A Comprehensive Insight Into Primary Intimal Sarcoma of the Pulmonary Artery; From Diagnosis to Management: A Case Report and Review of the Literature
ABSTRACT Primary intimal sarcoma of the pulmonary artery is a rare and aggressive malignancy that presents significant diagnostic and therapeutic challenges due to its nonspecific symptoms and propensity for late detection. This case report aimed to elucidate the diagnostic journey, surgical interve...
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| Format: | Article |
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Wiley
2024-11-01
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| Series: | Clinical Case Reports |
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| Online Access: | https://doi.org/10.1002/ccr3.9580 |
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| author | Azin Alizadehasl Soroush Najdaghi Maryam Mohseni Salehi Shahla Meshgi Seyedeh Fatemeh Hosseini Jebelli Azam Yalameh Aliabadi Hoda Hakimian Sara Forati Amineh Safavirad Delaram Narimani Davani |
| author_facet | Azin Alizadehasl Soroush Najdaghi Maryam Mohseni Salehi Shahla Meshgi Seyedeh Fatemeh Hosseini Jebelli Azam Yalameh Aliabadi Hoda Hakimian Sara Forati Amineh Safavirad Delaram Narimani Davani |
| author_sort | Azin Alizadehasl |
| collection | DOAJ |
| description | ABSTRACT Primary intimal sarcoma of the pulmonary artery is a rare and aggressive malignancy that presents significant diagnostic and therapeutic challenges due to its nonspecific symptoms and propensity for late detection. This case report aimed to elucidate the diagnostic journey, surgical intervention, and multidisciplinary management of this rare entity. In September 2023, a 42‐year‐old male presented with dyspnea on exertion and retrosternal chest pain, classified as NYHA FC II. Initial investigations, including ECG and lab tests, indicated tachycardia and elevated troponin and NT‐pro‐BNP levels. Transthoracic and transesophageal echocardiography identified a multilobulated mass in the right ventricular outflow tract and main pulmonary artery. Cardiac MRI and CT angiography confirmed a high‐grade pleomorphic spindle cell tumor, leading to surgical resection in October 2023. Histopathology confirmed intimal sarcoma. Postsurgery, the patient underwent chemotherapy and radiotherapy, showing significant clinical improvement and no recurrence on follow‐up PET‐CT. This case highlights the importance of a multidisciplinary approach in diagnosing and managing primary intimal sarcoma of the pulmonary artery, emphasizing the role of advanced imaging, timely surgical intervention, and combined chemotherapy with radiotherapy in improving patient outcomes. |
| format | Article |
| id | doaj-art-b304fd93dcd2450097695457d5cc4de0 |
| institution | Kabale University |
| issn | 2050-0904 |
| language | English |
| publishDate | 2024-11-01 |
| publisher | Wiley |
| record_format | Article |
| series | Clinical Case Reports |
| spelling | doaj-art-b304fd93dcd2450097695457d5cc4de02024-11-25T12:48:38ZengWileyClinical Case Reports2050-09042024-11-011211n/an/a10.1002/ccr3.9580A Comprehensive Insight Into Primary Intimal Sarcoma of the Pulmonary Artery; From Diagnosis to Management: A Case Report and Review of the LiteratureAzin Alizadehasl0Soroush Najdaghi1Maryam Mohseni Salehi2Shahla Meshgi3Seyedeh Fatemeh Hosseini Jebelli4Azam Yalameh Aliabadi5Hoda Hakimian6Sara Forati7Amineh Safavirad8Delaram Narimani Davani9Cardio‐Oncology Research Center Rajaie Cardiovascular Medical and Research Institute Tehran IranHeart Failure Research Center, Cardiovascular Research Institute Isfahan University of Medical Science Isfahan IranCardio‐Oncology Research Center Rajaie Cardiovascular Medical and Research Institute Tehran IranDepartment of Cardiac Imaging, Rajaie Cardiovascular Medical and Research Center, School of Medicine Iran University of Medical Sciences Tehran IranCardio‐Oncology Research Center Rajaie Cardiovascular Medical and Research Institute Tehran IranCardio‐Oncology Research Center Rajaie Cardiovascular Medical and Research Institute Tehran IranCardio‐Oncology Research Center Rajaie Cardiovascular Medical and Research Institute Tehran IranCardio‐Oncology Research Center Rajaie Cardiovascular Medical and Research Institute Tehran IranCardio‐Oncology Research Center Rajaie Cardiovascular Medical and Research Institute Tehran IranHeart Failure Research Center, Cardiovascular Research Institute Isfahan University of Medical Science Isfahan IranABSTRACT Primary intimal sarcoma of the pulmonary artery is a rare and aggressive malignancy that presents significant diagnostic and therapeutic challenges due to its nonspecific symptoms and propensity for late detection. This case report aimed to elucidate the diagnostic journey, surgical intervention, and multidisciplinary management of this rare entity. In September 2023, a 42‐year‐old male presented with dyspnea on exertion and retrosternal chest pain, classified as NYHA FC II. Initial investigations, including ECG and lab tests, indicated tachycardia and elevated troponin and NT‐pro‐BNP levels. Transthoracic and transesophageal echocardiography identified a multilobulated mass in the right ventricular outflow tract and main pulmonary artery. Cardiac MRI and CT angiography confirmed a high‐grade pleomorphic spindle cell tumor, leading to surgical resection in October 2023. Histopathology confirmed intimal sarcoma. Postsurgery, the patient underwent chemotherapy and radiotherapy, showing significant clinical improvement and no recurrence on follow‐up PET‐CT. This case highlights the importance of a multidisciplinary approach in diagnosing and managing primary intimal sarcoma of the pulmonary artery, emphasizing the role of advanced imaging, timely surgical intervention, and combined chemotherapy with radiotherapy in improving patient outcomes.https://doi.org/10.1002/ccr3.9580cardiac MRIcase reportchemotherapyintimal sarcomapulmonary artery massradiotherapy |
| spellingShingle | Azin Alizadehasl Soroush Najdaghi Maryam Mohseni Salehi Shahla Meshgi Seyedeh Fatemeh Hosseini Jebelli Azam Yalameh Aliabadi Hoda Hakimian Sara Forati Amineh Safavirad Delaram Narimani Davani A Comprehensive Insight Into Primary Intimal Sarcoma of the Pulmonary Artery; From Diagnosis to Management: A Case Report and Review of the Literature Clinical Case Reports cardiac MRI case report chemotherapy intimal sarcoma pulmonary artery mass radiotherapy |
| title | A Comprehensive Insight Into Primary Intimal Sarcoma of the Pulmonary Artery; From Diagnosis to Management: A Case Report and Review of the Literature |
| title_full | A Comprehensive Insight Into Primary Intimal Sarcoma of the Pulmonary Artery; From Diagnosis to Management: A Case Report and Review of the Literature |
| title_fullStr | A Comprehensive Insight Into Primary Intimal Sarcoma of the Pulmonary Artery; From Diagnosis to Management: A Case Report and Review of the Literature |
| title_full_unstemmed | A Comprehensive Insight Into Primary Intimal Sarcoma of the Pulmonary Artery; From Diagnosis to Management: A Case Report and Review of the Literature |
| title_short | A Comprehensive Insight Into Primary Intimal Sarcoma of the Pulmonary Artery; From Diagnosis to Management: A Case Report and Review of the Literature |
| title_sort | comprehensive insight into primary intimal sarcoma of the pulmonary artery from diagnosis to management a case report and review of the literature |
| topic | cardiac MRI case report chemotherapy intimal sarcoma pulmonary artery mass radiotherapy |
| url | https://doi.org/10.1002/ccr3.9580 |
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