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通过电视辅助胸腔镜手术诊断的合并结节性硬化症的肺淋巴管平滑肌瘤病

Pulmonary lymphangioleiomyomatosis with concomitant tuberous sclerosis complex diagnosed by video-assisted thoracoscopic surgery.

作者信息

Taniguchi Yuji, Haruki Tomohiro, Fujioka Shinji, Adachi Yoshin, Miwa Ken, Nakamura Hiroshige

机构信息

Operating Room Division, Tottori University Hospital, 36-1 Nishi-cho, Yonago, Tottori 683-8504, Japan.

出版信息

Gen Thorac Cardiovasc Surg. 2008 Feb;56(2):81-4. doi: 10.1007/s11748-007-0194-7. Epub 2008 Feb 24.

DOI:10.1007/s11748-007-0194-7
PMID:18297464
Abstract

We herein describe the case of a 20-year-old woman who was diagnosed as having tuberous sclerosis complex (TSC) at the age of 10 years. The patient had a history of right pneumothorax at the age of 19. This time, a right pneumothorax recurrence was detected, and video-assisted thoracoscopic surgery (VATS) was performed. In the intraoperative findings, an infinitesimal lung cyst was detected on the lung lobe, and partial resection was performed. Pathologically, antibody-positive smooth muscle cells of the human melanoma block (HMB)-45 had grown and been diagnosed as pulmonary lymphangioleiomyomatosis (LAM). About half a year later, left pneumothorax occurred, and VATS was performed again. Pathologically, antibody-positive smooth muscle cells of HMB-45 were not detected. Occasionally, TSC is known to cause LAM complications, but in some cases it is difficult to make an accurate pathological diagnosis. Making a pathological diagnosis based on the VATS findings and taking a sufficient amount of tissue specimen are considered useful for making the diagnosis.

摘要

我们在此描述一名20岁女性的病例,该患者10岁时被诊断为结节性硬化症(TSC)。患者19岁时有右气胸病史。此次,检测到右气胸复发,并进行了电视辅助胸腔镜手术(VATS)。术中发现,肺叶上有一个极小的肺囊肿,并进行了部分切除。病理检查显示,人黑色素瘤阻滞(HMB)-45抗体阳性的平滑肌细胞增生,被诊断为肺淋巴管平滑肌瘤病(LAM)。大约半年后,发生了左气胸,并再次进行了VATS。病理检查未检测到HMB-45抗体阳性的平滑肌细胞。众所周知,TSC偶尔会引起LAM并发症,但在某些情况下,很难做出准确的病理诊断。基于VATS检查结果进行病理诊断并采集足够的组织标本被认为有助于诊断。

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An early case of pulmonary lymphangioleiomyomatosis diagnosed by video-assisted thoracoscopic surgery.一例通过电视辅助胸腔镜手术诊断的肺淋巴管平滑肌瘤病早期病例。
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