Witkin G B, Rosai J
Department of Pathology, Yale University School of Medicine, New Haven, CT 06510.
Am J Surg Pathol. 1989 Jul;13(7):547-57. doi: 10.1097/00000478-198907000-00002.
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 antero-superior 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.
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。这些病变表现为咳嗽、胸痛、呼吸困难,或为影像学检查发现的无症状肿块。两名患者伴有低血糖。11个肿瘤位于前上纵隔。一个肿瘤起源于带蒂的胸腺,另一个包裹着胸腺组织。肿瘤细胞对波形蛋白和肌动蛋白呈不同程度的免疫反应,但对角蛋白无反应,且缺乏间皮或上皮分化的超微结构证据。8例有细胞高度丰富、有丝分裂活跃的区域,其中7例中有6例随访时表现为侵袭性生长;带蒂肿瘤为例外。一个13厘米的组织学表现温和的肿瘤已复发两次。侵袭性行为比胸膜孤立性纤维瘤报道的更为常见,但相同的标准(大小、细胞丰富度、有丝分裂活性、有无蒂)具有预后意义。纵隔出现孤立性纤维瘤,部分病例提示起源于胸腺,结合免疫组化和超微结构结果,支持该肿瘤起源于间叶组织。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。