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原发性肺软骨肉瘤:4 例临床病理研究。

Primary pulmonary chondrosarcomas: a clinicopathologic study of 4 cases.

机构信息

Departments of Pathology at the University of Texas, M D Anderson Cancer Center, Houston, TX 77030, USA.

出版信息

Hum Pathol. 2011 Nov;42(11):1629-34. doi: 10.1016/j.humpath.2011.01.009. Epub 2011 Apr 14.

Abstract

Malignant cartilaginous tumors of the lung are unusual, and although their occurrence has been reported in the literature in some cases, their separation from other benign cartilaginous tumors of the lung can be very difficult. Four cases of primary chondrosarcomas of the lung are presented. The patients are 2 men and 2 women between the ages of 51 and 69 years. Clinically, the most common symptoms were chest pain, dyspnea, and cough. Two tumors were centrally located, whereas 2 tumors were peripheral. Complete surgical resection was accomplished in all the patients. Histologically, 2 tumors were low grade of the hyaline type, whereas 2 tumors were predominantly myxoid chondrosarcomas. In the 2 myxoid chondrosarcomas immunohistochemical studies for keratin, desmin, smooth muscle actin, and CD31 were negative, whereas S-100 protein shows focal positive staining in both cases. Follow-up showed that one patient with low-grade tumor was alive and well at 36 months, whereas one patient with myxoid chondrosarcoma died 45 days after diagnosis because of surgical complications. Two additional patients were lost to follow-up. Our study highlights the ubiquitous distribution of chondrosarcomas and the histopathologic spectrum that these tumors may show when occurring in the lung.

摘要

肺部恶性软骨性肿瘤较为罕见,尽管已有文献报道过一些病例,但仍很难将其与肺部其他良性软骨性肿瘤区分开来。本文报道了 4 例原发性肺软骨肉瘤患者。患者为 2 男 2 女,年龄 51~69 岁。临床上,最常见的症状为胸痛、呼吸困难和咳嗽。2 个肿瘤位于中央,2 个肿瘤位于外周。所有患者均完成了完全手术切除。组织学上,2 个肿瘤为透明细胞型低度恶性,2 个肿瘤以黏液样为主。在 2 个黏液样软骨肉瘤中,免疫组织化学研究显示角蛋白、结蛋白、平滑肌肌动蛋白和 CD31 均为阴性,而 S-100 蛋白在两种情况下均呈局灶阳性染色。随访显示,1 例低度恶性肿瘤患者在 36 个月时存活且状况良好,而 1 例黏液样软骨肉瘤患者在诊断后 45 天因手术并发症死亡。另外 2 例患者失访。本研究强调了软骨肉瘤的广泛分布以及这些肿瘤在肺部发生时可能表现出的组织病理学谱。

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