Pulmonary Embolism Caused by Intimal Sarcoma of the Pulmonary Artery

Yamamoto, Kei; Nozue, Tsuyoshi; Tsuchida, Masayuki; Iwaki, Taku; Nagamine, Hiroshi; Yasuda, Tamotsu; Kawase, Hiroshi; Matsushita, Kazunobu; Michishita, Ichiro

doi:10.2169/internalmedicine.51.7558

Cited by 9 publications

(8 citation statements)

References 15 publications

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“…Most cases are discovered incidentally at autopsy or on surgical specimens; therefore, their true incidence is unknown [4,15]. Aortic intimal sarcoma occur less frequently than those of the pulmonary arteries, which is confirmed in our series in which no primary aortic tumors were seen, although in 2 cases the primary site of the disease could not be identified [7].…”

Section: Discussionsupporting

confidence: 66%

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Single-Center Experience with Intimal Sarcoma, an Ultra-Orphan, Commonly Fatal Mesenchymal Malignancy

et al. 2017

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Background: Intimal sarcoma is a rare malignancy that, clinically and radiographically, often mimics pulmonary embolism. The intravascular tumor tends to disseminate rapidly and metastases can be present at first diagnosis. Methods: We reviewed all cases of intimal sarcoma that were diagnosed, treated and followed at the University Hospitals Leuven between April 2006 and April 2016. Results: We identified 13 patients with a median age of 51 years. In 6 patients initial findings were suggestive of thromboembolic disease. Platelet-derived growth factor receptor α (PDGFRA) amplification was the most prevalent molecular finding, present in 11 patients. The MDM2 gene was amplified in 9 cases, and the EGFR gene in 3 patients. The median overall survival was 13 months. 11 patients underwent surgery. In 5 cases with inoperable and/or metastatic disease chemotherapy was given. Treatment with imatinib was initiated in 4 patients. Conclusions: Intimal sarcoma is an extremely rare and aggressive malignancy that has a very poor prognosis. Mimicking thromboembolic disease, diagnosis and treatment can be delayed. Surgery is the mainstay of treatment but is seldom curative. The disease is highly resistant to cytotoxic and targeted treatment. Given the fact that intimal sarcoma commonly expresses more than 1 molecular target, combination therapy might be an option, although toxicity may be a limitation.

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Section: Discussionsupporting

confidence: 66%

“…The clinical features, however, are slightly different. While pulmonary artery intimal sarcoma can result in dyspnea, chest pain, hemoptysis or cough, the aortic counterpart most commonly presents with embolic symptoms such as claudication, skin necrosis and abdominal angina [7,15].…”

Section: Discussionmentioning

confidence: 99%

Single-Center Experience with Intimal Sarcoma, an Ultra-Orphan, Commonly Fatal Mesenchymal Malignancy

et al. 2017

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“…The clinical symptoms of PAIS are not specific; most often they include dyspnoea, haemoptysis, chest pain, and cough. The initially suspected clinical diagnosis is often pulmonary embolism [ 5 ], making the diagnosis of PAIS often too late, at which time the disease is locally advanced. The pathological diagnosis is generally made on a surgical biopsy or on the surgical resection specimen, like with our patient.…”

Section: Discussionmentioning

confidence: 99%

Metastatic Intimal Sarcoma of the Pulmonary Artery Sensitive to Carboplatin-Vinorelbine Chemotherapy: Case Report and Literature Review

Cantaloube¹,

Moureau-Zabotto²,

Mescam³

et al. 2018

Case Rep Oncol

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Pulmonary artery intimal sarcoma (PAIS) is a very rare tumour with a very poor prognosis. In advanced stages, chemotherapy and radiotherapy are poorly efficient, and no standard chemotherapy guideline is currently available. Here, we report on a 37-year-old woman with PAIS initially treated with surgical resection who developed metastatic relapse refractory to anthracycline-based chemotherapy, then trabectedin, then pazopanib. The patient was then given carboplatin-vinorelbine chemotherapy. The treatment was well tolerated, and, rapidly, a CT scan showed an objective response that lasted 8 months despite the 4th therapeutic line. We review the literature and show that our case is the second one that provides evidence of the efficacy of platinum-vinorelbine regimens in this aggressive tumour.

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“…There are no specific guidelines for the management of patients with pulmonary artery intimal sarcoma . The rarity of this diagnosis made it difficult to standardize the therapy.…”

Section: Discussionmentioning

confidence: 99%

“…Distant metastases occur in 16–25% of the cases . It can metastasize to the brain, pancreas, adrenal glands, and lungs . The mean survival is 12–18 months .…”

Section: Discussionmentioning

confidence: 99%

An intimal sarcoma of pulmonary artery mimicking pulmonary embolism: a case report and literature review

Liu

Hou

Wang

et al. 2017

Respirology Case Reports

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Pulmonary artery intimal sarcoma is a highly aggressive disease, and is most often misdiagnosed as pulmonary thromboembolism (PTE) due to the similar clinical symptoms and its rarity, which leads to the use of inappropriate treatments such as prolonged anticoagulant therapy. We reported a case of pulmonary artery intimal sarcoma in a patient who was misdiagnosed as having PTE. Pathology after surgery confirmed malignant disease. We concluded that when a patient presents with mild clinical manifestations yet with strong imaging manifestations, pulmonary artery malignancy should be suspected.

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Pulmonary Embolism Caused by Intimal Sarcoma of the Pulmonary Artery

Cited by 9 publications

References 15 publications

Single-Center Experience with Intimal Sarcoma, an Ultra-Orphan, Commonly Fatal Mesenchymal Malignancy

Single-Center Experience with Intimal Sarcoma, an Ultra-Orphan, Commonly Fatal Mesenchymal Malignancy

Metastatic Intimal Sarcoma of the Pulmonary Artery Sensitive to Carboplatin-Vinorelbine Chemotherapy: Case Report and Literature Review

An intimal sarcoma of pulmonary artery mimicking pulmonary embolism: a case report and literature review

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