Sarcoma of the pulmonary artery: Report of two cases and a review of the literature

Ramp, Uwe; Gerharz, Claus Dieter; Iversen, Stein; Schweden, F; Steppling, H; Gabbert, Helmut E.

doi:10.1007/bf01225272

Cited by 27 publications

(9 citation statements)

References 25 publications

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“…Due to slow progress it may cause few symptoms, but sudden death will occur when near total occlusion by the tumor and/or an additional thrombus leads to acute right ventricular failure. This is corroborated by the fact that 60% of the reported cases were identified by autopsy [2].…”

Section: Commentsupporting

confidence: 80%

Sarcoma of the pulmonary trunk and the main pulmonary arteries

Huwer

Özbek

Waldmann

et al. 2008

Journal of Surgical Oncology

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We report on a sarcoma of the central pulmonary arteries. Surgical therapy consisted in replacing both main pulmonary arteries and the pulmonary trunk including the pulmonary valve. Six months later a left-sided pneumonectomy had to be performed due to an intravascular tumor. Fifteen months after first resection treatment, recurrent tumors of the right pulmonary artery and the right ventricle were resected. Two years after the first operation the patient has no detectable tumor.

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Section: Commentsupporting

confidence: 80%

Sarcoma of the pulmonary trunk and the main pulmonary arteries

Huwer

Özbek

Waldmann

et al. 2008

Journal of Surgical Oncology

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“…With some exceptions (Paulsen and Egeblad 1983;Nonomura et al 1988;McGlennen et al 1989), electron microscopic examinations have been carried out with poorly preserved autopsy material. Immunohistochemical findings have been reported in 18 cases altogether (Nonomura et al 1988;McGlennen et al 1989;Kaiser et al 1990;Nerlich et al 1990;Hiroshima et al 1992;Ramp et al 1992;Burke and Virmani 1993).…”

Section: Introductionmentioning

confidence: 97%

Sarcoma of the pulmonary artery: report of four cases with electron microscopic and immunohistochemical examinations, and review of the literature

Johansson

Carlén

1994

Vichows Archiv A Pathol Anat

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Herein we report the clinicopathological features of four cases of pulmonary artery sarcoma that appeared at our institution during a period of 30 years. The patients, 2 males and 2 females, were 50-62 years old. Tumour was found in the pulmonary trunk and right pulmonary artery in all cases, in the pulmonary valve and left pulmonary artery in three of the four cases, and in the right ventricular outflow tract in one case. There was direct extension or metastases to the lungs in two cases, the heart in one case, mediastinum or lymph nodes in two cases and the pleura in one case. Ultrastructural examination in one case revealed cells with features of smooth muscle cells and myofibroblasts. Immunohistochemical examination of three cases gave the following results: vimentin and smooth muscle specific actin was positive in all three cases, desmin in one case and cytokeratin in one case. No positivity was found for Factor VIII. This and other studies indicate that histologically most pulmonary artery sarcomas are leiomyosarcomas or "undifferentiated spindle cell sarcomas". Immunohistochemical and ultrastructural examinations favour an origin from myofibroblasts, probably derived from multipotent (undifferentiated) cells in the wall of the vessel. Most lesions show extensive intrathoracic growth although they rarely metastasize outside the thoracic cavity. They have a poor prognosis although some cases are currently being diagnosed during life.

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“…Tumors of the pulmonary artery are furthermore rare tumors; in fact, only approximately 200 cases have been reported in the literature. Since lethal events frequently occur in patients with pulmonary artery tumor when initial symptoms are present, 60% of these cases have previously been diagnosed by autopsy [5]. More recently, advances in imaging technologies have allowed more successful antemortem diagnosis [2].…”

Section: Discussionmentioning

confidence: 99%

Successful Chemoradiotherapy for Undifferentiated Malignant Neoplasm Arising from the Left Pulmonary Artery

Kumagai¹,

Nio²,

Okumura³

et al. 2014

Case Rep Oncol

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Undifferentiated malignant neoplasms, which occur primarily in the pulmonary artery, are extremely rare and associated with poor outcomes as there is no effective therapy. A 67-year-old woman visited our hospital with complaints of dry cough and dyspnea on exertion. A contrast-enhanced chest computed tomography revealed an intravascular tumor obstructing the left pulmonary artery and a pedunculated lesion extending to the main and right pulmonary artery. Multiple metastases in the lung, bones and bilateral adrenal glands were identified by fluorodeoxyglucose-positron emission tomography. A small sample was obtained by catheter aspiration biopsy of the intravascular tumor, and examination revealed undifferentiated small atypical cells. The tumor was diagnosed as an undifferentiated neoplasm arising from the pulmonary artery based on immunohistochemical findings, including the absence of expressions of organ-specific markers. Systemic chemotherapy (paclitaxel and carboplatin) and concurrent radiation were performed as treatment for the primary tumor. Marked shrinkage of the intravascular tumor was achieved, and no serious adverse events were observed during therapy. Chemotherapy was continued for 5 months, but the patient died because of tumor progression 9 months after the initial diagnosis. Chemoradiotherapy has efficacy against undifferentiated neoplasm of the pulmonary artery.

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Sarcoma of the pulmonary artery: Report of two cases and a review of the literature

Cited by 27 publications

References 25 publications

Sarcoma of the pulmonary trunk and the main pulmonary arteries

Sarcoma of the pulmonary trunk and the main pulmonary arteries

Sarcoma of the pulmonary artery: report of four cases with electron microscopic and immunohistochemical examinations, and review of the literature

Successful Chemoradiotherapy for Undifferentiated Malignant Neoplasm Arising from the Left Pulmonary Artery

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