Browse
Submit Data
Databases
API
Help

Dataset Information

26 Views

0 Connections

0 Citations

0 Reanalyses

0 Downloads

Omics score: 0

Pulmonary Artery Intimal Sarcoma: A Deadly Diagnosis in Disguise.

ABSTRACT: Pulmonary artery intimal sarcoma (PAIS) is a very rare tumour. The prevalence of PAIS is estimated to be between 0.001% and 0.003%, but this may be an underestimation because of potential misdiagnosis due to its similar presentation to that of pulmonary thromboembolism. The prognosis is very poor, with median overall survival between 11 and 18 months. We report a case of a 36-year-old man who presented to our cardiac surgery clinic reporting nonspecific symptoms and was found to have PAIS requiring surgical resection and adjuvant chemotherapy. We outline the radiologic features, pathologic characteristics, surgical approach, and chemotherapy treatment utilized.

SUBMITTER: Rabbani M

PROVIDER: S-EPMC7710993 | biostudies-literature | 2020 Nov

REPOSITORIES: biostudies-literature

ACCESS DATA

Json Xml

Publications

Pulmonary Artery Intimal Sarcoma: A Deadly Diagnosis in Disguise.

Rabbani Mohamad M Hafiz Ahmed A Algadheeb Muhanad M Tugaleva Elena E Bergin Margaret Lynn ML Ray Guo Lin-Rui LR

CJC open 20200717 6

Pulmonary artery intimal sarcoma (PAIS) is a very rare tumour. The prevalence of PAIS is estimated to be between 0.001% and 0.003%, but this may be an underestimation because of potential misdiagnosis due to its similar presentation to that of pulmonary thromboembolism. The prognosis is very poor, with median overall survival between 11 and 18 months. We report a case of a 36-year-old man who presented to our cardiac surgery clinic reporting nonspecific symptoms and was found to have PAIS requir ...[more]

PMID: 33305235

Similar Datasets

Pulmonary thromboembolic disease or pulmonary artery intimal sarcoma: Case report and literature review.

Project description:The present case study reported on a patient initially diagnosed with pulmonary embolism at the First Affiliated Hospital of Fujian Medical University (Fuzhou, China) in May 2021. Furthermore, a relevant literature review was performed. The patient was a 57-year-old Chinese male who presented with dyspnea and wheezing following exercise. Physical examination revealed pulmonary valve second heart sound > aortic valve second heart sound but lack of swelling on both lower limbs, while the imaging results suggested pulmonary artery filling defects. Initially, the patient was diagnosed with pulmonary embolism and was administered anticoagulation treatment, which lasted for 3 months but proved to be ineffective. Subsequent re-examination via chest computed tomography further indicated multiple nodules in the left hilum and lung. Therefore, the patient was hospitalized for lung aspiration biopsy, which led to the final diagnosis of pulmonary artery intimal sarcoma based on the pathological review.

| S-EPMC9478618 | biostudies-literature

Problems and countermeasures for surgical resection of primary pulmonary artery intimal sarcoma.

Project description:BackgroundPulmonary artery intimal sarcoma (PAIS) is a rare but aggressive malignancy. This study clarified the problems and countermeasures of surgical treatment by examining surgical cases of PAIS.MethodsBetween January 2007 and October 2020, 10 patients with PAIS who underwent surgery at our hospital were retrospectively examined.ResultsThe surgical procedure that aimed at complete resection was pulmonary resection only (three cases), along with pulmonary artery vascular replacement (six cases) and pulmonary endarterectomy (PE) (one case). The positive rate of vascular stumps was 7/10. In all cases, chest computed tomography scan showed positive margins of ≤20 mm between the tumor and surgical dissection (6/6). In addition, the distance between the location of the tumor on computed tomography and the dissection line during surgery needed to be at least 20 mm (2/3). However, even at a distance of 25 mm, one case with a positive margin was observed. Postoperative recurrence was 8/9 cases, and the median recurrence period was as short as 10 months (range, 3-19 months). Postoperative treatment was required in 7/9 cases (operation/chemotherapy/radiotherapy/chemoradiotherapy/heavy ion radiotherapy =1/2/2/1/1). The median survival was 15 months (range, 0.5-36 months).ConclusionsExtended surgery should be performed as much as possible, with a distance of at least 20 mm between the location of the tumor on computed tomography scan and the incision line during surgery. The median postoperative recurrence period was as short as 10 months. Therefore, intensive care for intrathoracic recurrence follow-up is required for 1 year after surgery.

| S-EPMC10007893 | biostudies-literature

An Unusual Cause of Acute Myocardial Infarction Caused by a Large Pulmonary Artery Intimal Sarcoma.

Project description: Not available

| S-EPMC6058249 | biostudies-literature

Pedunculated pulmonary artery intimal sarcoma with poor uptake in 18F-FDG PET/CT: A case report.

Project description:Pulmonary artery intimal sarcoma (PAIS) is a rare tumor with an incidence of 0.001%-0.03% that usually grows along artery walls and absorbs fluorodeoxyglucose. It is difficult to distinguish PAIS from pulmonary thromboembolism due to the similarities of their symptoms. Therefore, contrast-enhanced computed tomography and positron emission tomography-computed tomography (PET-CT) should be used to establish a correct diagnosis. Here we report a case of an extremely rare type of PAIS, pedunculated PAIS, which could not be visualized on PET-CT. Histological features of a tumor with a low accumulation of fluorodeoxyglucose revealed low-cellularity and necrotizing background. Multimodal imaging was useful to diagnose PET-CT negative PAIS accurately. <Learning objective: Pedunculated pulmonary artery intimal sarcoma (PAIS) is a rare form of neoplasm. You need to know that PAIS which has low cellularity with marked interstitial myxoid tissue cannot be detected on positron emission tomography-computed tomography.>.

| S-EPMC8380851 | biostudies-literature

Visualization of pulmonary artery intimal sarcoma by color-coded iodine map using dual-energy computed tomography.

Project description:Pulmonary artery intimal sarcomas (PAIS) are often misdiagnosed as pulmonary embolisms (PE) as their clinical findings and imaging findings are similar. However, given the clinical outcome of both diseases is different in its prognosis, accurate and rapid diagnosis is mandatory. This is a case report of a histologically-proven PAIS which was initially treated as a PE. The color-coded iodine map using dual-energy computed tomography (dual-energy CT iodine map) well reflected the distribution of the tumor consistent with 18fluoro-2-deoxyglucose-uptake region using positron emission tomography/CT. This case demonstrates the potential of using dual-energy CT iodine map to differentiate PAIS from PE.Learning objectiveUse of a dual-energy computed tomography iodine map to visualize a pulmonary artery intimal sarcoma may provide useful diagnostic information.

| S-EPMC9352408 | biostudies-literature

Obstructive Shock Due to a Crushed Left Atrium and Pulmonary Vein by Pulmonary Artery Intimal Sarcoma Successfully Treated with Pulmonary Vein Stenting.

Project description:A 46-year-old patient who had undergone right pneumonectomy for pulmonary artery intimal sarcoma presented with hypoxemia. The recurrent sarcoma in the mediastinum revealed external compression to the left pulmonary veins (PVs), leading to obstructive shock and cardiac arrest. Venous artery extracorporeal membrane oxygenation (VA-ECMO) was initiated; however, withdrawal was difficult, and the patient's survival seemed hopeless. However, the patient's condition improved with stenting for the compressed PV; therefore, VA-ECMO was discontinued, and he was discharged on foot. This is the first case report of obstructive shock due to critical PV stenosis caused by compression of a malignant tumor that responded to PV stenting.

| S-EPMC10864061 | biostudies-literature

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

Project description: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.

| S-EPMC5836187 | biostudies-literature

Pulmonary artery sarcoma mimicking pulmonary embolism.

Project description:Primary sarcomas that arise from major blood vessels are exceedingly rare, and some of the published cases have been autopsy reports. Most patients are adults. We report a case of pulmonary artery sarcoma in a 77-year-old man who presented with acute onset of dyspnea. Magnetic resonance imaging of the chest revealed a large mass within the pulmonary trunk and its main branches. Because massive pulmonary embolism was suspected, both anticoagulant and thrombolytic therapies were initiated. The patient responded poorly to these therapies, which then necessitated resection of both the mass and the pulmonary valve. A bioprosthetic porcine valve replaced the native valve, and we reconstructed the right ventricular outflow tract with a Dacron patch. Histopathologic examination revealed a high-grade sarcoma with focal myogenic and chondrogenic differentiation. The patient tolerated the procedure well and was discharged from the hospital on postoperative day 7. He was subsequently treated with chemotherapy and radiation and continued to show no evidence of disease. The diagnosis of pulmonary artery sarcoma should be suspected in patients who present with manifestations of pulmonary embolism, especially when there is no evidence of deep venous thrombosis and poor response to anticoagulant therapy. Multimodal therapy can provide prolonged survival.

| S-EPMC4189355 | biostudies-literature

The imitation game: Pulmonary artery sarcoma masquerading as an acute pulmonary embolism.

Project description: Not available

| S-EPMC8691795 | biostudies-literature

Missed pulmonary artery sarcoma requiring radical excision and pneumonectomy.

Project description: Not available

| S-EPMC8691900 | biostudies-literature