Intimal pulmonary artery sarcoma presenting as dyspnea: case report
© Hsing et al; licensee BioMed Central Ltd. 2007
Received: 17 May 2006
Accepted: 29 June 2007
Published: 29 June 2007
We report a case of pulmonary sarcoma which is a rare cause of the common symptom of dyspnea.
A fifty-one year old previously healthy male presented to the emergency room with complaints of dyspnea on exertion. A cardiac workup including an exercise stress test was negative but an echocardiography showed pulmonary stenosis. Cardiac MRI showed a large mass extending from the pulmonic valve to both the right and left pulmonary arteries suggestive of sarcoma. A complete resection and repair of the pulmonary artery was done and adjuvant chemotherapy with doxorubicin and ifosfamide was recommended. The patient is currently disease free after eighteen months.
Pulmonary artery sarcomas are a difficult diagnosis. The diagnosis may remain elusive for some time until the proper imaging techniques are utilized to make a diagnosis. Earlier and accurate diagnosis may lead to earlier interventions and improve survival.
Dyspnea is a frequent presentation to the emergency room. The differential diagnosis is extensive and often the diagnosis remains obscure. Pulmonary sarcomas are a rare cause of dyspnea, but do present as such and are often initially misdiagnosed.
The patient tolerated the surgery well and was discharged with oncology follow-up as an outpatient. Adjuvant chemotherapy with doxorubicin and ifosfamide was initiated soon thereafter. Eighteen months later and after 7 cycles of chemotherapy, he continues to remain disease free by imaging.
Sarcoma of the great vessels are rare and are usually found in the aorta (most often abdominal), inferior vena cava, or pulmonary artery. Typically, these sarcomas are rare and highly lethal that previously were diagnosed during surgery or autopsy. Patients typically present between the ages of 22 and 81 with female sex predominance. The prognosis is poor with a mean survival of 12 months after onset of symptoms and 1 and 2 year survival rates of 22% and 7% respectively. The presentation usually corresponds to the location of the sarcoma. In the case of a pulmonary artery sarcoma dyspnea, chest pain, cough and hemoptysis are the most frequent complaints. A common error involves diagnosing a pulmonary thromboembolism and treating it as such. There have been a number of case reports and several literature reviews discussing the findings of a pulmonary artery sarcoma. Older case reports usually only made the correct diagnosis during surgery or autopsy and only after a prolonged period of symptoms. More recent advances in imaging technology with multi-slice CT and MRI have enabled better tissue and anatomy characterization [3–5]. This has enabled making the correct diagnosis earlier and differentiating it from other pulmonary vascular pathologies, namely thromboembolism. Quicker diagnosis allows earlier potential curative treatment with surgical resection and adjuvant chemotherapy with or without radiation. At present it is unclear whether earlier diagnosis followed by earlier initiation of treatment leads to improved overall survival.
Summary of the common types of sarcomas of the great vessels
Cell of Origin
Vimentin, Factor VIII, CD 34
The mainstay of treatment for sarcoma is surgical resection as this remains the only potentially curative modality. Adjuvant radiation and chemotherapy can be considered following surgical excision although their role remains undefined. An approximate 20% response rate can be expected with a combination chemotherapy regimen involving an anthracycline and an alkylating agent, however, the value of this regimen in the adjuvant setting for pulmonary artery sarcoma is unclear[8, 9].
Magnetic Resonance Imaging
Positron Emission Tomography
Written consent was obtained from the patient for publication.
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