Management of Primary Pulmonary Artery Sarcomas

doi:10.1016/j.athoracsur.2008.08.018

The Annals of Thoracic Surgery

Volume 87, Issue 3, March 2009, Pages 977-984

https://doi.org/10.1016/j.athoracsur.2008.08.018 Get rights and content

The objective of this review is to determine the outcome of patients with sarcomas involving the main pulmonary artery, pulmonic valve, or right ventricular outflow tract. Survival data were analyzed using an aggregate series derived from the published literature in conjunction with a current series. Median survival was 36.5 ± 20.2 months for patients undergoing an attempt at curative resection compared with 11 ± 3 months for those undergoing incomplete resection. Median survival was 24.7 ± 8.5 months for patients undergoing multimodality treatment compared with 8.0 ± 1.7 months for patients having single-modality therapy. A complete review of diagnosis, evaluation, treatment, and surveillance of primary pulmonary artery sarcomas follows.

Section snippets

Methods

A literature search of Ovid/Medline was performed using the terms “sarcoma,” “pulmonary artery,” “surgery,” and/or “chemotherapy,” and/or “radiation therapy.” The search was limited to English articles about humans in the past 17 years. Each article was screened for central primary PA sarcomas as the diagnosis for each patient. Patients with lung parenchymal disease were included as long as their primary tumor was a central primary PA sarcoma. The analysis only included PA sarcomas undergoing

Comment

Primary PA sarcomas are rare tumors, with fewer than 250 cases reported. Although surgical resection is the standard of care, radical complete resection is rarely reported for tumors involving the main PA, pulmonic valve, or right ventricular outflow tract. In patients undergoing PA resection instead of thrombectomy, in conjunction with chemotherapy, our series reports a long median survival of 71 months. This improved survival is thought to be due to two factors: aggressive resection with a

References (62)

S. Miura et al.
Intimal sarcoma of the pulmonary artery: report of an autopsy case
Pathol Res Pract
(2005)
M.B. Anderson et al.
Primary pulmonary artery sarcoma: a report of six cases
Ann Thorac Surg
(1995)
M.J. Reardon et al.
Cardiac autotransplantation for primary cardiac tumors
Ann Thorac Surg
(2006)
M.L. Redmond et al.
Primary pulmonary artery sarcomaA method of resection
Chest
(1990)
H.D. Head et al.
Long-term palliation of pulmonary artery sarcoma by radical excision and adjuvant therapy
Ann Thorac Surg
(1992)
J.R. Goldblum et al.
Epithelioid angiosarcoma of the pulmonary artery
Hum Pathol
(1995)
H.R. Zerkowski et al.
Primary sarcoma of pulmonary artery and valve: multimodality treatment by chemotherapy and homograft replacement
J Thorac Cardiovasc Surg
(1996)
J.M. Parish et al.
Pulmonary artery sarcoma: clinical features
Chest
(1996)
C. Akomea-Agyin et al.
Pulmonary artery sarcoma mimicking pulmonary embolism: successful surgical intervention
Ann Thorac Surg
(1996)
H. Tanaka et al.
Successful radical resection of a leiomyosarcoma of the pulmonary trunk
J Thorac Cardiovasc Surg
(2001)

D. Govender et al.

Right pulmonary artery sarcoma

Pathology

(2001)

A. Mattoo et al.

Pulmonary artery sarcoma: a case report of surgical cure and 5-year follow-up

Chest

(2002)

M. Totaro et al.

Cardiac angiosarcoma arising from pulmonary artery: endovascular treatment

Ann Thorac Surg

(2004)

Y. Tsunezuka et al.

Primary chondromatous osteosarcoma of the pulmonary artery

Ann Thorac Surg

(2004)

B.J. Park et al.

Surgical management of thoracic malignancies invading the heart or great vessels

Ann Thorac Surg

(2004)

I. Furest et al.

Intimal sarcoma of the pulmonary artery: a rare cause of pulmonary hypertension

Arch Bronconeumol

(2006)

A. Maruo et al.

Surgical experience for the pulmonary artery sarcoma

Ann Thorac Surg

(2006)

F. Heid et al.

Extracorporal circulation and cardiac arrest in an awake patient: a safe approach for single lung pulmonary artery stenting?

Ann Thorac Surg

(2006)

E.S. Yi

Tumors of the pulmonary vasculature

Cardiol Clin

(2004)

B. Riedel et al.

Diagnosis and management of persistent pulmonary thromboembolism

J Cardiothorac Vasc Anesth

(2006)

R.B. Rafal et al.

Pulmonary artery sarcoma: diagnosis and postoperative follow-up with gadolinium- diethylenetriamine pentaacetic acid–enhanced magnetic resonance imaging

Mayo Clin Proc

(1995)

F. Ricou et al.

Catheter-based intravascular ultrasound imaging of chronic thromboembolic pulmonary disease

Am J Cardiol

(1991)

D.D. Esakof et al.

Delineation of pulmonary artery sarcoma with multiplane and panoramic transesophageal echocardiography

J Am Soc Echocardiogr

(1993)

R.L. Thurer et al.

FDG imaging of a pulmonary artery sarcoma

Ann Thorac Surg

(2000)

B.M. Shmookler et al.

Primary sarcoma of the pulmonary trunk and/or right or left main pulmonary arteryA rare cause of obstruction to right ventricular outflow; report on two patients and analysis of 35 previously described patients

Am J Med

(1977)

E. Mayer et al.

Surgical treatment of pulmonary artery sarcoma

J Thorac Cardiovasc Surg

(2001)

M. Mandelstamm

Uber primare Neubildungen des Herzens

Virchows Arch

(1923)

L. Huo et al.

Pulmonary artery sarcoma: a clinicopathologic and immunohistochemical study of 12 cases

Am J Clin Pathol

(2006)

A. Nonomura et al.

Primary pulmonary artery sarcomaReport of two autopsy cases studied by immunohistochemistry and electron microscopy, and review of 110 cases reported in the literature

Acta Pathol Jpn

(1988)

I. Kruger et al.

Symptoms, diagnosis, and therapy of primary sarcomas of the pulmonary artery

Thorac Cardiovasc Surg

(1990)

M.J. Reardon et al.

Therapy insight: malignant primary cardiac tumors

Nat Clin Pract Cardiovasc Med

(2006)

Cited by (186)

Pulmonary Sarcoma: A Wolf in Sheep's Clothing
2024, American Journal of Medicine
Primary cardiac sarcomas: Treatment strategies
2023, Journal of Thoracic and Cardiovascular Surgery
Our multidisciplinary cardiac tumor team now has an experience of operating on 122 cases of primary cardiac sarcoma over a 23-year period. The purpose of this study is to present our short- and long-term outcomes for cardiac sarcoma.
We performed a retrospective review of a prospectively collected Institutional Review Board–approved cardiac tumor database for cardiac sarcoma. Patient characteristics, surgical factors, and patient outcomes were analyzed. Perioperative data were collected from direct patient communication and all available medical records. The primary end point was all-cause mortality at 1, 3, and 5 years from the time of our surgery and 1, 3, and 5 years from the initial diagnosis. The secondary end point was all-cause mortality between the first and second halves of the study.
From October 1998 to April 2021, we operated on 122 patients with a primary cardiac sarcoma. The mean age was 45.3 years old, and 52.5% were male. Tumors were most frequently found in the left atrium (40.2%) and right atrium (32.0%). The most common type of tumor histologically was an angiosarcoma (38.5%), followed by high-grade sarcoma (14.8%). Survival from initial diagnosis at 1, 3, and 5 years was 88.4%, 43.15%, and 27.8%, respectively. Survival from surgery at our institution at 1 and 3 years was 57.1% and 24.5%, respectively. When comparing outcomes from different time periods, we found no significant difference in survival between the previous era (1998-2011) and the current era (2011-2021).
Management of these complex patients can show reasonable outcomes in centers with a multidisciplinary cardiac tumor team. Mortality has not improved with time and is likely related to the systemic nature of this disease.
Not always embolism: A case of pulmonary artery intimal sarcoma – The role of the radiologist in early diagnosis
2023, Respiratory Medicine Case Reports
Pulmonary artery intimal sarcoma (PAIS) is a rare malignant neoplasm with imaging features that can mimic pulmonary embolism (PE). It must be recognized early because a radical resection may be useful to prolong survival.
A clinical case of a 57-year-old Caucasian male affected by PAIS is presented, which describes the computed tomography (CT) findings found in PAIS and the elements of overlap and differentiation with PE. The main common element is represented by the endoluminal filling defect of the pulmonary arterial vessels in contrast-enhanced CT examinations; a characteristic polypoid morphology or polylobulated contours are typical findings of PAIS. Other specific elements of the neoplasm such as wall eclipse sign, extension beyond the arterial wall, and metastasis are also explained.
The overlap of the clinical–radiological findings and the epidemiological difference between PAIS and PE cause a diagnostic delay. By knowing the differential elements, the radiologist can detect the neoplasm early to accelerate diagnosis and suggest optimal management.
Management of Pulmonary Artery Synovial Sarcoma: The 3-Step Surgical Approach
2022, Annals of Thoracic Surgery
A 52-Year-Old Man With Chest Pain and Dyspnea
2022, Chest
A 52-year-old man came to the cardiac surgery clinic for pulmonary thromboendarterectomy (PTE) evaluation. He had initially appeared at an outside hospital 1 year earlier, with chest pain and shortness of breath. He had no known chronic conditions. A CT pulmonary angiogram (CTPA) at that time showed a filling defect at the bifurcation of the main pulmonary artery. A transthoracic echocardiogram revealed mild mitral valve regurgitation, but otherwise the results were normal. As he was hemodynamically stable and not hypoxemic, he was treated solely by anticoagulation. Despite adhering to prescribed apixaban, he developed progressive dyspnea and reduced exercise tolerance over the subsequent year. A repeat CTPA performed 12 months after the initial presentation showed a persistent filling defect at the level of the pulmonary artery bifurcation, with a new extension now completely occluding the right main pulmonary artery. A pulmonary angiogram confirmed this complete occlusion, and right heart catheterization revealed precapillary pulmonary hypertension, with a mean pulmonary artery pressure of 50 mm Hg. His anticoagulation was transitioned to enoxaparin for presumed apixaban treatment failure, and an investigation for hypercoagulable conditions was initiated. His lupus anticoagulant test result was positive, but he did not meet the criteria for antiphospholipid syndrome because he was negative for anticardiolipin and β₂-glycoprotein antibodies. Assays for antithrombin III, protein C, prothrombin gene, and factor V Leiden mutations produced normal results.
A Not So Innocent Murmur
2022, Heart Lung and Circulation

View all citing articles on Scopus

View full text

ReviewManagement of Primary Pulmonary Artery Sarcomas

Section snippets

Methods

Comment

Pathol Res Pract

Ann Thorac Surg

Ann Thorac Surg

Chest

Ann Thorac Surg

Hum Pathol

J Thorac Cardiovasc Surg

Chest

Ann Thorac Surg

J Thorac Cardiovasc Surg

Pathology

Chest

Ann Thorac Surg

Ann Thorac Surg

Ann Thorac Surg

Arch Bronconeumol

Ann Thorac Surg

Ann Thorac Surg

Cardiol Clin

J Cardiothorac Vasc Anesth

Mayo Clin Proc

Am J Cardiol

J Am Soc Echocardiogr

Ann Thorac Surg

Am J Med

J Thorac Cardiovasc Surg

Uber primare Neubildungen des Herzens

Virchows Arch

Pulmonary artery sarcoma: a clinicopathologic and immunohistochemical study of 12 cases

Am J Clin Pathol

Primary pulmonary artery sarcomaReport of two autopsy cases studied by immunohistochemistry and electron microscopy, and review of 110 cases reported in the literature

Acta Pathol Jpn

Symptoms, diagnosis, and therapy of primary sarcomas of the pulmonary artery

Thorac Cardiovasc Surg

Therapy insight: malignant primary cardiac tumors

Nat Clin Pract Cardiovasc Med

Review
Management of Primary Pulmonary Artery Sarcomas