Leiomyosarcoma of the pulmonary artery — a diagnostic chameleon

Hoffmeier, A.; Semik, M; Fallenberg, Eva Maria; Hh, Scheld

doi:10.1016/s1010-7940(01)00939-3

Cited by 28 publications

(27 citation statements)

References 9 publications

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“…Echocardiograms reveal pulmonary hypertension and right ventricular overload [60]. Chest X-rays may be normal, or may reveal a pulmonary nodule, hilar mass, PA enlargement, decreased vascularity, or parenchymal infiltrates.…”

Section: Pa Sarcoma (Pas)mentioning

confidence: 99%

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Primary cardiac sarcoma

Shanmugam¹

2006

European Journal of Cardio-Thoracic Surgery

132

157

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Primary malignant lesions of the heart are rare. Although myxomas have been extensively described, there is a paucity of large studies on non-myxomatous cardiac tumours. On the other hand, there are several case reports on specific histopathological variants, in small numbers. Consequently there exists no consensus on therapeutic modalities for cardiac sarcomas. The prognosis for these lesions remains dismal, despite the enhanced diagnostic ability of newer technology. The reasons for the dismal prognosis are (1) the advanced tumour stage at presentation, (2) non-specific symptomatolgy, (3) insufficient awareness of these lesions, due to their rarity, (4) delayed diagnosis and/or misdiagnosis, which leads to (5) advanced tumour stage at presentation. Thus a vicious cycle is created. This article addresses these issues, deals with the surgically relevant modes of presentation, rather than the histopathology, and reviews the diagnosis and management options for the various sarcomas, categorized by the site and extent of cardiac involvement. Clinicians should be familiar with the presentation of these tumours and have a high index of suspicion, since the potential for long-term survival following resection does exist. Wide surgical resection remains the cornerstone of sarcoma therapy. Complete characterization of tumour extent using echocardiography and CT/MRI is mandatory to achieve this goal. Radical resections such as 'bench surgery' and transplantation may reduce local recurrence, but the risk of metastatic disease remains. The clinical experience with such approaches is limited. The role of adjuvant therapy is not yet established. In no other field of cardiac surgery would a multidisciplinary approach be more useful, in achieving cure or long-term palliation.

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Section: Pa Sarcoma (Pas)mentioning

confidence: 99%

“…Angiography shows PA occlusion and suggests pulmonary embolism [60,61]. A pedunculated mobile lesion suggests PAS rather than thromboembolism, although distinction is difficult with sessile tumours, covered with thrombus.…”

Section: Pa Sarcoma (Pas)mentioning

confidence: 99%

Primary cardiac sarcoma

Shanmugam¹

2006

European Journal of Cardio-Thoracic Surgery

132

157

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“…The first case of a primary sarcoma originating from the pulmonary artery was described by Mandelstamm [3]. Since that time, approximately 150 cases have been reported in the scientific literature [4]. Leiomyosarcomas of the great vessels are most often observed in the inferior vena cava [5], and have only rarely been described in the pulmonary artery.…”

Section: Discussionmentioning

confidence: 99%

“…Back pain [4] or chest pain [8] and syncope [9] are less common presenting symptoms. In one case, the cause of death in a 67-year-old female patient was ascribed to rightsided heart failure secondary to pulmonary artery stenosis caused by a locally invasive pulmonary artery leiomyosarcoma [10].…”

Section: Discussionmentioning

confidence: 99%

Pulmonary artery leiomyosarcoma: an unusual cause of shortness of breath

2009

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“…The corresponding author has the right to grant on behalf of all authors and does grant on behalf of all authors, an exclusive licence (or non-exclusive for government employees) on a worldwide basis to the publisher. [15] 37 Male 11 Dizziness Dumont et al [7] 42 Female 11 Chest pain Marchetti et al [21] 44 Male 24 Syncope Yamada et al [34] 49 Female 21 -Minakata et al [22] 57 Male 2 -Chan et al [5] 57 Female 12 -Hoffmeier et al [11] 60 Female 11 -Yamada et al [34] 65 Male 12 -Croitoru et al [6] 75 Male 36 Chest pain -Data unavailable.…”

Section: Competing Interests/copyrightmentioning

confidence: 99%

Vascular smooth muscle tumors: 13 cases and a review of the literature

et al. 2011

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Vascular smooth muscle tumors, benign or malignant, are rare and encompass a variety of neoplastic lesions characterized histologically by their similarity to adult smooth muscle tissue. Leiomyomatosis, a benign neoplasm, primarily arising from within the uterine and extrauterine venous systems, and leiomyosarcoma, a malignant neoplasm arising predominantly in larger veins, while relatively rare, are nevertheless a cause of significant morbidity and mortality. We present a review of case of leiomyomatosis and leiomyosarcomas seen over a 35-month period, and an 18-year review of the literature. All tissues were fixed in 10% formalin, photographed, examined, and gross features described. Tissue samples were processed for light microscopy and stained with hematoxylin-eosin. The clinical records were reviewed. Eight cases of intravascular leiomyomatoses and five cases of leiomyosarcomas are presented in this series. Gross findings of resected intravascular leiomyomatosis were remarkably similar, although tumor size varied greatly between patients. Histologically, the tumor masses were similar. Gross and histological analysis of leiomyosarcoma masses revealed tumors of varying grades of malignancy. Vascular smooth muscle tumors that extend through or directly involve the vasculature are rare and difficult to diagnose and manage. Surgical resection remains the most effective treatment, although chemotherapy in isolated cases has been shown to have a positive effect. Appropriate interpretation and diagnosis are crucial for effective management.

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Leiomyosarcoma of the pulmonary artery — a diagnostic chameleon

Cited by 28 publications

References 9 publications

Primary cardiac sarcoma

Primary cardiac sarcoma

Pulmonary artery leiomyosarcoma: an unusual cause of shortness of breath

Vascular smooth muscle tumors: 13 cases and a review of the literature

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