A Case of Intimal Sarcoma of the Pulmonary Artery Treated with Chemoradiotherapy

Hirose, Takashi; Ishikawa, Noboru; Hamada, Kenji; Inagaki, Tomoko; Kusumoto, Shoichi; Shirai, Tomoyuki; Okuda, K; Ohnishi, Tsukasa; Kadokura, Mitsutaka; Adachi, Mitsuru

doi:10.2169/internalmedicine.48.1560

Cited by 31 publications

(24 citation statements)

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“…Only surgical resection prolongs survival times (2,3). The effectiveness of radiation therapy and chemotherapy remains controversial although some studies have reported that both therapies may be effective (5)(6)(7). The use of these therapies after surgical treatment has been found to prolong survival time compared with surgery alone (14,15).…”

Section: Discussionmentioning

confidence: 99%

“…However, no standard regimens for the use of these chemotherapeutic agents have been established. Some reports have shown a potential usefulness for radiation therapy (7,20). Considering the side effects of chemotherapy, including bone marrow suppression, we first selected radiation therapy to prevent tumor recurrence in the present case.…”

Section: Discussionmentioning

confidence: 99%

“…Surgical resection decreases the clinical symptoms and improves the survival time of patients (3,4). The effectiveness of radiation therapy and chemotherapy remains controversial, although some studies have reported that both therapies may be effective (5)(6)(7). We herein describe the case of a 39-year-old woman with a pulmonary embolism caused by intimal sarcoma of the pulmonary artery.…”

Section: Introductionmentioning

confidence: 96%

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Pulmonary Embolism Caused by Intimal Sarcoma of the Pulmonary Artery

et al. 2012

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We herein report the case of a 39-year-old woman with a pulmonary embolism caused by intimal sarcoma of the pulmonary artery. She presented with shortness of breath and leg edema. Computed tomography showed a low density area that extended from the main pulmonary artery to the bilateral pulmonary arteries. We diagnosed her to have a pulmonary thromboembolism. The thrombosis did not decrease after the administration of anti-coagulant therapy, and she underwent resection of the thrombotic tissue. Histopathologically, the surgical specimen was not found to be thrombotic tissue but rather an intimal sarcoma of the pulmonary artery. After undergoing surgery, she received radiation therapy and chemotherapy; however, she died 31 months after being diagnosed.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 96%

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Pulmonary Embolism Caused by Intimal Sarcoma of the Pulmonary Artery

et al. 2012

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“…However, preoperative severity of right heart failure seemstobeapredictorforpatientoutcome.Theroleofneoadjuvant or adjuvant chemotherapy or radiation treatment strategies needs to be evaluated. Some reports have shown beneficial effects [7,[11][12][13]. Anthracycline-and ifosfamidebased chemotherapies are available options [7].…”

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confidence: 99%

Intimal Pulmonary Artery Sarcoma Presenting as Severe Dyspnea and Right Heart Insufficiency

et al. 2010

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Background: Pulmonary artery sarcoma is a rare tumor with a poor prognosis. Case Report: We report the case of a 64-year-old man with an intimal pulmonary artery sarcoma presenting with severe high oxygen flow-demanding dyspnea and weight loss of 12 kg in the last 6 months. On echocardiography, right heart insufficiency, markedly elevated right ventricular pressure, a pressure gradient along the right outflow tract, and a tumor mass adherent to the wall of the truncus pulmonalis were detected. The tentative diagnosis by echocardiographic findings was pulmonary artery sarcoma. Computed tomography of the thorax and 18-fluorodeoxyglucose positron emission tomography showed an advanced local tumor manifestation. Surgical resection of the tumor to improve hemodynamics confirmed the diagnosis. Conclusions: Pulmonary artery sarcoma should be considered as a rare differential diagnosis in patients with dyspnea due to right heart failure, particular in the case of additional weight loss, and echocardiographic examination is a useful first diagnostic approach in establishing the diagnosis.

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“…However, in isolated cases chemotherapy or radiotherapy seems to be effective against pulmonary artery sarcoma [3,8,12]. Adjuvant radiochemotherapy after surgical treatment seems to be superior to surgery alone [4], from a retrospective point of view.…”

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Intimal sarcoma of the pulmonary artery with unusual findings: a case report

Hoiczyk¹,

Iliodromitis

Bauer³

et al. 2012

Clin Res Cardiol

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Sirs:Intima sarcoma is a rare tumor often localized in the pulmonary artery. Previous descriptions can be found dating all the way back to 1923 [9].This tumor's evolvement is characterized by endoluminal growth with later vessel obstruction. This type of tumor can also originate from the heart valves and the right ventricular outflow tract. Peripheral thromboembolism is a common concomitant finding, often leading to the false diagnosis of chronic thromboembolism.The prognosis of inoperable patients with extensive disease is very poor with a median survival of 6 weeks [7]. Due to the rareness of this tumor, prospective data regarding the benefit of neoadjuvant or adjuvant chemo-or radiotherapy are not available.We present the case of a 76-year-old female with persisting shortness of breath over 6 months. The patient has a smoking history of more than 30-pack years. On physical examination, the patient exhibited tachypnea, with 21 breaths per minute, a regular pulse at 92 beats per minute and a blood pressure of 123/80 mmHg. Cardiac auscultation revealed a 3/6 systolic murmur over the second intercostal space at the left sternal border. Further physical examination was unremarkable.Blood count and chemistry profile were within normal range, including the level of brain natriuretic peptide. DDimers were at 0.5 (normal range\0.55 mg/l); CRP was at 1.1 (normal range \0.5 mg/dl).The transthoracic echocardiography showed a hypertrophic free wall of the right ventricle with a mild dilation of the right atrium (area was assessed as 2.2 cm 2 ). The color Doppler at the level of the tricuspidal annulus revealed a moderate tricuspidal regurgitation and the systolic pulmonary arterial pressure was assessed with continuous wave Doppler at 78 mmHg plus central venous pressure. The left ventricular ejection fraction was 70% and the transesophageal echocardiography showed an uncommon structure on the wall of the right pulmonary artery (Fig. 1). All echocardiographic measurements of the right ventricle were made in line with similar studies for evaluation of pulmonary artery hypertension [2].CT diagnostics showed no sign of peripheral pulmonary embolism; however, it revealed a mural thrombus in the right pulmonary artery stem, which seemed to arise from the pulmonary valve (Fig. 1b). Left and right heart catheterization and pulmonary angiogram followed for further diagnostics. A nearly 100% stenosis across the right pulmonary artery trunk was shown. We initially hypothesized the existence of a massive mural thrombus and a concomitant chronic thromboembolic disease (CTEPH) (Fig. 2. The right catheterization recorded a systolic pressure of 78 mmHg proximal and 36 mmHg distal to the stenosis. The same systolic parameter was recorded from the hemodynamic measurements in the left pulmonary artery. The wedge pressure was 3 mmHg.IVUS was performed and confirmed an occluding mass (Fig. 3). The hemodynamic results revealed an isolated

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A Case of Intimal Sarcoma of the Pulmonary Artery Treated with Chemoradiotherapy

Abstract: We report on a 45-year-old

Cited by 31 publications

References 16 publications

Pulmonary Embolism Caused by Intimal Sarcoma of the Pulmonary Artery

Pulmonary Embolism Caused by Intimal Sarcoma of the Pulmonary Artery

Intimal Pulmonary Artery Sarcoma Presenting as Severe Dyspnea and Right Heart Insufficiency

Intimal sarcoma of the pulmonary artery with unusual findings: a case report

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