Polypoid angiomyofibroblastoma-like tumor of the oral cavity: a hitherto unreported soft tissue tumor mimicking embryonal rhabdomyosarcoma
Polypoid angiomyofibroblastoma-like tumor of the oral cavity: a hitherto unreported soft tissue tumor mimicking embryonal rhabdomyosarcoma
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DOI：
10.1016/j.prp.2008.05.007
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发表时间：
2008-01-01
影响因子：
2.8
通讯作者：
Ninfo, Vito
中科院分区：
文献类型：
--
作者：
Magro, Gaetano;Greco, Paolo;Ninfo, Vito
我们报告一个以前未被识别的纤维-肌纤维母细胞肿瘤在口腔的一个15岁的女孩。在形态学上，肿瘤类似于横纹肌肉瘤，botroid变体。它由具有有丝分裂活性的小到中型、波形蛋白+ /聚蛋白+、圆形到卵圆形到上皮样形状的细胞组成，包埋在纤维状到黏液状/水肿的交替基质中。这些细胞被一层纤维间质边缘从上覆的鳞状上皮中分离出来。肿瘤含有丰富的中小薄壁血管，未见透明化。通常，肿瘤细胞在这些血管周围凝聚。一个不寻常且显著的特征是存在大量透明化的胶原垫，包括“类淀粉样纤维”，与肌成纤维细胞瘤中观察到的相似。这些胶原垫的存在和desmin的表达，与肌原素和MyoD1无免疫反应性相关，符合肿瘤的纤维-肌成纤维性质，排除胚胎性横纹肌肉瘤的诊断。关于纤维-肌纤维母细胞肿瘤，我们认为本病例属于良性间质肿瘤的广泛范围，最初描述为下女性生殖道，但也可能发生在生殖器外部位。由于形态和免疫组织化学特征与血管肌纤维母细胞瘤相似，但又不完全相同，因此提出“息肉样血管肌纤维母细胞瘤样肿瘤”这一术语。意识和认识这种肿瘤是至关重要的，以避免恶性诊断。(C) 2008爱思唯尔有限公司版权所有。
We report on a previously unrecognized fibro-myofibroblastic tumor in the oral cavity of a 15-year-old girl. Morphologically, the tumor mimicked a rhabdomyosarcoma, botryoid variant. It was composed of mitotically active small- to medium-sized, vimentin + /desmin +, round- to oval- to epithelioid-shaped cells embedded in an alternating fibrous to myxoid/edematous stroma. These cells were separated from the overlying squamous epithelium by a rim of fibrous stroma. The tumor contained abundant small- to medium-sized, thin-walled blood vessels without hyalinization. Frequently, neoplastic cells condensed around these vessels. An unusual and striking feature was the presence of numerous hyalinized collagen mats, including "amianthoid-like fibers", similar to those observed in myofibroblastomas. The presence of these collagen mats and the expression of desmin, in association with no immunoreactivity to myogenin and MyoD1, were in keeping with the fibro-myofibroblastic nature of the tumor, excluding the diagnosis of embryonal rhabdomyosarcoma. Regarding fibro-myofibroblastic tumors, we believe that the present case falls within the wide spectrum of benign stromal tumors, originally described in the lower female genital tract, but potentially occurring also at extragenital sites. As morphological and immunohistochemical features were reminiscent of, but not identical with, angiomyofibroblastoma, the term "polypoid angiomyofibroblastoma-like tumor" is proposed. Awareness and recognition of this tumor is crucial to avoid a diagnosis of malignancy. (C) 2008 Elsevier GmbH. All rights reserved.