Surgical Management of Primary Pulmonary Artery Sarcoma

Abstract Background: Pulmonary artery intimal sarcoma(PAS) is a very rare disease, the prevalence of it is about 0.001%-0.003%1. PAS is often misdiagnosed as acute or chronic pulmonary thromboembolism due to its clinical presentation and radiological findings. Thus early diagnosis is very crucial and may improve patient outcome.Case presentation: Here we report a case in a Chinese male which the symptom as presentation was episodes of shortness of breath. Transthoracic echocardiography showed a solid mass in the pulmonary valve orifice, which was demonstrated to be a pulmonary artery intimal sarcoma diagnosed by histopathology2. In this case the initial differential diagnosis included pulmonary embolism. Because the initial symptom of primary pulmonary artery sarcoma is extremely similiar to the pulmonary embolism. Half of them may be misdiagnosed as pulmonary embolism. Imaging studies are very helpful. Ultrasound and CT are the best due to their resolution and ability to assess the relationship of the mass with the surrounding structures. The final diagnosis is mostly made after surgical excision and this is the most effective treatment. At the same time, radiotherapy and chemotherapy after surgery is also a adjuvant treatment3.Conclusion: We report a very rare case of pulmonary artery intimal sarcoma, due to late diagnosis and delayed treatment in this case, the patient display a poor prognostic. Early diagnosis and right treatment can improve the prognosis of PAS and optimize overall health.

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Primary pulmonary artery sarcoma with deep vein thrombosis

Medicine ◽

10.1097/md.0000000000015874 ◽

2019 ◽

Vol 98 (23) ◽

pp. e15874 ◽

Cited By ~ 1

Author(s):

Xin Li ◽

Quan Qi ◽

Fuxiang Liang ◽

Xuxia Zhang ◽

Shuai Dong ◽

...

Keyword(s):

Deep Vein Thrombosis ◽

Pulmonary Artery ◽

Pulmonary Artery Sarcoma ◽

Vein Thrombosis ◽

Primary Pulmonary Artery Sarcoma ◽

Deep Vein

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A Case of Primary Pulmonary Artery Sarcoma Mimicking Pulmonary Embolism: Role of PET/CT for Differential Diagnosis

Tuberculosis and Respiratory Diseases ◽

10.4046/trd.2007.62.3.232 ◽

2007 ◽

Vol 62 (3) ◽

pp. 232

Author(s):

Sang-Soo Lim ◽

Koo-Hyun Hong ◽

Jae-Min Shin ◽

Youn Seup Kim ◽

Young Koo Jee ◽

...

Keyword(s):

Pulmonary Embolism ◽

Differential Diagnosis ◽

Pulmonary Artery ◽

Pulmonary Artery Sarcoma ◽

Pet Ct ◽

Primary Pulmonary Artery Sarcoma

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Surgical treatment of primary pulmonary artery sarcoma

General Thoracic and Cardiovascular Surgery ◽

10.1007/s11748-020-01476-2 ◽

2020 ◽

Author(s):

Yongxin Han ◽

Yanan Zhen ◽

Xiaopeng Liu ◽

Xia Zheng ◽

Jianbin Zhang ◽

...

Keyword(s):

Pulmonary Artery ◽

Pulmonary Valve ◽

Circulatory Arrest ◽

Tumor Resection ◽

Preoperative Imaging ◽

Survival Duration ◽

Current Standard ◽

Pulmonary Artery Sarcoma ◽

Primary Pulmonary Artery Sarcoma

Abstract Objective Primary pulmonary artery sarcoma (PAS) is a rare tumor that originates from the intimal layer of the pulmonary artery or pulmonary valve and has a poor prognosis. The standard treatment for this devastating disease remains unclear. This study aimed to summarize the current standard treatments for PAS. Methods From September 2015 to January 2020, six patients were diagnosed with PAS and underwent pulmonary endarterectomy (PEA) at our department. Their medical records were retrospectively reviewed to analyze the clinical characteristics, histopathological features, and postoperative outcomes. Fourteen articles, each reporting at least 6 cases, identified 201 patients diagnosed with PAS, and 158 patients had detailed treatments and follow-up data. Results All of the patients who successfully underwent PEA were alive at follow-up, with a mean survival duration of 11.6 months (7–28 months), and one patient developed recurrence in the right upper lobe lung. Two patients received postoperative chemotherapy. In one patient, the tumor invaded the pulmonary valve. Conclusions PAS resection combined with PEA via the aid of cardiopulmonary bypass and deep hypothermic circulatory arrest could achieve maximal tumor resection in patients without metastatic lesions. An individualized surgery strategy relies on a precise preoperative imaging examination. Moreover, postoperative adjuvant therapy could yield improved survival outcomes.

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