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原发性支气管平滑肌肉瘤:一项诊断挑战。

Primary bronchial leiomyosarcoma: a diagnostic challenge.

作者信息

Chen Dan, Luo Shuai

机构信息

Department of Pathology, Beijing Jishuitan Hospital Guizhou Hospital, Guiyang City, China.

Department of Pathology, Affiliated Hospital of Zunyi Medical University, Zunyi City, Guizhou Province, P.R. China.

出版信息

BMC Pulm Med. 2025 Jan 8;25(1):8. doi: 10.1186/s12890-024-03472-1.

DOI:10.1186/s12890-024-03472-1
PMID:39780087
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11707924/
Abstract

BACKGROUND

Pulmonary bronchial leiomyosarcoma is an extremely rare malignant tumour of the lung originating from the mesenchymal tissue. The retroperitoneal region is the most common site of leiomyosarcoma. It exhibits a high degree of malignancy and a poor prognosis, thereby highlighting the significance of early diagnosis of this disease.

CASE DEMONSTRATION

A 61-year-old male with a smoking history of 40 + years was admitted to the hospital due to cough, expectoration, and shortness of breath that extended for half a year, which aggravated for two months and was succeeded by intermittent bloody sputum for one month. Chest computed tomography (CT) revealed a mass in the upper lobe of the right lung, which was identified as a malignant tumour lesion involving the upper thoracic oesophagus. The boundaries of the mass with the trachea, brachiocephalic artery, and right brachiocephalic vein were unclear. Brush liquid-based cytology of the right middle lobe bronchus was performed, followed by histopathological biopsy. The pathological diagnosis was leiomyosarcoma in the middle lobe of the right lung. After the diagnosis, he underwent chemotherapy in another hospital, and the case was followed up for six months, during which no recurrence was noted.

CONCLUSIONS

Pulmonary and bronchial leiomyosarcomas often occur in young males with rapid progression and poor prognosis, but metastasis is rare. Surgical resection is the first-line treatment. We report a case of pulmonary bronchial leiomyosarcoma. The cytological, histological, immunohistochemical, and differential diagnoses were analysed to improve the understanding of the disease. Presently, there exist some challenges in diagnosing and treating pulmonary bronchial leiomyosarcoma. Early detection and treatment are important to improve prognosis.

摘要

背景

肺支气管平滑肌肉瘤是一种极其罕见的起源于间叶组织的肺部恶性肿瘤。腹膜后区域是平滑肌肉瘤最常见的发病部位。它具有高度恶性和较差的预后,因此凸显了早期诊断该病的重要性。

病例展示

一名有40多年吸烟史的61岁男性因咳嗽、咳痰、气短半年入院,症状持续加重两个月,随后出现间断咯血一个月。胸部计算机断层扫描(CT)显示右肺上叶有一个肿块,经确诊为累及胸段上段食管的恶性肿瘤病变。肿块与气管、头臂干动脉及右头臂静脉的边界不清。对右中叶支气管进行了刷检液基细胞学检查,随后进行了组织病理学活检。病理诊断为右肺中叶平滑肌肉瘤。确诊后,他在另一家医院接受了化疗,并对该病例进行了6个月的随访,期间未发现复发。

结论

肺和支气管平滑肌肉瘤常发生于年轻男性,进展迅速且预后较差,但转移罕见。手术切除是一线治疗方法。我们报告了一例肺支气管平滑肌肉瘤病例。对其细胞学、组织学、免疫组化及鉴别诊断进行了分析,以增进对该疾病的认识。目前,肺支气管平滑肌肉瘤的诊断和治疗存在一些挑战。早期发现和治疗对改善预后很重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa9f/11707924/05d88cae7b23/12890_2024_3472_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa9f/11707924/9eded913fa67/12890_2024_3472_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa9f/11707924/3b32ad857c4f/12890_2024_3472_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa9f/11707924/4f1179167b4d/12890_2024_3472_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa9f/11707924/05d88cae7b23/12890_2024_3472_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa9f/11707924/9eded913fa67/12890_2024_3472_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa9f/11707924/3b32ad857c4f/12890_2024_3472_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa9f/11707924/4f1179167b4d/12890_2024_3472_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa9f/11707924/05d88cae7b23/12890_2024_3472_Fig4_HTML.jpg

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