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Lung Cancer: Lung Cancer I |

Clinicopathological and Immunohistochemical Features of Pulmonary Artery Sarcoma- a Report of Three Cases FREE TO VIEW

Dong Chen
Author and Funding Information

Beijing Anzhen Hospital, Beijing, China


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


Chest. 2016;149(4_S):A294. doi:10.1016/j.chest.2016.02.306
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SESSION TITLE: Lung Cancer I

SESSION TYPE: Original Investigation Poster

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

PURPOSE: To evaluate the clinicopathological characteristics, and immunohistochemical phenotypes of pulmonary artery sarcoma (PAS).

METHODS: The clinical data, histopathological and immunohistochemical examinations in three patients with PAS confirmed by surgical biopsy in our hospital between 2008 and 2012 were retrospectively analyzed.

RESULTS: All three patients (two women, one man, average age 41 years old) presented with dyspnea on exertion, two had chest tightness, and one had intermittent syncope. Computed tomography pulmonary angiography showed that two patients had filling defect in the main pulmonary artery, left and right pulmonary arteries, and one patient had filling defect in the right upper pulmonary artery. All patients were misdiagnosed as pulmonary thromboembolic disease and underwent surgery. Macroscopically, the PAS (with length of 5.0 cm-9.5 cm, diameter of 1.2 cm -4.5cm) appealed as mucoid intraluminal or nodular sessile mass spreading along the pulmonary artery. Microscopically, the tumor consisted of spindle cells with fascicular and storiform patterns and was accompanied by necrosis and stromal myxoid change. Immunohistochemically, vimentin, desmin and CD34 were highly positive, and α-smooth muscle actin (α-SMA) was slightly positive in one patient, and vimentin and α-SMA were highly positive in the other two patients. Intimal sarcoma was diagnosed in one patient, and leiomyosarcoma was diagnosed in the other two patients.

CONCLUSIONS: PAS is an extremely rare tumor in the cardiovascular system. Because of non-specific clinical manifestation and radiologic features, PAS is often misdiagnosed.

CLINICAL IMPLICATIONS: The diagnosis of PAS should be based on typical morphological features and immunohistochemical analysis of the tumor tissue.

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

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