Pulmonary artery sarcoma mimicking chronic thromboembolic disease: Computed tomography and magnetic resonance imaging findings

Kauczor, Hans‐Ulrich; Schwickert, Heidi C.; Mayer, E.; Kersjes, W.; Moll, Roland; Schweden, F

doi:10.1007/bf00571532

Cited by 106 publications

(44 citation statements)

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“…Several reports suggest that CT and MRI may be the most useful modalities for differentiation between tumor and thrombotic material. 6 Therefore, the mass appeared to be a tumor rather than a thrombus and most likely malignant. In particular, heterogenous enhancement on MRI after application of gadolinium-diethylene-triamine-pentaacetic acid is characteristic of a vascularized tumor.…”

Section: Discussionmentioning

confidence: 99%

Surgical Treatment for Primary Cardiac Leiomyosarcoma Causing Right Ventricular Outflow Obstruction

Ishikawa

Takanashi

Mihara

et al. 2005

Circ J

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rimary cardiac leiomyosarcoma is extremely rare, constituting less than 0.25% of all cardiac tumors, 1 but it has a lethal prognosis even if surgical resection, with or without adjuvant therapy, is performed. 2,3 Only a few cases of surgical resection of cardiac leiomyosarcoma have been reported. 4 We present a case of surgical resection of a primary cardiac leiomyosarcoma involving the pulmonary valve, which was reconstructed with a bioprosthetic valve and Xenomedica patch. Case ReportA 55-year-man was admitted to another hospital because of dry cough and exertional breathlessness that had rapidly worsened over the previous 1-2 weeks. He had no remarkable medical history. A computed tomographic (CT) examination of the chest revealed a filling defect that almost completely obstructed the main pulmonary arterial trunk.A diagnosis of pulmonary thromboembolism was assumed, and the patient was treated with catheter-directed thrombolysis therapy over 3 days. However, his dyspnea and shortness of breath worsened despite an optimal regimen of thrombolytic and anticoagulation therapy. The patient was referred to us for further evaluation and possible surgical management of the presumed pulmonary thromboembolism.Upon admission, laboratory results showed elevated lactate dehydrogenase (785 U/L) and liver enzymes (AST Circulation Journal Vol.69, January 2005 49 U/L, ALT 95 U/L). Chest X-ray showed mild cardiomegaly with a cardiothoracic ratio of 52%. The patient underwent magnetic resonance imaging (MRI) of the chest, which revealed a solid mass at the pulmonary valve, measuring approximately 50×40×30 mm with low signal intensity on T1-weighted image and increased signal intensity on T2-weighted image, that appeared to be invading the main pulmonary arterial trunk (Fig 1a,b). There was no evidence of distal thromboembolism in either of the pulmonary arteries. Because the mass appeared to be a tumor rather than a thrombus, the possibility of malignancy was raised. Transthoracic echocardiography disclosed a large mass involving the pulmonary valve and extending to the main pulmonary arterial trunk, enlargement of the right ventricle (end-diastolic diameter, 52 mm), and displacement of the ventricular septum toward the left ventricle. The mass almost completely obstructed the main pulmonary arterial trunk (Fig 1c), and right ventricular failure was suspected. The patient was scheduled to undergo surgery for tumor extirpation and histological evaluation of the mass.At surgery, a hard mass was palpated in the slightly dilated main pulmonary arterial trunk. Conventional cardiopulmonary bypass was established with ascending aortic perfusion and bicaval drainage through a median sternotomy. After the heart was arrested, the main pulmonary arterial trunk was incised longitudinally from just above the pulmonary valve to the bifurcation, whereupon a whitish tumor originating from the pulmonary valve was found to have almost completely blocked the lumen of the main pulmonary arterial trunk and invaded its anterior wall. We also o...

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Section: Discussionmentioning

confidence: 99%

Surgical Treatment for Primary Cardiac Leiomyosarcoma Causing Right Ventricular Outflow Obstruction

Ishikawa

Takanashi

Mihara

et al. 2005

Circ J

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“…In this context, computer-assisted tomography and/or magnetic resonance imaging, particularly with gadolinium enhancement, may be helpful in detecting intravascular images and hilar nodules or determining tumoural vascularity [1,5]. Therefore, the importance of neither method should be underestimated.…”

Section: Discussionmentioning

confidence: 99%

Primary pulmonary artery sarcoma resembling chronic thromboembolic pulmonary disease

Kaplinsky

Favaloro²,

Pombo³

et al. 2000

Eur Respir J

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Two cases of primary pulmonary artery sarcoma resembling chronic thromboembolic disease features are presented. Tumour identification was achieved after pulmonarv thromboendarterectomy, which was indicated by documentation of a prothrombotic state in both patients. A doubtful history of pulmonary emboli or deep venous thrombosis should alert medical personnel to the possible presence of a primary pulmonary artery sarcoma. Advanced imaging methods such as gadolinium-enhanced magnetic resonance imaging could be useful in considering pulmonary thromboendarterectomy. If a tumour is detected, its surgical resection should be considered with caution, taking into account the poor survival results. Invasion of the adventitia or the right ventricle, as documented in the present cases, is unusual. As far as the present authors know, this is the first report of this kind of tumour and its coexistence with an activated protein C resistance state and type II heparin-induced thrombocytopenia.

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“…Ventilasyonla uyumsuz bir ya da daha fazla segmental ya da büyük perfüzyon defektinin varlığı KTEPH düşündürür (23). Bu bulgular varlığında en olası tanı KTEPH olmakla birlikte pulmoner kapiller hemanjiyomatozis, pulmoner venooklüzif hastalık, pulmoner vaskülit, fibrozan mediyastinit ya da pulmoner arterlerin sarkomunda da benzer bulguların görüle-bileceği akılda tutulmalıdır (17,(24)(25)(26)(27). Bu nedenle vasküler anormalliği tanımlamak ve tanıyı doğrulamak için başka görün-tüleme yöntemlerine ihtiyaç vardır.…”

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Chronic thromboembolic pulmonary hypertension: diagnosis, medical therapy and monitoring

Gölbaşı¹

2010

Anadolu Kardiyol Derg

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Chronic thromboembolic pulmonary hypertension (CTEPH) is caused by obstruction of the large pulmonary arteries by acute and recurrent pulmonary emboli with subsequent organization of these blood clots. Because of a lack of clinically evident acute-pulmonary embolism episodes in >50% of patients, the diagnosis of CTEPH can be difficult. Lung ventilation/perfusion scintigraphy showing segmentary mismatched perfusion defects is the best diagnostic tool to detect CTEPH. Although pulmonary endarterectomy is considered the treatment of choice for patients with CTEPH, it can not be performed in a substantial proportion of patients. Medical therapies have been increasingly used in subcategories of CTEPH patients. (Anadolu Kardiyol Derg 2010; 10: Suppl 2; 56-60) Key words: Pulmonary embolism, pulmonary hypertension, chronic thromboembolic pulmonary hypertension ÖZETKronik tromboembolik pulmoner hipertansiyon (KTEPH), akut ve yineleyen pulmoner emboliler ve bunların organize olması sonucu büyük pulmoner arterlerin obstrüksiyonu ile oluşur. Hastaların %50'den fazlasında klinik olarak belirti veren akut pulmoner emboli atağının bulunmayışı sebebiyle KTEPH tanısı zor olabilir. KTEPH'nun tespitinde ventilasyonla uyumsuz segmental perfüzyon defekti bulunan akciğer ventilasyon/perfüzyon sintigrafisi en iyi tanı aracıdır. KTEPH'lu hastaların tedavisinde pulmoner endarterektomi (PEA) en iyi tedavi seçeneği olarak görülmekle birlikte, hastaların önemli bir bölümünde yapılamayabilir. KTEPH'lu hastaların bazı alt gruplarında tıbbi tedavi giderek daha artan oranda kullanılmaktadır.

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Pulmonary artery sarcoma mimicking chronic thromboembolic disease: Computed tomography and magnetic resonance imaging findings

Abstract: CT and MRI provide complementary findings suggesting advanced pulmonary artery sarcoma instead of chronic thromboembolism.

Cited by 106 publications

References 20 publications

Surgical Treatment for Primary Cardiac Leiomyosarcoma Causing Right Ventricular Outflow Obstruction

Surgical Treatment for Primary Cardiac Leiomyosarcoma Causing Right Ventricular Outflow Obstruction

Primary pulmonary artery sarcoma resembling chronic thromboembolic pulmonary disease

Chronic thromboembolic pulmonary hypertension: diagnosis, medical therapy and monitoring

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