Lung Cancer: Lung Cancer I |

Clinical Features of Pulmonary Artery Sarcoma-a Report of Three Cases FREE TO VIEW

Guangfa Zhu
Author and Funding Information

Beijing Anzhen Hospital, Beijing, China

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;149(4_S):A295. doi:10.1016/j.chest.2016.02.307
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SESSION TYPE: Original Investigation Poster

PRESENTED ON: Saturday, April 16, 2016 at 11:45 AM - 12:45 PM

PURPOSE: Pulmonary artery sarcoma (PAS) is an extremely rare and highly malignant tumor of pulmonary artery origin. Since 1923, when the first case was reported by Mandelstamm, no more than 300 cases have been reported worldwide.

METHODS: We report here three cases of PAS who were initially misdiagnosed as PTE or CTEPH, and were later found to be PAS after surgery.

RESULTS: We examined the clinical features of these patients for better diagnosis of PAS in the early stages of the disease because early diagnosis is critical for favorable prognosis for PAS patients.

CONCLUSIONS: PAS has a very poor prognosis, and early diagnosis with radical surgical resection offers PAS patients the only chance of survival.

CLINICAL IMPLICATIONS: Because of its rarity and non-specificity of clinical manifestations and imaging presentation, PAS is frequently misdiagnosed as pulmonary thromboembolic diseases such as pulmonary thromboembolism (PTE) or its late sequel chronic thromboembolic pulmonary hypertension (CTEPH).

DISCLOSURE: Guangfa Zhu: Consultant fee, speaker bureau, advisory committee, etc.: The national natural science foundation

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