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以胸壁转移为首发表现的无症状性肺动脉内膜肉瘤:一例尸检病例

Asymptomatic Pulmonary Artery Intimal Sarcoma with Chest Wall Metastasis as an Initial Manifestation: An Autopsy Case.

作者信息

Kakimoto Tomoo, Sasaki Mamoru, Morinaga Shojiroh, Nakayama Robert, Minematsu Naoto

机构信息

Department of Medicine, Hino Municipal Hospital, 4-3-1 Tamadaira, Hino-shi, Tokyo, Japan.

Department of Diagnostic Pathology, Hino Municipal Hospital, 4-3-1 Tamadaira, Hino-shi, Tokyo, Japan.

出版信息

Case Rep Med. 2018 May 27;2018:6153658. doi: 10.1155/2018/6153658. eCollection 2018.

Abstract

Pulmonary artery intimal sarcoma (PAIS) is a rare mesenchymal malignancy arising in the pulmonary trunk or proximal pulmonary artery and shows intraluminal growth. Clinical manifestations in PAIS are predominantly related to the pulmonary artery embolism, so cases with initial symptoms related to an extrapulmonary metastasis are unusual. The present report describes an 82-year-old man without any cardiopulmonary symptoms who was detected with an abnormal shadow on chest radiography during a routine health checkup. Contrast medium-enhanced chest computed tomography revealed an enhancing mass in the right pulmonary artery, pulmonary nodules, and a chest wall tumor corresponding to the abnormal shadow observed using chest radiography. A core needle biopsy for the chest wall tumor determined a pathological diagnosis of unclassified sarcoma. The patient was diagnosed with PAIS on the basis of clinical, radiological, and pathological correlations. He was scheduled to receive supportive care, but died of respiratory failure 1 year from the first visit. An autopsy revealed the pleomorphic sarcoma occupying the entire lumen of the right pulmonary artery with the only site of extrapulmonary metastasis in the chest wall. We should be aware of rare cases of asymptomatic PAIS found through routine health checkups.

摘要

肺动脉内膜肉瘤(PAIS)是一种罕见的间叶性恶性肿瘤,起源于肺动脉干或近端肺动脉,呈腔内生长。PAIS的临床表现主要与肺动脉栓塞有关,因此首发症状与肺外转移相关的病例并不常见。本报告描述了一名82岁男性,在常规健康检查中胸部X线检查发现异常阴影,但无任何心肺症状。胸部增强计算机断层扫描显示右肺动脉有强化肿块、肺结节以及与胸部X线检查发现的异常阴影相对应的胸壁肿瘤。对胸壁肿瘤进行粗针活检确定为未分类肉瘤的病理诊断。根据临床、影像学和病理相关性,该患者被诊断为PAIS。他原计划接受支持性治疗,但自首次就诊起1年后死于呼吸衰竭。尸检显示多形性肉瘤占据右肺动脉整个管腔,胸壁是唯一的肺外转移部位。我们应该注意通过常规健康检查发现的无症状PAIS罕见病例。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b0ac/5994268/1c1dd55606eb/CRIM2018-6153658.001.jpg

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