Why the study?
Primary pulmonary artery sarcoma is a rare disease with nonspecific clinical manifestations that require better characterization of its clinical features, diagnostic points, and treatment strategies.
Case Report (n=1)
No
This case report demonstrates that multimodal imaging and genetic testing can guide surgical resection and targeted adjuvant therapy (palbociclib, immunotherapy, and radiotherapy) to achieve tumor reduction and survival exceeding 14 months in a patient with primary pulmonary artery sarcoma initially misdiagnosed as pulmonary embolism.
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May support multimodal therapy in primary pulmonary artery sarcoma; leaves open need for prospective validation.
Objective This study aimed to explore the clinical features, diagnostic points, and treatment strategies of primary pulmonary artery sarcoma (PPAS) and improve the understanding of this rare disease. Methods A retrospective analysis of the clinical data of a PPAS patient admitted to our hospital in December 2021 was conducted. The clinical manifestations, imaging features, pathological diagnosis, and treatment process were systematically summarized, followed by a discussion based on the literature. Results The patient was a 39-year-old female who presented with dry cough and fatigue, and gradually developed hemoptysis, chest pain, and difficulty breathing. Multimodal imaging examination revealed a mass in the upper lobe of the left lung with extensive pulmonary embolism, and PET/CT suggested increased glucose metabolism at the site of pulmonary embolism. The postoperative pathological diagnosis was polymorphic undifferentiated sarcoma, and immunohistochemistry exhibited high expression of Ki-67 (60%). Genetic testing revealed TP53 mutations and increased CDK4 copy number. The patient underwent surgical resection combined with targeted therapy and radiotherapy. Follow-up indicated tumor reduction, symptom relief, and survival exceeding 14 months. Conclusion The clinical manifestations of PPAS lack specificity and are easily misdiagnosed as pulmonary thromboembolism. Multimodal imaging evaluation, timely histopathological examination, and individualized comprehensive treatment are crucial for improving prognosis.
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Chen et al. (2026) conducted a case report in Primary pulmonary artery sarcoma (polymorphic undifferentiated sarcoma) (n=1). Surgical resection combined with palbociclib, camrelizumab, helical tomotherapy (TOMO) radiotherapy, and warfarin was evaluated on Tumor response (RECIST 1.1) and survival.
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