METHODS: We present the case of a 33-year-old woman with no significant past medical history who presented with abdominal pain and was incidentally found to have a right hilar mass with pleural effusion and empyema. After undergoing surgery for a perforated gastric ulcer, her pulmonary lesions were further worked up. Despite an extensive diagnostic evaluation, including imaging, bronchoscopy, and thoracotomy, establishing a diagnosis was challenging. Ultimately, PPA was diagnosed on surgical lung biopsy, and the patient was started on pazopanib and paclitaxel chemotherapy but expired after 1 month due to multiple complications.
CONCLUSIONS: This case highlights the difficulty in diagnosing this rare tumor and its poor prognosis regardless of therapy. Greater awareness of PPA and more research are needed to improve early detection and treatment options for this deadly disease.
方法:我们介绍一例33岁女性,无明显既往病史,表现为腹痛,偶然发现右侧肺门肿块伴胸腔积液和脓胸。胃溃疡穿孔手术后,她的肺部病变被进一步处理。尽管进行了广泛的诊断评估,包括成像,支气管镜检查,开胸手术,建立诊断是具有挑战性的。最终,在外科肺活检中诊断出PPA,患者开始接受帕唑帕尼和紫杉醇化疗,但由于多种并发症,1个月后过期。
结论:该病例突出了诊断这种罕见肿瘤的困难和无论如何治疗的不良预后。需要提高对PPA的认识和更多的研究,以改善这种致命疾病的早期发现和治疗选择。