Ann Thorac Surg 2008;86:1354-1355. doi:10.1016/j.athoracsur.2008.04.005
© 2008 The Society of Thoracic Surgeons
Case Reports
Pulmonary Artery Sarcoma Mimicking a Pulmonary Artery Aneurysm
Ricardo M. Terra, MDa,*,
Angelo Fernandez, MDa,
Ricardo H. Bammann, MDa,
Jader J.M. Junqueira, MDa,
Vera L. Capelozzi, MDb
a Advanced Thoracic Nucleus, Sírio-Libanes Hospital, São Paulo, Brazil
b Diagnostika, Surgical Pathology, São Paulo, Brazil
Accepted for publication April 1, 2008.
* Address correspondence to Dr Terra, Rua Adma Jafet, 50–Cjto. 55. Cerqueira César, São Paulo, 01308-050, Brazil (Email: rmterra{at}uol.com.br).
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Abstract
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Pulmonary artery sarcoma is an uncommon neoplasm, and its clinical and radiological presentation usually simulates chronic thromboembolic disease. We present the case of a 77-year-old woman admitted with dyspnea, chest pain, and hemoptysis. A chest computed tomographic scan showed moderate right-sided pleural effusion and a saccular dilatation of the interlobar portion of the right pulmonary artery, which was filled with contrast and surrounded by an irregular soft-tissue attenuation mass, suggesting a ruptured pulmonary artery aneurysm. The patient was operated on. Intraoperatively, a pseudoaneurysm and a solid mass were identified within the oblique fissure around the interlobar artery. Therefore, a right pneumonectomy was performed. Definitive pathologic examination was consistent with pulmonary artery sarcoma. The patient had a good outcome and is free of disease 2 years after surgery.
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Introduction
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Primary sarcomas of the pulmonary artery are rare and only approximately 200 cases have been reported [1]. Diagnosis is always a challenge because, due to its rarity, suspicion is low and the clinical presentation and radiologic features frequently mimic acute or chronic thromboembolic disease [2, 3]. We report an uncommon case of a pulmonary artery sarcoma masquerading as pulmonary artery pseudoaneurysm. We believe that this presentation has not ever been previously described.
A 77-year-old woman, with previous history of hypertension, presented at the emergency room complaining of increasing dyspnea, chest pain, and hemoptoic sputum that had begun 2 hours before admission. During physical examination, mild tachypnea (18 breaths per minute) and signs of right pleural effusion were observed. Although thromboembolic disease was initially considered, the chest roentgenogram showed a round shadow at the right lung hilum and moderate right pleural effusion. D–dimer, cardiac enzymes, electrocardiography, and transthoracic echocardiography showed no abnormalities. A contrast-enhanced computed tomographic scan showed moderate right-sided pleural effusion and a saccular dilatation of the interlobar portion of the right pulmonary artery that was filled with contrast and surrounded by an irregular soft-tissue attenuation mass (Fig 1A). A magnetic resonance imaging scan was also performed; it confirmed the findings of a computed tomographic scan and allowed a better definition of the relationship between the mass and the anterior pulmonary artery trunk (Fig 1B). Thoracentesis revealed a hemorrhagic pleural effusion. With the hypothesis of a ruptured but contained pulmonary artery aneurysm, the patient was operated on through a right lateral thoracotomy, and cardiopulmonary bypass was available. A pseudoaneurysm and a solid mass (the frozen section proved to be of malignant cause) were identified within the oblique fissure around the interlobar artery. Due to the extension of the lesion, a more conservative procedure was not possible, and a right pneumonectomy was performed to completely resect the mass together with the partially ruptured pseudoaneurysm. The patient experienced no postoperative complications and was discharged on postoperative day 4. Gross examination showed a mass resembling mucoid or gelatinous clots. The cut surface showed firm fibrotic areas with mixoid foci, hemorrhage, and necrosis. Histologically, a typical proliferation of spindle cells in a myxoid background was found, alternating with hypocellular collagenized stroma. By immunohistochemistry, the tumor cells exhibited strong and diffuse immunoreactivity for vimentin, foci of reactivity for smooth muscle actin, desmins, and CD34 endothelial marker. Final pathologic examination of the specimen was consistent with a pulmonary artery sarcoma. As resection was complete, no further adjuvant therapy was considered. Follow-up of the patient has been made for each 3 months thereafter, and she is free of disease for 2 years since the operation, based on clinical signs, physical examination, and image studies (ie, chest and abdominal computed tomographic scans and brain magnetic resonance imaging scan).
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Fig 1. (A) Chest computed tomographic scan shows: 1 = right pulmonary artery, 2 = dilated interlobar artery, and 3 = irregular soft-tissue attenuation mass surrounding saccular dilation. (B) Chest magnetic resonance imaging scan shows: 1 = right pulmonary artery, 2 = interlobar pulmonary artery, 3 = saccular dilation of the interlobar artery, and 4 = soft-tissue attenuation mass mimicking a thrombus.
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Comment
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In appropriate clinical settings, an aneurysm or pseudoaneurysm should be suspected in patients who present with hemoptysis and in whom chest radiography shows hilar enlargement or a new focal lung mass that has stable or increased size at subsequent radiographic examination [4]. Behcet's disease and mycotic aneurysms are the most common causes of acquired pulmonary artery aneurysm; however, the typical clinical findings of mucosal ulcers, as well as an active infection were not present. Primary lung tumors or metastatic disease are also uncommon causes of pulmonary artery pseudoaneurysm. In this case, the first hypothesis was a ruptured but contained pulmonary artery aneurysm surrounded by a mural thrombus, but the actual diagnosis was only elucidated intraoperatively. Early diagnosis is crucial as the mortality rate for patients with a ruptured pulmonary artery aneurysm is high [5]. Therefore, due to the risk of massive bleeding, the patient was referred to urgent surgery. Pulmonary artery sarcoma treatment is complete excision, even though few reported cases were considered cured. Most series describe pulmonary artery sarcoma as a centrally located disease mimicking chronic thromboembolism, but the interlobar position of the tumor observed in this patient, which is also uncommon, allowed a complete resection and a margin-free specimen achieved through a pneumonectomy. The patient is doing well 2 years after surgery with no evidence of recurrence. A pulmonary artery sarcoma presenting as a pulmonary artery pseudoaneurysm has never been previously reported, and this case shows that this rare disease may have different clinical courses than the usual thromboembolic disease-like scenario. Reviewing the case, the key point for a correct preoperative diagnosis would be the irregular soft-tissue attenuation mass surrounding the contrast-filled lumen. At first sight we considered that as a mural thrombus, but looking back it was too thick and irregular. Therefore, we conclude that pulmonary artery sarcoma may present as a ruptured pseudoaneurysm of the interlobar artery, and in this case surgical excision was successful.
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References
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- Kerr KM. Pulmonary artery sarcoma masquerading as chronic thromboembolic pulmonary hypertension Nat Clin Pract Cardiovasc Med 2005;2:108-112.[Medline]
- Christopher K, Bahaaldin A, Irving S, et al. Recurrent pulmonary artery sarcoma J Card Surg 2006;21:587-589.[Medline]
- Delany SG. Pulmonary artery sarcoma mimicking pulmonary embolism Chest 1993;103:1631-1633.[Medline]
- Remy-Jardin M, Remy J. Spiral CT angiography of the pulmonary circulation Radiology 1999;212:615-636.[Abstract/Free Full Text]
- DeLima LG, Wynands JE, Bourke ME, et al. Catheter-induced pulmonary artery false aneurysm and rupture: case report and review J Cardiothorac Vasc Anesth 1994;8:70-75.[Medline]
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Abstract
Introduction
