A Case of Right Ventricular Outflow Tract and Pulmonary Artery Reconstruction Using a Composite Graft in a Patient with Pulmonary Artery Intimal Sarcoma / 日本心臓血管外科学会雑誌

A 46-year-old man had a 3-week history of persistent cough. Chest radiography showed a mass in the left pulmonary hilum and contrast-enhanced computed tomography (CT) showed an infiltrative mass extending from the main pulmonary trunk to the left hilar region into the lung. He was referred to our hospital for multidisciplinary treatments. Echocardiography showed that the proximal side of the tumor reached the pulmonary valve. CT revealed that the heterogeneous low-density tumor extended from the main pulmonary trunk to the left and right pulmonary arteries, and the left side of the tumor extended beyond the left pulmonary hilum into the left lung. A mass shadow of 54 mm in length was also seen in the lower lobe of the left lung along the pulmonary artery. Although the left bronchus was compressed by the tumor, there was no obvious intratracheal invasion. Direct invasion to the descending thoracic aorta was suspected. He underwent the resection of the main pulmonary trunk and the left pulmonary artery along with the tumor concomitant with total left pneumonectomy. Reconstruction of the pulmonary artery and right ventricular outflow tract were performed as follows: The right ventricular outflow tract was reconstructed by using a semilunar shaped bovine pericardial patch. The pulmonary artery was replaced by using a composite graft with a Dacron tube graft and an aortic bio-prosthesis. He was discharged on the 22nd postoperative day. The pathological diagnosis of the tumor was pulmonary artery intimal sarcoma. Although there are various reconstruction methods for pulmonary artery intimal sarcoma depending on the affected site, reconstruction of the pulmonary artery and the right ventricular outflow tract by using a composite graft are considered to be a useful method.

Pulmonary Artery Intimal Sarcoma versus Pulmonary Artery Thromboembolism: CT and Clinical Findings

OBJECTIVE: To describe CT and clinical findings of pulmonary artery intimal sarcoma (PAIS) compared with those of pulmonary thromboembolism (PTE), to investigate MRI and positron emission tomography (PET)-CT findings of PAIS, and to evaluate the effect of delayed diagnosis of PAIS on survival outcomes. MATERIALS AND METHODS: Twenty-six patients with PAIS were retrospectively identified and matched for sex, with patients with PTE at a ratio of 1:2. CT and clinical findings of the two groups were compared using Student's t test or chi-square test. The effect of delayed diagnosis on survival was investigated using Kaplan-Meier analysis. RESULTS: The most common tumor pattern in PAIS was tumoral impaction. Heterogeneous attenuation, wall eclipse signs, intratumoral vessels, acute interphase angles, single location, presence of lung ischemia, and central location were significantly more common in PAIS than in PTE (all p < 0.01). Levels of D-dimers and brain natriuretic peptide were lower in PAIS than in PTE (p < 0.05). In three patients of PAIS, long inversion time sequence MRI showed intermingled dark signal intensity foci suggestive of intermingled thrombi. All nine patients who had undergone PET-CT displayed hypermetabolism. Diagnosis was delayed in 42.3% of the PAIS patients and those patients had a significantly shorter overall survival than patients whose diagnosis was not delayed (p < 0.05). CONCLUSION: The characteristic CT and clinical findings of PAIS may help achieve early diagnosis of PAIS and make better survival outcomes of patients. MRI and PET-CT can be used as second-line imaging modalities and could help distinguish PAIS from PTE and to plan clinical management.

Pulmonary arterial intimal sarcoma with retrograde extension: Report of a case and review of literature.

Most of the pulmonary arterial sarcomas arise from multi-potential mesenchymal intimal cells and are designated as intimal sarcomas. These tumors grow in the direction of blood flow into peripheral arteries producing clinical features mimicking pulmonary thromboembolism. Retrograde extension is rare. We report one such case of intimal sarcoma that had a retrograde extension into the right ventricular outflow tract, and review such a presentation in the last ten years.

Long-Term Survival of a Patient with Pulmonary Artery Intimal Sarcoma after Sequential Metastasectomies of the Thyroid and Adrenal Glands

Cancer metastases to the thyroid or adrenal gland are uncommon. Furthermore, cases showing long-term survival after surgical resection of those metastatic tumors are rare. We report a case of pulmonary artery intimal sarcoma with metastases to the thyroid and adrenal glands sequentially that was successfully treated with sequential metastasectomies. A 62-year-old woman presented with a 4-week history of dyspnea on exertion and facial edema in November 1999. Echocardiography and chest computed tomography (CT) revealed an embolism-like mass in the pulmonary trunk. Pulmonary artery endarterectomy with pulmonary valve replacement was performed, and histopathology revealed pulmonary artery intimal sarcoma. A thyroid nodule was found by chest CT in November 2001 (2 years after initial surgery). During follow-up, this lesion showed no change, but we decided to obtain fine needle aspiration cytology (FNAC) in August 2004 (4.7 years after initial surgery). FNAC revealed atypical spindle cells suggestive of metastatic intimal sarcoma. She underwent total thyroidectomy. During follow-up, a right adrenal gland mass was detected by chest CT in March 2006 (6.3 years after initial surgery), and adrenalectomy was done, which also revealed metastatic sarcoma. She has been followed up without any evidence of recurrent disease until May 2012 (12.5 years after initial surgery).

A Case of Intimal Sarcoma Arising in the Left Common Iliac Artery

Primary tumors of the great vessels are rare. Most encountered cases are sarcomas which most commonly develop in the aorta, pulmonary artery, and inferior vena cava. We experienced an intimal sarcoma arising in the left common iliac artery in a 68-year-old male, who suffered from claudication in his left lower extremity for a year and was diagnosed as arteriosclerosis obliterans, clinically. Bypass surgery was performed on the obstructive lesion. Grossly, the vascular lumen was filled with dark hemorrhagic materials. Microscopically, the lesion showed proliferation of anaplastic spindle cells with a marked nuclear atypia, arranged haphazardly. There were numerous mitotic figures. Foci of cholesterol clefts were also found in the intima. Immunohistochemically, the tumor cells were positive for vimentin, smooth muscle actin, and cytokeratin in certain areas. Stains for CD34, desmin, myosin heavy chain, caldesmon, and S-100 protein were negative. A pathologic diagnosis was made as intimal sarcoma with myofibroblastic differentiation.

A Case of Pulmonary Artery Intimal Sarcoma Diagnosed with Multislice CT Scan with 3D Reconstruction

Pulmonary artery intimal sarcoma is a rare highly lethal disease, with additional retrograde extension to pulmonic valve and right ventricle being an extremely rare condition. It is frequently mistaken for pulmonary thromboembolism. We report a case of 64-year-old woman with progressive dyspnea initially suspected and treated for pulmonary thromboembolism. Her helical chest CT scan with 3 dimensional (3D) reconstruction combined with echocardiography revealed a compacting main pulmonary artery mass extending to the right ventricular outflow tract and the right pulmonary artery. After excision of the mass, the patient's condition improved dramatically, and the pathologic findings revealed pulmonary intimal sarcoma. This report emphasizes that helical chest CT with 3D reconstruction can be an important tool to differentiate the characteristics of pulmonary artery lesions, such as intimal sarcoma and thromboembolism.