Chest

Chest

Volume 110, Issue 6, December 1996, Pages 1480-1488
Journal home page for Chest

Clinical Investigations: Oncology
Pulmonary Artery Sarcoma: Clinical Features

https://doi.org/10.1378/chest.110.6.1480Get rights and content

Study objectives

To define characteristic clinical and radiographic presentations of patients found to have primary sarcoma of the pulmonary artery and differentiate this entity from acute and chronic pulmonary thromboembolism. To review pathologic characteristics of pulmonary artery sarcoma.

Design

A retrospective review of medical records at a large medical center and a review of the literature.

Setting

Tertiary care medical center.

Patients

Patients found to have pulmonary artery sarcoma based on surgical or autopsy findings.

Interventions

Review of medical records.

Measurements and results

Presenting symptoms of patients with pulmonary artery sarcoma are similar to those of patients with other pulmonary vascular disease, including progressive dyspnea, cough, hemoptysis, and chest pain. Symptoms not commonly associated with pulmonary vascular disease consist of progressive weight loss, anemia, and fever, which are symptoms commonly associated with malignancy. The diagnosis is often not suspected prior to surgery, which is often undertaken with an alternative diagnosis such as bronchogenic carcinoma or chronic pulmonary thromboembolism. Prolonged survival is rare; most patients survive only a few months. Survival longer than 3.5 years was not observed. Recent experience with MRI of the thorax and transesophageal echocardiography suggests that these diagnostic entities may be rewarding in separating the abnormalities seen on chest radiographs and angiography from thrombus in the pulmonary artery, mediastinal adenopathy, or primary bronchogenic carcinoma.

Conclusions

Although a rare entity, the key is to suspect the presence of pulmonary artery sarcoma in evaluation of patients with atypical presentations of pulmonary vascular disease especially if associated with symptoms such as weight loss, fever, or anemia.

Section snippets

Materials and Methods

We reviewed retrospectively the clinical records of all patients that we could identify through a computer-assisted search of all medical records with a diagnosis of pulmonary artery sarcoma or pulmonary artery tumor seen at the Mayo Clinic. The diagnosis could have been entered in the medical record as surgical diagnosis, a pathologic diagnosis, or a medical diagnosis. We excluded from this review cases of primary malignancies elsewhere that metastasized to the pulmonary vascular tree and

Results

We identified nine patients in a 30-year period from 1963 to 1993 (Table 1). Five were male, and the average age was 55 years. The presenting symptoms were similar in all and not dissimilar from patients with other pulmonary vascular disease; these included dyspnea, chest pain, cough, and hemoptysis (Table 2). Seven of the nine patients were originally thought to have subacute episodes of pulmonary embolism. Two were presumptively thought to have bronchogenic carcinoma as the cause of their

Discussion

In recent years, chest physicians have become more aggressive in the diagnosis and therapy of pulmonary vascular disease; for example, thoracotomy and thromboendarterectomy have become more common for pulmonary hypertension secondary to chronic pulmonary thromboembolism.4 In view of this trend, it is important to consider the full range of pulmonary vascular disorders, including the clinical and pathologic presentation of tumors of the pulmonary artery. Primary neoplasms arising in systemic

Addendum

Since submission of our article, another case of pulmonary artery sarcoma has been reported in the literature.50 This case reports that clubbing can occur in association with pulmonary artery sarcoma.

References (50)

  • SethiGK et al.

    Primary sarcoma of the pulmonary artery

    J Thorac Cardiovasc Surg

    (1972)
  • HeadHD et al.

    Long-term palliation of pulmonary artery sarcoma by radical excision and adjuvant therapy

    Ann Thorac Surg

    (1992)
  • SatoH et al.

    Primary pulmonary artery sarcoma: modern diagnostic imaging methods and successful surgical treatment

    J Thorac Cardiovasc Surg

    (1992)
  • KlinkeWP et al.

    Primary sarcoma of the pulmonary trunk: successful surgical intervention and prolonged survival

    Clin Cardiol

    (1985)
  • JamiesonSW et al.

    Experience and results with 150 pulmonary thromboendartereetomy operations over a 29-month period

    J Thorac Cardiovasc Surg

    (1993)
  • HynesJK et al.

    Preoperative angiographic diagnosis of primary sarcoma of the pulmonary artery

    Circulation

    (1982)
  • BleischVR et al.

    Polypoid sarcoma of the pulmonary trunk: analysis of the literature and report of a case with leptomeric organelles and ultrastructural features of rhabdomyosarcoma

    Cancer

    (1980)
  • BakerPB et al.

    Pulmonary artery sarcomas

    Arch Pathol Lab Med

    (1985)
  • HenrichsKJ et al.

    Leiomyosarcoma of the pulmonary artery

    Virchows Arch A Path Anat Histol

    (1979)
  • McGlennenRC et al.

    Pulmonary artery trunk sarcoma: a clinicopathologic ultrastructural, and immunohistochemical study of four cases

    Mod Pathol

    (1989)
  • JohanssonL et al.

    Sarcoma of the pulmonary artery: report of four cases with electron microscopic and immunohistochemical examinations

    Virchows Arch

    (1994)
  • BalduinR et al.

    Ante mortem diagnosis of primary leiomyosarcoma of the pulmonary artery

    Respiration

    (1986)
  • ElphinstoneRH et al.

    Sarcoma of the pulmonary artery

    Thorax

    (1959)
  • AltmanNH et al.

    Primary intimal sarcoma of the pulmonary artery

    Johns Hopkins Med J

    (1973)
  • BergGA et al.

    Primary pulmonary artery sarcoma: intra-operative similarity to pulmonary embolus

    Postgrad Med J

    (1987)

    • Cited by (0)

    Supported by the Mayo Foundation, Rochester, Minn. The authors have no financial interest in any organization or entity involving this manuscript.

    revision accepted July 11.

    View full text
    Copyright © 1996 The American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.