Lucioni M, Boveri E, Rosso R, Benazzo M, Necchi V, Danova M, Incardona P, Franco C, Viglio A, Riboni R, Lazzarino M, Magrini U, Canevari A, Paulli M
Pathology Section, Department of Human Pathology, I.R.C.C.S. Policlinico S. Matteo, University of Pavia, Italy.
Histopathology. 2003 Dec;43(6):583-91. doi: 10.1111/j.1365-2559.2003.01725.x.
To detail on sequential biopsies the morphological and immunohistochemical features of a case of primary lymph nodal fibroblastic reticulum cell (FBRC) tumour which progressed into a clinically aggressive cytokeratin-positive interstitial reticulum cell (CIRC) sarcoma.
A 70-year-old female underwent surgical excision of an enlarged submandibular lymph node. The nodal architecture was effaced by a neoplastic proliferation of medium to large cells, round to oval to spindle in shape, growing in a storiform pattern. The tumour stained for vimentin, CD68, factor XIIIa, alpha1-antitrypsin, fascin and actin. Dendritic and endothelial cell markers were negative. A diagnosis of FBRC tumour was made by combining pathological and clinical data. The patient received no therapy but 5 months later the tumour relapsed, exhibiting a deceptively pleomorphic cytology, phenotypic changes (strong cytokeratin positivity), intense p53 expression and aggressive clinical course with fatal outcome. In-situ hybridization for Epstein-Barr virus was negative.
We speculate that the morphological changes and p53 expression of the relapsing neoplasm might reflect tumour cell dedifferentiation, in keeping with the aggressive clinical course. The intense p53 expression suggests that this oncoprotein might also play a role in reticulum cell tumorigenesis.
详细描述一例原发性淋巴结成纤维细胞性网状细胞（FBRC）肿瘤的连续活检的形态学和免疫组化特征，该肿瘤进展为具有临床侵袭性的细胞角蛋白阳性间质网状细胞（CIRC）肉瘤。
一名70岁女性接受了下颌下肿大淋巴结的手术切除。肿瘤性增殖的中到大细胞使淋巴结结构消失，细胞呈圆形、椭圆形至梭形，呈席纹状生长。肿瘤对波形蛋白、CD68、因子ⅩⅢa、α1-抗胰蛋白酶、肌动蛋白束蛋白和肌动蛋白染色阳性。树突状细胞和内皮细胞标记物阴性。结合病理和临床资料诊断为FBRC肿瘤。患者未接受治疗，但5个月后肿瘤复发，表现出具有欺骗性的多形性细胞学、表型改变（强烈的细胞角蛋白阳性）、强烈的p53表达以及具有致命结局的侵袭性临床病程。爱泼斯坦-巴尔病毒原位杂交阴性。
我们推测复发性肿瘤的形态学改变和p53表达可能反映肿瘤细胞去分化，这与侵袭性临床病程一致。强烈的p53表达表明这种癌蛋白可能也在网状细胞肿瘤发生中起作用。
