• Journal of Chest Surgery
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• 제33권2호
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• pp.195-198
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• 2000

Primary sarcomas of the pulmonary arteries are and the clinical presentation closely resembles several more common conditions, Therefore they are seldomly diagnosed preoperatively without a pathologic esxamination of sufficient surgical specimen. Treatment of these tumors remains primarily surgical as possible and the role of the adjuvant chemotherapy and radiotherapy is not yet evident We report a case of primary sarcoma of pulmonary artery clinically suspected of carcinoid partially involving the pulmonary parechyme and the bronchus mainly in the left main pulmonary artery and the vein with left intrapericardial pneumonectomy.

• Journal of Chest Surgery
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• 제36권12호
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• pp.979-984
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• 2003

원발성 폐동맥 육종은 매우 드문 질환이다. 임상증상과 방사선학적 소견이 폐동맥 색전증과 유사하기 때문에 진단 시 폐동맥 색전증으로 오진되는 예가 빈번하고, 본 질환의 빠른 진행속도로 인하여, 사망 후 부검을 통해 확인되는 예도 있다. 따라서 폐동맥 색전증으로 진단된 환자가 혈전의 원발병소가 불분명하면서 항응고제에 반응하지 않는다면, 원발성 폐동맥 육종을 의심해 보아야 한다. 폐동맥 색전증으로 진단받은 57세 남자 환자가 5개월간의 항응고제 치료 후에도 우측폐의 종괴모양 병변이 증가하고 주폐동맥 색전증의 크기가 증가되어 수술적인 치료과정에서 폐동맥 내막육종(Pulmonary artery intimal sarcoma)으로 확인되었다. 수술전 혈전으로 의심되었던 저음영의 종괴는 동결조직검사 상 폐동맥 육종으로 의심되었으며, 심낭에 침윤이 있었다. 그 병변과 독립적으로 우폐동맥과 폐실질에서도 혈관육종이 발견되었으며, 인공심폐기하에서 주폐동맥의 완전 절제술 시행 후 Gore-tex graft 치환술과 우측 전폐절제술을 시행하였다. 수술 후 2차례의 항암치료를 시행한 후에 퇴원하였다.

• Tuberculosis and Respiratory Diseases
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• 제62권3호
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• pp.232-236
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• 2007

폐동맥 육종은 극히 드문 질환으로 폐동맥 혈전증과 혼동되는 경우가 많다. 육종에서는 FDG 섭취가 증가되므로 $^{18}F-FDG-PET$를 시행하면 FDG 섭취가 없는 색전증과 구별할 수 있지만 해상도가 떨어지는 단점이 있다. 그러므로 CT 영상을 접목한 PET/CT는 폐동맥 육종과 혈전증을 보다 선명하게 구별할 수 있다. 저자들은 혈전증과 감별이 어려운 폐동맥 병변에 대해 PET/CT로 폐동맥 육종의 진단에 도움을 받았던 증례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• Journal of Yeungnam Medical Science
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• 제34권2호
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• pp.279-284
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• 2017

Pulmonary artery sarcoma (PAS) is a rare and fatal disease that often mimics chronic thromboembolic pulmonary hypertension (CTEPH); therefore, diagnosis of PAS is often delayed. Herein, a healthy 74-year-old man was presented with a 4-month history of dyspnea. Chest computed tomography showed wall thickening and stenosis in the main pulmonary artery as well as in both proximal pulmonary arteries. In order to differentiate between unusual CTEPH, vasculitis, and PAS, we performed right heart catheterization and pulmonary angiography. The mean pulmonary arterial pressure was 21 mmHg, and there was severe pulmonary artery stenosis. Thrombi on the pulmonary arterial wall lesions were observed in intravascular ultrasound and optical coherence tomography. Furthermore, the patient had a history of deep vein thrombosis. Therefore, we diagnosed unusual CTEPH. After 6 months of rivaroxaban anticoagulation therapy, a chest X-ray revealed a left lower lobe lung mass, and a positron emission tomography later showed hypermetabolic lesions in the main pulmonary artery wall, in both pulmonary arteries walls, in the lung parenchyma, and in the bones. A biopsy of the right proximal humerus lesion revealed undifferentiated intimal sarcoma. Pulmonary sarcoma is rare, but should be considered when differentially diagnosing main pulmonary artery wall thickening and stenosis. A positron emission tomography may aid in this diagnosis.

• Journal of Chest Surgery
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• 제30권10호
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• pp.1036-1039
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• 1997

원발성 폐동맥 육종은 드물며, 흔히 폐동맥 색전증으로 오진하기 쉽다. 예후는 매우 좋지않아서 치료하지 않았을 때의 생존율이 약 1.5개월이며,수술후에도 생존율을 일년정도까지 연장할 수있다. 본 교실에서는 원발성 폐동맥 육종을 1례 치험하였기에 보고하고자 한다. 환자는 55세 여자로 주증상은 호흡곤란, 우측흉통, 및 객혈이었다. 술전 흉부 전산화단층촬영상 폐동맥 색전증과 유사한 종괴음영을 나타내었다. 수술은 부분 체외순환하에 폐동맥 성형술과 우측전폐 절제술이 시행되었다. 술후 조직학적 진단은 미분화 육종이었고, 증상은 다소 호전되었으나 술후 3개월에 원인 미상으로 사망하였다.

• Journal of Chest Surgery
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• 제33권10호
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• pp.823-826
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• 2000

Primary intimal sarcoma of the pulmonary artery is a rare disease and there has been no report of any case originating from the pulmonary valve. Recently we experienced a 62 year-old female patient who had a primary intimal sarcoma of the pulmonary valve with distal metastasis. She was brought to medical attention due to exertional dyspnea facial edema productive coughing and general weakness for 1 month. Chest CT and echocardi-ography suggest an acute pulmonary thromboembolism or tumor. Exploration showed a large polypoid mass arising from the pulmonary leaflets and multiple masses on distal pulmonary arteries. We replaced the pulmonary valve and reconstructed the pulmonary artery. She received radiotherapy 1 month postoperatively and now 4 months after surgery she has begun receiving chemotherapy.

• Journal of Chest Surgery
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• 제37권2호
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• pp.173-175
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• 2004

원발성 폐동맥 육종, 특히 폐동맥 판막에서 기원한 폐동맥 육종은 극히 드물다. 35세 남자 환자가 노작성 호흡곤란과 전신무력감을 주소로 입원하였다. 흉부 컴퓨터 단층촬영, 흉부 자기공명영상, 심장초음파 검사, 폐동맥 조영술상 주 폐동맥 및 양측 폐동맥의 내강에서 커다란 종괴가 관찰되었다. 폐동맥의 원발성 악성 종양으로 생각하고 심폐순환하에 절제를 시행하였다. 폐동맥 판막의 후엽에서 기원한 것으로 보이는 종괴가 폐동맥 판막의 하부에서부터 주폐동맥 및 양측 폐동맥까지 뻗어 있었고, 폐동맥 내막절제술을 통한 종괴의 절제 및 폐동맥 판막 치환술을 시행하였으며, 병리조직학적 검사에서 폐동맥 육종으로 진단되었다.

• Tuberculosis and Respiratory Diseases
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• 제72권2호
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• pp.218-222
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• 2012

Pulmonary artery intimal sarcoma is a rare tumor with no characteristic symptoms. It is frequently misdiagnosed as pulmonary embolism. We report a case of pulmonary artery intimal sarcoma in a 48-year-old man with dyspnea, cough and blood-tinged sputum. He was initially suspected and treated as a pulmonary embolism. Computed tomography of the chest showed filling defects occupying the entire luminal diameter of the right and left pulmonary artery as well as extraluminal extension of the intraluminal mass. Surgical resection of the tumor confirmed pulmonary artery intimal sarcoma. After surgery, he received 8 cycles of combined chemotherapy consisting of doxorubicin and ifosfamide. After 8 cycles, Computed tomography of the chest showed interval regression of the residual tumor. Radiotherapy was done as total 6,000 cGy for 5 weeks, following the 8th chemotherapy. The patient's condition was successfully stabilized with chemotherapy and radiotherapy.

• 대한핵의학회지
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• 제37권5호
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• pp.336-339
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• 2003

Purpose: A 6-year-old boy with osteogenic sarcoma of the left humerus underwent bone scintigraphy. Tc-99m MDP was accumulated not only in the primary tumor but also in the osseous and extraosseous (pulmonary and pericardial) metastases. Osteogenic sarcoma directly produces osteoid, both in the primary and metastatic lesions. Tc-99m MDP is avidly taken up by tumor osteoid. At initial presentation, only 2% of cases have both pulmonary and osseous metastases. The patient had osseous, pulmonary, and pericardial metastases at presentation. This case presents that increased uptakes of Tc-99m MDP by the primary and metastatic tumor were demonstrated on bone scintigraphy at presentation.

• Tuberculosis and Respiratory Diseases
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• 제70권5호
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• pp.423-427
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• 2011

Epithelioid sarcomas are rare soft tissue sarcomas with a high tumor grade and high local recurrence and metastasis rates. Although the lung is the most common site of metastasis, endobronchial metastasis hasn't been reported yet. We now report a case of epithelioid sarcoma with endobronchial metastasis. A 28-year-old man had recurrent pneumothorax and underwent wedge resection. He presented at our hospital with hemoptysis, dyspnea, and chest pain. Chest computed tomography revealed left pneumothorax, multiple lung nodules and endobronchial lesions at the right lower basal lobe. Bronchoscopy showed a hemorrhagic mass obstructing the bronchus of the right lower basal lobe. Magnetic resonance imaging revealed multiple nodular lesions in the left thigh muscles. The bronchoscopic biopsy of the endobronchial lesion and the muscle biopsy of the thigh showed the same feature epithelioid sarcoma. This is the first case report of an epithelioid sarcoma with endobronchial metastasis that was diagnosed by bronchoscopic biopsy.