SOLITARY FIBROUS TUMOR OF THE MEDIASTINUM - A REPORT OF 14 CASES
SOLITARY FIBROUS TUMOR OF THE MEDIASTINUM - A REPORT OF 14 CASES
复制标题
DOI：
10.1097/00000478-198907000-00002
复制
发表时间：
1989-07-01
影响因子：
5.6
通讯作者：
ROSAI, J
中科院分区：
文献类型：
--
作者：
WITKIN, GB;ROSAI, J
观察到 14 例与胸膜孤立性纤维瘤（所谓纤维性间皮瘤）相同的纵隔肿瘤。病变表现为咳嗽、胸痛、呼吸困难，或放射线检查发现无症状肿块。两名患者患有相关的低血糖。其中 11 个肿瘤位于前上纵隔。一个从胸腺的蒂上产生，另一个则捕获了胸腺成分。肿瘤细胞对波形蛋白和肌动蛋白具有不同的免疫反应性，但对角蛋白没有反应，并且缺乏间皮或上皮分化的超微结构证据。八个病例具有高度细胞有丝分裂活跃区域，其中七个随访病例中有六个表现出攻击性；例外的是带蒂肿瘤。一个 13 厘米、组织学上平淡的肿瘤已经复发两次。攻击性行为比报道的胸膜孤立性纤维瘤更常见，但相同的标准（大小、细胞结构、有丝分裂活性、蒂的存在）具有预后意义。纵隔中出现孤立性纤维瘤，某些病例提示胸腺起源，结合免疫组织化学和超微结构发现，支持该肿瘤的间质起源。鉴别诊断包括梭形细胞胸腺瘤、血管外皮细胞瘤和周围神经肿瘤。
Fourteen cases of a mediastinal neoplasm identical to solitary fibrous tumor (so-called fibrous mesothelioma) of the pleura were observed. The lesions presented with cough, chest pain, dyspnea, or as asymptomatic masses detected radiographically. Two patients had associated hypoglycemia. Eleven of the tumors were in the anterosuperior mediastinum. One arose on a pedicle from the thymus, and another had entrapped thymic elements. Tumor cells were variably immunoreactive for vimentin and actin, but not for keratin, and lacked ultrastructural evidence of mesothelial or epithelial differentiation. Eight cases had highly cellular mitotically active regions of which six of seven with follow-up behaved aggressively; the exception was the pedunculated tumor. A 13-cm, histologically bland tumor has recurred twice. Aggressive behavior was more common than reported for solitary fibrous tumor of the pleura, but the same criteria (size, cellularity, mitotic activity, presence of pedicle) were of prognostic significance. The occurrence of solitary fibrous tumor in the mediastinum with the suggestion of thymic origin for some cases, combined with the immunohistochemical and ultrastructural findings, support a mesenchymal origin for this tumor. The differential diagnosis includes spindle cell thymoma, hemangiopericytoma, and peripheral nerve tumors.