CASE 528 Published on 24.10.2000

Teaching Case

Section

Chest imaging

Case Type

Clinical Cases

Authors

M. Micallef, A.J.Collins, C.S.Elliott

Patient

38 years, female

Categories

No Area of Interest ; Imaging Technique CT, MR ;

No Procedure ; No Special Focus ;

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Clinical History

Recurrent right chest pain and dyspnea.

Differential Diagnosis List

Pulmonary angiosarcoma

Imaging Findings

First admitted with cough and right sided chest pain worse on inspiration. Chest radiograph and perfusion scan are shown. Spiral CT showed a filling defect in the lower and middle lobe vessels. The clinical condition did not improve. Thrombolysis was attempted with no success. Six months from the first admission the patient was readmitted with dyspnea, chest pain and hemoptysis. On examination there was a right pleural effusion and a pulmonary outflow murmur. Chest radiograph, lung perfusion scan and CT angiogram (shown) demonstrated a mass filling the lumen of the right main pulamonary artery and no perfusion of the right lung. Embolectomy was peformed in a specialist institution.

Discussion

Primary pulmonary angiosarcoma is very rare with only some 200 cases reported. However angiosarcomas remain the most common primary tumours of the heart and great vessels. The majority arise from the right atrium or pulmonary vessels. The associated clinical symptoms are varied and non-specific. Infiltration of the pulmonary arteries can mimic pulmonary embolism as in our case. Early diagnosis is therefore difficult but improves survival. Approximately 80% of the tumours behave as aggressive intra-luminal neoplasms arising from the intimal cells . They are characterised histologically by staining with factor VIII related antigen. Despite the increasing availability of echocardiography , spiral CT and MR studies distinction from chronic pulmonary embolism remains difficult with 50% of diagnoses made at thoracotomy/cytology. Recent interest has focused on dynamic contrast enhanced MR to obtain 2D multiplanar and ultrafast 3D sequences with some success.

References

[1] Kacl GM, Bruder E, Pfammatter T, Follath F, Salomon F, Debatin JF.
Primary angiosarcoma of the pulmonary arteries: Dynamic Contrast-Enhanced MRI.
J Comput Assist Tomogr. 1998 Sep-Oct;22(5):687-91. (PMID: 9754098)

[2] Bacha EA, Wright CD, Grillo HC, Wain JC, Moncure A, Keel SB, Donahue DM, Mathisen DJ.
Surgical treatment of primary pulmonary sarcomas.
Eur J Cardiothorac Surg. 1999 Apr;15(4):456-60. (PMID: 10371121)

[3] Ananthasubramaniam K, Farha A.
Primary right atrial angiosarcoma mimicking acute pericarditis, pulmonary embolism, and tricuspid stenosis.
Heart. 1999 May;81(5):556-8. (PMID: 10212178)

[4] Araoz PA, Eklund HE, Welch TJ, Breen JF.
CT and MR imaging of primary cardiac malignancies.
Radiographics. 1999 Nov-Dec;19(6):1421-34. Review. (PMID: 10555666)

Case information