Pulmonary Vascular Disease |

Primary Pulmonary Artery Sarcoma Presenting as a Massive Pulmonary Embolism FREE TO VIEW

Umair Tariq, MD; Marina Dolina, MD
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York Hospital, York, PA

Chest. 2015;148(4_MeetingAbstracts):981A. doi:10.1378/chest.2280998
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SESSION TITLE: Pulmonary Vascular Disease Student/Resident Case Report Posters

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: Spindle cell sarcoma is a rare form of cancer that develops from tissues such as bone, fat, muscle, nerves, fibrous tissues and the blood vessels . Sarcoma of the intimal layer of large vessels has female to male ratio of 1.3:1. Pulmonary artery spindle cell sarcoma, a highly malignant tumor that can present similar to a pulmonary embolism, with shortness of breath, chest pain, and hemoptysis. The definitive diagnosis is made via biopsy of the mass or during an autopsy.

CASE PRESENTATION: A 52-year-old previously healthy female was seen a few times in ER with non-significant hemoptysis.During the third visit to the ER she reported recent travel overseas. A CAT scan of her chest showed a massive saddle pulmonary embolus occupying the pulmonary trunk and bilateral pulmonary artery branches. While in ER, she became hypoxemic and hemodynamically unstable and received tissue plasminogen activator (TPA). Patient developed massive hemoptysis within a few hours after TPA administration. Patient was placed on VV-ECMO due to profound hypoxia and hemodynamic instability. Cardiac echocardiogram demonstrated severe pulmonary hypertension. Repeated CAT scan showed no improvement in pulmonary artery blood flow, and patient was taken for embolectomy. During surgery, a mass adherent to pulmonary artery walls was noted and thromboendarterectomy was performed. Pathologic evaluation of mass showed high-grade intimal spindle cell sarcoma. Patient had an extensive hospital stay, requiring tracheostomy placement and slow liberation from ventilatory support. Chemotherapy was started in hospital with follow up as an outpatient.

DISCUSSION: Pulmonary artery intimal sarcomas can invade the pulmonary valve and right ventricle, and 40% of the patients develop metastasis to the lungs. Distant metastasis to lymph glands or myocardium occurs in 16% to 25% of cases. Surgical removal of the tumor offers the best therapy. The tumor has a poor response to radiation therapy and chemotherapy. Without tumor resection, the average life expectancy is 12 to 18 months from diagnosis.

CONCLUSIONS: Our case signifies the importance of proper work-up in otherwise healthy patients with hemoptysis, as well as considering other diagnosis in patients presenting with “pulmonary embolism” and not responding to established therapy. Availability of highly trained surgeon and ECMO in our institution, allowed patient to survive through a catastrophic event and established a proper diagnosis.

Reference #1: Lee DH , Jung TE Lee JH, Shin DG, Park,WJand Choi HJ; Pulmonary artery intimal sarcoma: poor 19F fluorodexyglucose uptake in positron emission computed tomography. J Cardiothorac Surg. 2013;8:40.

Reference #2: Furest M, Marín P Escribano MA, Gómez J, Cortinac J, Blanquer R, Intimal sarcoma of the pulmonary artery: A rare case of pulmonary hyptertension. Arch Bronconeumol. 2006 Mar;42(3):148-50.

Reference #3: Varriale P, Chryssos B.; Pulmonary artery sarcoma: another cause of sudden death. Clin Cardiol. 1991;14(2):160-64.

DISCLOSURE: The following authors have nothing to disclose: Umair Tariq, Marina Dolina

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