Department of Internal Medicine, Chungbuk National University College of Medicine, Cheongju, Korea.
J Korean Med Sci. 2011 Feb;26(2):304-7. doi: 10.3346/jkms.2011.26.2.304. Epub 2011 Jan 24.
Interdigitating dendritic cell sarcoma (IDCS) is an extremely rare neoplasm arising from the antigen-presenting cells of the immune system. This disease usually involves the lymph nodes, and rarely, extranodal sites may be affected. The authors report a case of extranodal IDCS presenting in the pleura. A 32-yr-old man presented with progressive chest pain. Imaging studies showed diffuse pleural thickening with pleural effusion. Morphological and immunohistochemical analysis of an incisional biopsy of the pleura were consistent with a diagnosis of IDCS; tumor cells were positive for S100 and CD45, but negative for CD1a, CD21, CD35, B cell and T cell markers. The patient was administered chemotherapy, but died of progressive disease. Although its incidence is extremely rare, this case suggests that extranodal IDCS should be considered in the differential diagnosis of undifferentiated neoplasms and that immunohistochemical staining be performed using appropriate markers.
指状突树突细胞肉瘤（IDCS）是一种极为罕见的肿瘤，起源于免疫系统的抗原呈递细胞。这种疾病通常累及淋巴结，很少累及结外部位。作者报告了一例发生在胸膜的结外 IDCS 病例。一名 32 岁男性因进行性胸痛就诊。影像学研究显示弥漫性胸膜增厚伴胸腔积液。胸膜切开活检的形态学和免疫组织化学分析符合 IDCS 的诊断；肿瘤细胞对 S100 和 CD45 呈阳性，但对 CD1a、CD21、CD35、B 细胞和 T 细胞标志物呈阴性。患者接受了化疗，但死于疾病进展。尽管发病率极低，但该病例提示在鉴别诊断未分化肿瘤时应考虑结外 IDCS，并使用适当的标志物进行免疫组织化学染色。
