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肺良性转移性平滑肌瘤表现为急性呼吸窘迫。

Pulmonary benign metastasizing leiomyoma presented as acute respiratory distress.

作者信息

Pastré Jean, Juvin Karine, Grand Bertrand, Gibault Laure, Valcke Judith, Israël-Biet Dominique

机构信息

Université Paris Descartes Sorbonne Paris Cité and AP-HP Paris France; Service de Pneumologie Hôpital Européen Georges Pompidou Paris France.

Service de Pneumologie Hôpital Européen Georges Pompidou Paris France.

出版信息

Respirol Case Rep. 2017 Jan 19;5(2):e00216. doi: 10.1002/rcr2.216. eCollection 2017 Mar.

Abstract

Benign metastasizing leiomyoma (BML) is a very rare condition and is characterized by the presence of benign smooth muscle tumours in organs distant from the uterus, most commonly the lung. It generally affects women of reproductive age and prognostic is usually excellent. However, the course of the disease is unpredictable. We report here the case of a 76-year-old woman with a previous medical history of uterine benign leiomyomas in whom BML was acutely revealed by a respiratory distress due to voluminous pulmonary and pleural leiomyomas requiring surgical extraction. Clinical evolution was remarkable by resistance to medical treatment and development of rare bone localization. BML is a contradictory entity characterized by benign histological features but with metastatic potential. Pulmonologists as well as oncologists in charge of patients with multiple pulmonary nodules and a history of uterine leiomyoma should be aware of this potential diagnosis in order to implement appropriate diagnostic procedures for this benign tumour.

摘要

良性转移性平滑肌瘤(BML)是一种非常罕见的病症,其特征是在远离子宫的器官中存在良性平滑肌肿瘤,最常见于肺部。它通常影响育龄女性,预后通常良好。然而,疾病进程不可预测。我们在此报告一例76岁女性病例,该患者既往有子宫良性平滑肌瘤病史,因大量肺和胸膜平滑肌瘤导致呼吸窘迫而急性发现BML,并需要手术切除。临床病程表现为对药物治疗耐药且出现罕见的骨转移,十分显著。BML是一个矛盾的实体,具有良性组织学特征但有转移潜能。负责患有多个肺结节且有子宫平滑肌瘤病史患者的肺科医生和肿瘤学家应意识到这种潜在诊断,以便对这种良性肿瘤实施适当的诊断程序。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a27b/5244452/21ed0fcc3918/RCR2-5-0-g001.jpg

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