PDF(Pubmed)
Abstract：
BACKGROUND: Primary pulmonary angiosarcoma (PPA) is a highly aggressive and rare malignancy originating from the endothelial cells of blood vessels in the lungs. PPA is an extremely rare subtype, with less than 30 cases reported to date. PPA is not only challenging to diagnose but also has a poor prognosis, often resulting in a high mortality rate within a year of diagnosis, regardless of the treatment approach.
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.
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.
摘要：
背景：原发性肺血管肉瘤（PPA）是一种高度侵袭性和罕见的恶性肿瘤，起源于肺部血管的内皮细胞。PPA是一种极为罕见的亚型,迄今报告的病例不到30例。PPA不仅诊断具有挑战性，而且预后不良。通常导致诊断后一年内的高死亡率，不管治疗方法如何。
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。
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。
