Pulmonary Artery Sarcoma Masquerading as Chronic Pulmonary Thromboembolism

We describe the case of a 60-year-old woman who presented with pulmonary artery sarcoma, a very rare tumor of the cardiovascular system. Her tumor was initially misdiagnosed as chronic pulmonary thromboembolism, and she underwent pulmonary endarterectomy. Early diagnosis of primary pulmonary artery sarcoma is crucial. That alternative should always be considered before settling on a diagnosis of pulmonary embolism. Suspicion should be aroused by the failure of anticoagulant treatment to alleviate pulmonary perfusion abnormalities and systemic symptoms. Surgical resection of the tumor—preferably by pulmonary endarterectomy, followed by reconstruction as needed—is currently the most promising treatment for pulmonary artery sarcoma.

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Primary pulmonary artery sarcoma masquerading as pulmonary thromboembolism: a rare diagnosis unveiled

Clinical Sarcoma Research ◽

10.1186/s13569-017-0080-8 ◽

2017 ◽

Vol 7 (1) ◽

Cited By ~ 3

Author(s):

Abhishek Mahajan ◽

Bharat Rekhi ◽

Siddhartha Laskar ◽

Jyoti Bajpai ◽

Lekshmy Jayasree ◽

...

Keyword(s):

Pulmonary Artery ◽

Pulmonary Thromboembolism ◽

Pulmonary Artery Sarcoma ◽

Primary Pulmonary Artery Sarcoma ◽

Rare Diagnosis

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Primary Pulmonary Artery Sarcoma: Difficult Differential Diagnosis from Chronic Pulmonary Thromboembolism

Annals of Vascular Surgery ◽

10.1016/j.avsg.2006.08.007 ◽

2007 ◽

Vol 21 (4) ◽

pp. 505-507 ◽

Cited By ~ 3

Author(s):

Yuka Kondo ◽

Akihito Muto ◽

Toshiya Nishibe ◽

Masato Sato ◽

Motomi Ando

Keyword(s):

Differential Diagnosis ◽

Pulmonary Artery ◽

Pulmonary Thromboembolism ◽

Pulmonary Artery Sarcoma ◽

Primary Pulmonary Artery Sarcoma

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Primary pulmonary artery sarcoma versus pulmonary thromboembolism: a multimodal imaging comparison

Journal of Thrombosis and Thrombolysis ◽

10.1007/s11239-021-02464-w ◽

2021 ◽

Author(s):

Ellen Kronzer ◽

Steven I. Robinson ◽

Douglas A. Collins ◽

Robert D. McBane

Keyword(s):

Pulmonary Artery ◽

Multimodal Imaging ◽

Pulmonary Thromboembolism ◽

Pulmonary Artery Sarcoma ◽

Primary Pulmonary Artery Sarcoma

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Surgical treatment of pulmonary artery sarcoma: a report of 17 cases

Pulmonary Circulation ◽

10.1177/2045894020986394 ◽

2021 ◽

Vol 11 (1) ◽

pp. 204589402098639

Author(s):

Wu Song ◽

Long Deng ◽

Jiade Zhu ◽

Shanshan Zheng ◽

Haiping Wang ◽

...

Keyword(s):

Pulmonary Artery ◽

Survival Rates ◽

Tumor Resection ◽

Long Term Results ◽

Pulmonary Endarterectomy ◽

Pulmonary Artery Sarcoma ◽

The Mean ◽

Operative Findings

Pulmonary artery sarcoma (PAS) is a rare and devastating disease. The diagnosis is often delayed, and optimal treatment remains unclear. The aim of this study is to report our experience in the surgical management of this disease. Between 2000 and 2018, 17 patients underwent operations for PAS at our center. The medical records were retrospectively reviewed to evaluate the clinical characteristics, operative findings, the postoperative outcomes, and the long-term results. The mean age at operation was 46.0 ± 12.4 years (range, 26–79 years), and eight (47.1%) patients were male. Six patients underwent tumor resection alone, whereas the other 11 patients received pulmonary endarterectomy (PEA). There were two perioperative deaths. Follow-up was completed for all patients with a mean duration of 23.5 ± 17.6 months (1–52 months). For all 17 patients, the median postoperative survival was 36 months, and estimated cumulative survival rates at 1, 2, 3, and 4 years were 60.0%, 51.4%, 42.9%, and 21.4%, respectively. The mean survival was 37.0 months after PEA and 14.6 months after tumor resection only ( p = 0.046). Patients who had no pulmonary hypertension (PH) postoperatively were associated with improved median survival (48 vs. 5 months, p = 0.023). In conclusion, PAS is often mistaken for chronic pulmonary thromboembolism. The prognosis of this very infrequent disease remains poor. Early detection is essential for prompt and best surgical approach, superior to tumor resection alone, and PEA surgery with PH relieved can provide better chance of survival.

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Primary pulmonary artery sarcoma: diagnosis with CT, MR imaging, and transthoracic needle biopsy.

American Journal of Roentgenology ◽

10.2214/ajr.159.4.1529830 ◽

1992 ◽

Vol 159 (4) ◽

pp. 702-704 ◽

Cited By ~ 30

Author(s):

E L Bressler ◽

J M Nelson

Keyword(s):

Mr Imaging ◽

Pulmonary Artery ◽

Needle Biopsy ◽

Pulmonary Artery Sarcoma ◽

Transthoracic Needle Biopsy ◽

Primary Pulmonary Artery Sarcoma

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A Case Report of Primary Pulmonary Artery Intimal Sarcoma

10.21203/rs.3.rs-673296/v1 ◽

2021 ◽

Author(s):

Xiaofang Bai ◽

Litao Ruan

Keyword(s):

Pulmonary Embolism ◽

Pulmonary Artery ◽

Surgical Excision ◽

Final Diagnosis ◽

Initial Symptom ◽

Pulmonary Artery Sarcoma ◽

Intimal Sarcoma ◽

Delayed Treatment ◽

Primary Pulmonary Artery Sarcoma ◽

Relationship Of

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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