Surgical treatment of pulmonary artery sarcoma

Mayer, Eckhard; Kriegsmann, Jörg; Gäumann, Andreas; Kauczor, Hans Ulrich; Dahm, Manfred; Hake, U; Schmid, Franz X.; Oelert, H.

doi:10.1067/mtc.2001.111423

Cited by 102 publications

(75 citation statements)

References 16 publications

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“…Since intimal PAS is more common than intramural PAS, PAS is often referred to as intimal sarcoma (4). PAS often occurs in the dorsal region of the main pulmonary artery, mostly with a polypoid or finger-like form, an extends to the bifurcation of the main pulmonary artery, and the left and right pulmonary arteries (22). Certain tumors that involve the pulmonary valve may also simultaneously involve or spread to the outflow tract of the right ventricle (3).…”

Section: Discussionmentioning

confidence: 99%

Clinicopathological and immunohistochemical features of pulmonary artery sarcoma: A report of three cases and review of the literature

et al. 2016

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Abstract. Pulmonary artery sarcoma (PAS) is an extremely rare and highly malignant tumor that originates in the pulmonary artery. The majority of reported cases of PAS are confirmed by pathological examination subsequent to surgery or by autopsy. The present study reports the clinicopathological characteristics and immunohistochemical phenotypes of three cases of PAS, and aims to facilitate the identification of this lethal disease. In the present study, the data from clinical, histopathological and immunohistochemical examinations of three patients with PAS, whose diagnoses were confirmed by surgical biopsy conducted at the Beijing Anzhen Hospital (Beijing, China) between 2008 and 2012, were retrospectively analyzed. The patients (two women and one man; average age, 41.3 years old) presented with dyspnea on exertion. In addition, two of the patients experienced chest tightness, and one patient experienced intermittent syncope. Computed tomography pulmonary angiography revealed that two of the patients possessed a filling defect in the main, left and right pulmonary arteries, and one patient possessed a filling defect in the right upper pulmonary artery. Macroscopically, the PAS appeared as a mucoid intraluminal or nodular sessile mass spreading along the pulmonary artery. Microscopically, the tumor consisted of spindle cells with fascicular and storiform patterns, and was accompanied by necrosis and stromal myxoid changes. Immunohistochemically, vimentin, desmin and cluster of differentiation 34 were highly expressed in the patient that was diagnosed with intimal sarcoma, while vimentin and α-smooth muscle actin were highly expressed in the other two patients, who were diagnosed with leiomyosarcoma. PAS is often misdiagnosed due to nonspecific clinical manifestations and radiological features. Therefore, the diagnosis of PAS should be based on typical morphological features and immunohistochemical analysis of the tumor tissue. IntroductionPulmonary artery sarcoma (PAS) is an extremely rare and highly malignant tumor that originates in the pulmonary artery (1). It has an incidence rate of 0.001-0.030% (2). Since 1923, when the first case of PAS was reported by Mandelstamm (3), ≤300 cases have been reported to date worldwide (4-7). The etiology of PAS is unknown, and this disease has a poor prognosis (5). Early diagnosis followed by radical surgical resection constitutes the only chance of survival for patients with PAS (5), and patients have an average survival time of ~1.5 months without surgical treatment (8). However, due to the rare and nonspecific clinical manifestations and imaging findings, PAS is frequently misdiagnosed as various pulmonary thromboembolic diseases, including pulmonary thromboembolism (PTE) or chronic thromboembolic pulmonary hypertension (CTEPH), and the majority of reported PAS cases are confirmed by pathological examination subsequent to surgery or by autopsy (2,9,10). The present study reports three cases of PAS that were initially misdiagnosed as PTE or CTEPH, and were later identifi...

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Section: Discussionmentioning

confidence: 99%

Clinicopathological and immunohistochemical features of pulmonary artery sarcoma: A report of three cases and review of the literature

et al. 2016

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“…Early radical surgical resection with cardiopulmonary bypass is the only strategy for potential cure (3). With this strategy, it is important to obtain a tumor-free margin, thus as much as possible of the pulmonary artery and the tumor is resected, and reconstruction with a cryopreserved pulmonary artery allograft and/or pneumonectomy can also be undertaken if necessary (4,11).…”

Section: Discussionmentioning

confidence: 99%

“…Diagnosis is difficult as the clinical presentation is similar to that of pulmonary thromboembolism. Aggressive surgical treatment is considered the only strategy for prolonged survival (1)(2)(3)(4). Herein, we present a patient with a pulmonary artery sarcoma who survived for 20 months with adjuvant chemotherapy and medical treatment following incomplete tumor resection.…”

Section: Introductionmentioning

confidence: 99%

Primary Leiomyosarcoma of the Pulmonary Artery: A Case of a 20-Month Survivor after Incomplete Surgical Resection

et al. 2012

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We report a 53-year-old man who presented with dyspnea and low-grade fever. Cardiac ultrasound showed pulmonary hypertension and an ill-defined echogenic mass within the pulmonary trunk. Computed tomography scan revealed an inhomogeneous mass which filled the main pulmonary trunk with near-total occlusion, and extended into both pulmonary arteries. Anticoagulant therapy was administered based on a presumptive diagnosis of pulmonary thromboembolism. Positron-emission tomography scan was useful for differentiating the mass, which was determined as a pulmonary artery sarcoma by surgical resection. Although complete resection was impossible, the patient survived for 20 months with adjuvant chemotherapy and medical treatment.

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“…The median survival time of patients without surgical resection is only 1.5 months, and 10 months with resection (12). Surgical resection is the main treatment of this tumor (7). The effects of adjunctive chemotherapy and radiotherapy remain uncertain and standard chemotherapy regimens have not been unified.…”

Section: A B Cmentioning

confidence: 99%

“…The prognosis of this tumor is extremely poor, with a survival time of 12-18 months subsequent to the appearance of clinical symptoms (6). Surgical resection is the main treatment to prolong survival, which includes pulmonary endarterectomy (PEA), lobectomy and pneumonectomy (7). Combination doxorubicin and ifosfamide as first-line chemotherapy has been reportedto be transiently effective in the inhibition of rapid enlargement of the tumor.…”

Section: Introductionmentioning

confidence: 99%

Pulmonary artery intimal sarcoma misdiagnosed as pulmonary embolism: A case report

Jiang

Zeng

et al. 2017

Oncology Letters

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Abstract. Intimal sarcoma of the pulmonary artery is rare, but false diagnosis of this sarcoma as pulmonary embolism is not infrequent. The present study reports a case of pulmonary artery intimal sarcoma misdiagnosed as pulmonary artery embolism in a 37-year-old female patient. The patient was admitted to the cardiac intensive care unit of Guangdong General Hospital (Guangzhou, China) with the complaint of progressive exertional dyspnea over the past two years. Multi-slice spiral computed tomography, transthoracic echocardiography, right-heart catheterization and cardiac magnetic resonance imaging revealed mimicking severe pulmonary embolism in the pulmonary trunk and right pulmonary artery, with symptoms including chest pain, cough and breathing difficulties. In addition, positron emission tomography-computed tomography results did not identify increased 18F-fluorodeoxyglucose uptake and failed to distinguish whether the mass was a thrombus or a malignancy. The patient was diagnosed with pulmonary embolism and a subcutaneous injection of 5,000 AxaIU enoxaparin sodium (100 AXAIU/kg) was administered every 12 h, but no improvement was achieved after 5 days of treatment. Finally, pulmonary endarterectomy was performed to relieve the worsening clinical symptoms. The clinicopathological diagnosis was pulmonary artery intimal sarcoma with poor clinical outcome. For this type of tumor with fatal prognosis, early and correct diagnosis may lead to appropriate intervention and prolong survival. IntroductionSince Moritz Mandelstamm first described intimal sarcoma of the pulmonary artery in 1923 (1), this tumor has been an uncommon but aggressive malignant tumor among the pulmonary artery, and may develop a fatal course leading to heart failure without treatment (2,3). The incidence is reported to be 0.001%, but this incidence appears to be inaccurate due to the challenging (4). Pulmonary artery intimal sarcoma has similar clinical manifestations to pulmonary embolism (including chest pain, difficulty breathing, cough, chest tightness, oedema and hemoptysis), which leads to an initially false diagnosis and delayed interventions (5). Percutaneous intravascular biopsy using a bronchoscopy forceps or ultrasound-guided transbronchial needle aspiration may obtain an early definitive diagnosis (2,3). The prognosis of this tumor is extremely poor, with a survival time of 12-18 months subsequent to the appearance of clinical symptoms (6). Surgical resection is the main treatment to prolong survival, which includes pulmonary endarterectomy (PEA), lobectomy and pneumonectomy (7). Combination doxorubicin and ifosfamide as first-line chemotherapy has been reportedto be transiently effective in the inhibition of rapid enlargement of the tumor. However, the effects of adjuvant radiation, radiofrequency ablation and chemotherapy subsequent to surgery remain unclear, and further studies are warranted (5). Case reportA 37-year-old woman was admitted to Guangdong General Hospital (Guangzhou, China) on December 25, 2014 with the complaint o...

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Surgical treatment of pulmonary artery sarcoma

Cited by 102 publications

References 16 publications

Clinicopathological and immunohistochemical features of pulmonary artery sarcoma: A report of three cases and review of the literature

Clinicopathological and immunohistochemical features of pulmonary artery sarcoma: A report of three cases and review of the literature

Primary Leiomyosarcoma of the Pulmonary Artery: A Case of a 20-Month Survivor after Incomplete Surgical Resection

Pulmonary artery intimal sarcoma misdiagnosed as pulmonary embolism: A case report

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