Abstract:
Fibroblastic reticulum cell tumor (FRCT) is a rare dendritic neoplasm arising from fibroblastic reticulum cells (FBRCs) and exhibiting peculiar cytokeratin expression. FRCTs usually involve the lymph nodes, although they can also be encountered in the spleen and soft tissues. FRCTs are composed of mildly atypical spindle or ovoid cells, arranged in loose whorls, which express almost invariably low-weight cytokeratins, smooth muscle actin, and CD68. An admixed lymphoplasmacytic infiltrate is also frequently present in solid organ sites. The clinical picture may vary from very indolent to aggressive disease exhibiting features of malignancy, such as cytological pleomorphism, necrosis, or high mitotic rate and metastatic potential. FRCT is a challenging diagnosis, due to its rarity and deceptive cytokeratin expression. Hereafter, we revise the most recent literature regarding such condition and report the case of an extremely indolent splenic FRCT, with no features of malignancy.
摘要:
纤维母细胞网状细胞瘤(FRCT)是一种罕见的树突状肿瘤,由纤维母细胞网状细胞(FBRC)引起,并表现出独特的细胞角蛋白表达。FRCT通常累及淋巴结,尽管它们也可以在脾脏和软组织中遇到。FRCT由轻度不典型的梭形或卵形细胞组成,排列在松散的螺纹中,几乎总是表达低重量的细胞角蛋白,平滑肌肌动蛋白,CD68混合的淋巴浆细胞浸润也经常存在于实体器官部位。临床表现可能从非常惰性到表现出恶性肿瘤特征的侵袭性疾病,如细胞学多态性,坏死,或高有丝分裂率和转移潜力。FRCT是一个具有挑战性的诊断,由于其稀有和欺骗性的细胞角蛋白表达。此后,我们修订了关于这种情况的最新文献,并报道了一个非常惰性的脾FRCT的病例,没有恶性肿瘤的特征.