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目的:探讨咽部淋巴结外滤泡树突细胞肉瘤(Follicular dendritic cell sarcoma,FDCS)的临床病理特点、生物学行为及诊断与治疗要点。方法:对1例咽部结外FDCS进行临床病理观察、免疫组织化学及原位杂交检测,并结合文献进行分析。结果:肿瘤组织位于咽部粘膜下,呈巢团状、片状排列,瘤组织间见多少不一的淋巴细胞浸润;免疫组化显示瘤细胞CD21、CD35、CXCL-13弥漫阳性,CD23、Podoplanin(D2-40)灶性阳性;EBER原位杂交阴性。结合文献分析发现咽部结外FDCS多发生于中青年,无明显性别差异。大多数肿瘤病变比较局限,体积相对较小,可能属于一类低度恶性肿瘤。由于对其认识不足,常常疏于考虑而误诊。据现有病例资料推荐采用根治性手术切除,目前尚没有证据支持术后的辅助放化疗可以改善预后。结论:咽部结外FDCS是一种罕见且容易误诊的低度恶性肿瘤,加深临床及病理医师对该肿瘤的认识有助于减少误诊。
Objective: To investigate the clinicopathological characteristics, biological behavior, diagnosis and treatment of follicular dendritic cell sarcoma (FDCS) in the throat lymph nodes. Methods: One case of pharyngeal extranodal FDCS was examined with clinicopathological and immunohistochemical methods and in situ hybridization, and analyzed with literature. Results: Tumor tissues were located in the pharynx and submucosa with nest-like, flake arrangement and infiltration of lymphocytes in the tumor tissues. Immunohistochemistry showed that the tumor cells were diffusely positive for CD21, CD35 and CXCL-13, CD23, Podoplanin (D2-40) Focal positive; EBER negative in situ hybridization. Combined with literature analysis found that pharyngeal extranodal FDCS occurred in young and middle-aged, no significant gender differences. Most tumor lesions are relatively limited, the volume is relatively small, may belong to a class of low-grade cancer. Because of its lack of understanding, often overlooked and misdiagnosed. According to the current case data recommended radical surgery, there is no evidence to support postoperative adjuvant radiotherapy and chemotherapy can improve the prognosis. Conclusion: FDCS is a rare and easily misdiagnosed low-grade malignant tumor. To deepen the understanding of this tumor by clinicians and pathologists is helpful to reduce misdiagnosis.