A Giant Atypical Neurofibroma in the Right Thoracic Cavity of a 57-Year-Old Man: A Case Report with Review of the Literature

Rongjun Guo, Frank Chen, R. Heffner
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引用次数: 2

Abstract

Intrathoracic neurogenic tumors are not uncommon, but presentation as a giant mass in the thoracic cavity is rare. Although several cases of intrathoracic giant malignant peripheral nerve sheath tumor have been reported, only one case of intrathoracic giant benign neurofibroma appears in the literature. In this report, we describe a very rare case of atypical giant neurofibroma in the right thoracic cavity. The patient was a 57 year old African American man, who developed sudden cardiac arrest and passed away in the emergency room. At autopsy, a huge encapsulated firm tumor was found in the right thoracic cavity, attached to the vertebral bodies and superficially adherent to the upper and middle lobes of the right lung. This giant mass weighed 2140 grams and measured 31 x 30 x 5.5 cm. Microscopically, the tumor consisted of interweaving fascicles of spindle cells with scattered atypical nuclei. Immunohistochemical studies showed that tumor cells were focally positive for S100, and negative for SMA, desmin, calretinin, Pan CK, CK5/6, EMA, CD99, CD34 and p53. The overall morphological and immunohistochemical features were diagnostic of an atypical neurofibroma. [N A J Med Sci. 2009;2(4):135-138.]
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57岁男性右胸腔巨大非典型神经纤维瘤1例并文献复习
胸内神经源性肿瘤并不罕见,但在胸腔内表现为巨大肿块是罕见的。虽然有几例胸内巨大恶性周围神经鞘瘤的报道,但文献中只报道了一例胸内巨大良性神经纤维瘤。在此报告中,我们描述了一例非常罕见的右胸腔非典型巨大神经纤维瘤。患者是一名57岁的非裔美国人,他突发心脏骤停,在急诊室去世。尸检发现右胸腔内有一巨大的包裹性硬瘤,附着于椎体,浅表贴附于右肺上叶和中叶。这个庞然大物重达2140克,尺寸为31 x 30 x 5.5厘米。镜下,肿瘤由纺锤形细胞交织成束状,分散有非典型细胞核。免疫组化研究显示肿瘤细胞局灶性S100阳性,SMA、desmin、calretinin、Pan CK、CK5/6、EMA、CD99、CD34和p53阴性。整体形态学和免疫组织化学特征是非典型神经纤维瘤的诊断。[J] .中华医学杂志,2009;2(4):135-138。
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