[Angiomatoid fibrous histiocytoma in the pulmonary artery: case report and review of literature].

Objective

To explore the clinical features, diagnosis, and treatment progress of angiomatoid fibrous histiocytoma (AFH) primary to the pulmonary artery.

Methods

A retrospective analysis was conducted on one case of AFH primary to the pulmonary artery in a middle-aged female. Databases including SinoMed, the Wanfang Data Knowledge Service Platform, and the China National Knowledge Infrastructure (CNKI) were searched using the keywords (angiomatoid fibrous histiocytoma) and (pulmonary artery). Meanwhile, PubMed, Embase, the Cochrane Library, and Web of Science were searched using the keywords "angiomatoid fibrous histiocytoma" and "pulmonary artery." The search was updated to April 2025.

Results

The patient was admitted to our hospital in February 2025 due to symptoms such as chest tightness and dizziness, and a space-occupying lesion of the pulmonary artery was detected. Postoperative histopathological examination confirmed the diagnosis of pulmonary artery AFH. Only one relevant article was retrieved from domestic databases and five from foreign databases. A total of seven patients were included (including the current case), with four males and three females, aged 20-76 years (mean, 54.57±18.18 years). The lesions were located in the left main pulmonary artery (three cases), right main pulmonary artery (two cases), right upper pulmonary artery (three cases), and right interlobar pulmonary artery (one case). D-dimer levels were elevated in two cases, normal in three cases, and unknown in two cases. PET-CT showed significantly increased SUVmax in four cases and was unknown in three cases. EWSR1 gene translocation was detected in five cases, absent in one case, and unknown in one case. Surgical procedures included extended local resection of the pulmonary artery (three cases) and partial or total pneumonectomy (four cases). No recurrence or metastasis was observed in five patients, and the outcomes of two patients were unknown.

Conclusion

AFH primary to the pulmonary artery is extremely rare. Clinically, it needs to be mainly differentiated from pulmonary thromboembolism and pulmonary artery intimal sarcoma, and clinicians in respiratory medicine and vascular intervention departments should raise awareness of this disease. In the differential diagnosis from pulmonary thromboembolism, clinical manifestations and laboratory tests (e.g., D-dimer) lack specificity, while imaging examinations such as CTPA and PET-CT are effective auxiliary methods. Definitive diagnosis still relies on histopathological examination, and EWSR1 molecular detection is an important basis for pathological confirmation. Pulmonary artery resection combined with artificial vascular replacement is an effective treatment, and long-term follow-up is necessary.