Pulmonary artery intimal sarcoma: a case report and literature review

Chang, Ding-Yu; Lin, Kung‐Hung; Pan, Jun-Yen; Liu, Hung-Wei; Kuo, Shu-Hung; Lei, Lin

doi:10.1002/rcr2.530

Cited by 15 publications

(19 citation statements)

References 17 publications

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“…It was first reported by doctor of Mandelstamml in autopsy. The diseases can occur in different age periods, the youngest is 2 months, the oldest is 89 years old, and the average age is about 45–54 years [ 2 ].…”

Section: Discussionmentioning

confidence: 99%

A case report of primary pulmonary artery intimal sarcoma

Ruan

2021

Eur J Med Res

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Background Pulmonary artery intimal sarcoma (PAS) is a very rare disease, its prevalence is about 0.001–0.003%. 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 where the symptom 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 histopathology. In this case, the initial differential diagnosis included pulmonary embolism. Because the initial symptom of primary pulmonary artery sarcoma is extremely similar 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 an adjuvant treatment. Conclusion We report a very rare case of pulmonary artery intimal sarcoma. Due to late diagnosis and delayed treatment in this case, the patient displayed a poor prognostic. Early diagnosis and right treatment can improve the prognosis of PAS and optimize overall health.

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Section: Discussionmentioning

confidence: 99%

A case report of primary pulmonary artery intimal sarcoma

Ruan

2021

Eur J Med Res

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“…It was rst reported by doctor of Mandelstamml in autopsy. The diseases can occur in different age periods, the youngest is 2 months, the oldest is 89 years old, the average age is about 45 to 54 years old 5 .…”

Section: Discussionmentioning

confidence: 99%

A Case Report of Primary Pulmonary Artery Intimal Sarcoma

Ruan

2021

Preprint

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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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“…Chemotherapeutic agents include adriamycin plus isosfamide, gemcitabine plus taxane, and dacarbazine. Recent studies show survival benefit when multimodality treatment is applied with adjuvant or neoadjuvant chemotherapy and/or radiotherapy compared to monotherapy such as surgery alone [14,15].…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Artery Sarcoma Diagnosed by Transbronchial Endosonographic (EBUS-TBNA) Approach

Frangia-Tsivou¹,

Alsaid²,

Karetsi³

et al. 2020

SCR

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Background: Pulmonary Artery Sarcomas are the rarest and the most aggressive vascular tumors. Their accurate diagnosis is challenging due to the lack of specific symptoms and clinical manifestations and they are often initially misdiagnosed and managed as chronic pulmonary thromboembolic disease until an alternate diagnosis is suspected based on the lack of response to anticoagulants. Proper management is thus often delayed until histological confirmation. Moreover, the lack of consensus regarding their diagnosis and treatment further contributes to their reported high mortality [1]. Case Presentation: We present the case of a 34-year-old male, nonsmoker, who presented with hemoptysis, fatigue and dyspnea on exertion 3 months prior to admission. His chest x-ray showed enlargement of the left hilum and chest CT revealed a large intraluminal low attenuation filling defect at the level of the left PA. Chest MRI confirmed the presence of a large endovascular tumor of the left PA. Before referring the patient for surgical biopsy, he underwent bronchoscopy during which the vascular tumor was successfully located, observed and sampled by endobronchial ultrasound (EBUS) transbronchial needle aspiration (TBNA). Tissue biopsy through EBUS-TBNA revealed an intimal sarcoma of the PA. The patient was subsequently referred for surgical excision with curative intent. Conclusion: Bronchoscopy and EBUS-TBNA may safely and accurately diagnose even rare pathologies of the mediastinal vasculature adjacent to the airways through transbronchial approach.

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Pulmonary artery intimal sarcoma: a case report and literature review

Cited by 15 publications

References 17 publications

A case report of primary pulmonary artery intimal sarcoma

A case report of primary pulmonary artery intimal sarcoma

A Case Report of Primary Pulmonary Artery Intimal Sarcoma

Pulmonary Artery Sarcoma Diagnosed by Transbronchial Endosonographic (EBUS-TBNA) Approach

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