Pillay K, Solomon R, Daubenton J D, Sinclair-Smith C C
Department of Pathology, Red Cross War Memorial Children's Hospital/University of Cape Town Medical School, Cape Town, South Africa.
Histopathology. 2004 Mar;44(3):283-91. doi: 10.1111/j.0309-0167.2004.01816.x.
To report a series of four paediatric cases of interdigitating dendritic cell sarcoma (IDCS) and add to the known extranodal sites of occurrence for this tumour. Neoplasms derived from interdigitating dendritic cells are rare, with only 33 cases being reported in the literature (Medline search). These tumours usually occur in lymph nodes in the adult population.
The patients were a 10-year-old girl with a large soft tissue mass bulging into the left chest, a 12-year-old girl with a right paraspinal mass, a 21-month-old boy with generalized lymphadenopathy and hepatosplenomegaly and a 6-year-old girl with a large bladder mass. Paraffin blocks and haematoxylin and eosin slides were available in all cases. In addition, immunohistochemistry and electron microscopy were performed. A diagnosis of IDCS was made in all cases.
The diagnosis of IDCS can rarely be entertained on clinical information alone. Microscopically, there is a wide spectrum of features. Thus, immunohistochemistry and electron microscopy are crucial in making the diagnosis. The differential diagnosis includes inflammatory pseudotumour, follicular dendritic cell sarcoma, true histiocytic lymphoma, malignant Langerhans cell histiocytosis, anaplastic large-cell lymphoma, melanoma, and a range of sarcomas. IDCS displays aggressive behaviour and approximately half of the patients die of the disease.
报告4例儿童指突状树突细胞肉瘤（IDCS）病例系列，并补充该肿瘤已知的结外发生部位。源自指突状树突细胞的肿瘤较为罕见，文献（Medline检索）中仅报道了33例。这些肿瘤通常发生于成人的淋巴结。
患者包括一名10岁女童，有一个突入左胸的巨大软组织肿块；一名12岁女童，有一个右侧椎旁肿块；一名21个月大的男童，有全身淋巴结肿大和肝脾肿大；以及一名6岁女童，有一个巨大的膀胱肿块。所有病例均有石蜡块及苏木精-伊红染色切片。此外，还进行了免疫组织化学和电子显微镜检查。所有病例均诊断为IDCS。
仅根据临床信息很少能考虑到IDCS的诊断。在显微镜下，其特征范围广泛。因此，免疫组织化学和电子显微镜检查对做出诊断至关重要。鉴别诊断包括炎性假瘤、滤泡树突细胞肉瘤、真性组织细胞淋巴瘤、恶性朗格汉斯细胞组织细胞增多症、间变性大细胞淋巴瘤、黑色素瘤以及一系列肉瘤。IDCS表现出侵袭性，约半数患者死于该病。
