Pulmonary Artery Intimal Sarcoma: A Deadly Diagnosis in DisguiseNovel Teaching Points
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, w...
| Main Authors: | , , , , , |
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| Format: | Article |
| Language: | English |
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Elsevier
2020-11-01
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| Series: | CJC Open |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2589790X20301037 |
| _version_ | 1818324612624154624 |
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| author | Mohamad Rabbani, MD, CM Ahmed Hafiz, MD Muhanad Algadheeb, MD Elena Tugaleva, MD, FRCPC Margaret Lynn Bergin, MD, FRCPC Lin-Rui Ray Guo, MD, FRCSC |
| author_facet | Mohamad Rabbani, MD, CM Ahmed Hafiz, MD Muhanad Algadheeb, MD Elena Tugaleva, MD, FRCPC Margaret Lynn Bergin, MD, FRCPC Lin-Rui Ray Guo, MD, FRCSC |
| author_sort | Mohamad Rabbani, MD, CM |
| collection | DOAJ |
| description | 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. Résumé: Le sarcome intimal de l’artère pulmonaire est une tumeur très rare. On estime que sa prévalence se situe entre 0,001 % et 0,003 %. Elle pourrait cependant être plus élevée, étant donné que sa présentation est comparable à celle des thromboembolies pulmonaires et que les erreurs diagnostiques sont possibles. Le pronostic du sarcome intimal de l’artère pulmonaire est très sombre, la survie globale médiane variant de 11 à 18 mois. Nous décrivons le cas d’un homme de 36 ans qui s’est présenté à notre clinique de chirurgie cardiaque en décrivant des symptômes non distinctifs et qui a reçu un diagnostic de sarcome intimal de l’artère pulmonaire nécessitant une résection chirurgicale ainsi qu’une chimiothérapie adjuvante. Nous soulignons les ca-ractéristiques radiologiques et pathologiques du patient, l’approche chirurgicale adoptée et la chimiothérapie sélectionnée. |
| first_indexed | 2024-12-13T11:31:21Z |
| format | Article |
| id | doaj.art-61935f8729604466b356725a6513d8e6 |
| institution | Directory Open Access Journal |
| issn | 2589-790X |
| language | English |
| last_indexed | 2024-12-13T11:31:21Z |
| publishDate | 2020-11-01 |
| publisher | Elsevier |
| record_format | Article |
| series | CJC Open |
| spelling | doaj.art-61935f8729604466b356725a6513d8e62022-12-21T23:47:54ZengElsevierCJC Open2589-790X2020-11-0126711715Pulmonary Artery Intimal Sarcoma: A Deadly Diagnosis in DisguiseNovel Teaching PointsMohamad Rabbani, MD, CM0Ahmed Hafiz, MD1Muhanad Algadheeb, MD2Elena Tugaleva, MD, FRCPC3Margaret Lynn Bergin, MD, FRCPC4Lin-Rui Ray Guo, MD, FRCSC5Division of Cardiac Surgery, Western University, London, Ontario, Canada; Corresponding author: Dr Mohamad Rabbani, Division of Cardiac Surgery, London Health Sciences Centre, 339 Windermere Rd, London, Ontario, N6A 5A5, Canada. Tel.: (519) 685-8500.Division of Cardiac Surgery, Western University, London, Ontario, CanadaDivision of Cardiac Surgery, Western University, London, Ontario, CanadaDivision of Pathology, Western University, London, Ontario, CanadaDivision of Cardiology, Western University, London, Ontario, CanadaDivision of Cardiac Surgery, Western University, London, Ontario, CanadaPulmonary 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. Résumé: Le sarcome intimal de l’artère pulmonaire est une tumeur très rare. On estime que sa prévalence se situe entre 0,001 % et 0,003 %. Elle pourrait cependant être plus élevée, étant donné que sa présentation est comparable à celle des thromboembolies pulmonaires et que les erreurs diagnostiques sont possibles. Le pronostic du sarcome intimal de l’artère pulmonaire est très sombre, la survie globale médiane variant de 11 à 18 mois. Nous décrivons le cas d’un homme de 36 ans qui s’est présenté à notre clinique de chirurgie cardiaque en décrivant des symptômes non distinctifs et qui a reçu un diagnostic de sarcome intimal de l’artère pulmonaire nécessitant une résection chirurgicale ainsi qu’une chimiothérapie adjuvante. Nous soulignons les ca-ractéristiques radiologiques et pathologiques du patient, l’approche chirurgicale adoptée et la chimiothérapie sélectionnée.http://www.sciencedirect.com/science/article/pii/S2589790X20301037 |
| spellingShingle | Mohamad Rabbani, MD, CM Ahmed Hafiz, MD Muhanad Algadheeb, MD Elena Tugaleva, MD, FRCPC Margaret Lynn Bergin, MD, FRCPC Lin-Rui Ray Guo, MD, FRCSC Pulmonary Artery Intimal Sarcoma: A Deadly Diagnosis in DisguiseNovel Teaching Points CJC Open |
| title | Pulmonary Artery Intimal Sarcoma: A Deadly Diagnosis in DisguiseNovel Teaching Points |
| title_full | Pulmonary Artery Intimal Sarcoma: A Deadly Diagnosis in DisguiseNovel Teaching Points |
| title_fullStr | Pulmonary Artery Intimal Sarcoma: A Deadly Diagnosis in DisguiseNovel Teaching Points |
| title_full_unstemmed | Pulmonary Artery Intimal Sarcoma: A Deadly Diagnosis in DisguiseNovel Teaching Points |
| title_short | Pulmonary Artery Intimal Sarcoma: A Deadly Diagnosis in DisguiseNovel Teaching Points |
| title_sort | pulmonary artery intimal sarcoma a deadly diagnosis in disguisenovel teaching points |
| url | http://www.sciencedirect.com/science/article/pii/S2589790X20301037 |
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