Jagdale Rakhi V, Pol Jaydeep N
Department of Pathology, Shri Siddhivinayak Ganpati Cancer Hospital, Miraj, Maharashtra, India.
Department of Histopathology, Deep Pathology Laboratory, Miraj, Sangli, Maharashtra, India.
Indian J Pathol Microbiol. 2021 Oct-Dec;64(4):759-762. doi: 10.4103/IJPM.IJPM_663_20.
Follicular dendritic cell sarcoma (FDCS) is a rare tumor derived from follicular dendritic cells (FDC) occurring in lymph nodes and extranodal sites. It is usually regarded as an indolent tumor with a tendency of local recurrence but a low risk of metastasis. Common extranodal sites are liver, lung, tonsil, spleen, soft tissue, and mediastinum. Extranodal FDCS of gastrointestinal tract (GIT) is exceedingly rare, with just 36 cases reported in the literature.
We report an unusual case of FDCS of caecum in a 13-year-old boy who presented as intussuception. On histology, it posed a diagnostic challenge for us. An inconclusive initial immunohistochemistry (IHC) lead us to suspect FDCS which was confirmed by FDC markers.
The diagnosis of FDCS at extranodal site like GIT is all the more challenging because of its rarity, morphologic heterogeneity, and lack of awareness.
滤泡性树突状细胞肉瘤（FDCS）是一种罕见的肿瘤，起源于滤泡性树突状细胞（FDC），可发生于淋巴结和结外部位。它通常被视为一种惰性肿瘤，有局部复发倾向，但转移风险较低。常见的结外部位有肝脏、肺、扁桃体、脾脏、软组织和纵隔。胃肠道（GIT）的结外FDCS极为罕见，文献中仅报道了36例。
我们报告了一例13岁男孩盲肠FDCS的罕见病例，该病例表现为肠套叠。在组织学上，它给我们带来了诊断挑战。最初不确定的免疫组化（IHC）结果使我们怀疑为FDCS，最终通过FDC标志物得以确诊。
由于GIT等结外部位的FDCS罕见、形态学异质性以及认识不足，其诊断更具挑战性。
