中的14例）、S-100蛋白（17例中的6例）和CD68（17例中的2例）呈免疫反应，但对细胞角蛋白无反应。超微结构研究显示有通过桥粒连接的绒毛状突起。仅1例含有EB病毒。在13例中位随访3年的患者中，6例发生局部复发，6例发生转移，3例死于疾病。
滤泡树突状细胞肉瘤具有独特的组织学特征，可据此进行初步识别，但明确诊断需要特殊检查来证实。由于其具有显著的复发和转移潜能（后者的风险此前被低估），应将其视为中度恶性肿瘤。腹腔内发生的该肿瘤具有特别侵袭性的临床病程。
