Key result
Rare primary bronchopulmonary epithelioid leiomyosarcoma progresses to fatal respiratory failure two months after diagnosis.
Why the study?
Bronchopulmonary epithelioid leiomyosarcoma is a rare malignant neoplasm whose distinctive epithelioid cell morphology often leads to misinterpretation with epithelial-derived tumors, posing substantial clinical diagnostic challenges.
Case Report (n=1)
No
This case report highlights the diagnostic challenges of primary bronchopulmonary epithelioid leiomyosarcoma, emphasizing the critical role of immunohistochemistry in differentiating it from epithelial-derived tumors.
Bronchopulmonary epithelioid leiomyosarcoma (BP-ELS) represents a rare malignant neoplasm arising from bronchial smooth muscle. Its distinctive epithelioid cell morphology often leads to potential misinterpretation with epithelial-derived tumors, thereby posing substantial challenges for clinical diagnosis. This report describes a 68-year-old Han Chinese male with a 2-year history of exertional dyspnea and palpitations. Chest computed tomography (CT) revealed a neoplastic lesion causing luminal obstruction of the right main bronchus. Primary bronchopulmonary epithelioid leiomyosarcoma was confirmed via bronchoscopic biopsy and immunohistochemical analysis. By integrating imaging characteristics, pathological findings, and a comprehensive literature review, this article provides a detailed analysis of the clinical features and proposes evidence-based diagnostic and therapeutic strategies for this rare entity. Primary bronchopulmonary epithelioid leiomyosarcoma (BP-ELS) typically presents with nonspecific clinical and radiological features, and histopathological examination combined with immunohistochemistry remains the cornerstone of definitive diagnosis. Curative surgical resection is the primary treatment modality, while neoadjuvant chemotherapy has been reported to improve resectability in patients with locally advanced disease. Early identification and complete surgical resection are critical determinants for optimizing patient prognosis in this rare oncological entity.
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Sun et al. (2026) conducted a case report in Primary bronchopulmonary epithelioid leiomyosarcoma (n=1). Bronchoscopic biopsy and immunohistochemical analysis was evaluated. A 68-year-old male was diagnosed with primary bronchopulmonary epithelioid leiomyosarcoma, a rare malignant neoplasm, and succumbed to respiratory failure two months after diagnosis.
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