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Case Reports
. 2020 Jul 17;2(6):711-715.
doi: 10.1016/j.cjco.2020.07.008. eCollection 2020 Nov.

Pulmonary Artery Intimal Sarcoma: A Deadly Diagnosis in Disguise

Mohamad Rabbani  1 , Ahmed Hafiz  1 , Muhanad Algadheeb  1 , Elena Tugaleva  2 , Margaret Lynn Bergin  3 , Lin-Rui Ray Guo  1
Affiliations
Case Reports

Pulmonary Artery Intimal Sarcoma: A Deadly Diagnosis in Disguise

Mohamad Rabbani et al. CJC Open. .

Abstract
in English, French

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.

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.

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Figures

Figure 1
Figure 1
(A) Transesophageal echocardiogram image showing the large mobile mass of 20 × 21 mm in the main pulmonary artery above the pulmonic valve, and a second large fixed mass of 17 × 14 mm in the distal main pulmonary artery. (B-E) Series of images of computed tomography pulmonary angiography taken when the patient presented to the hospital for the first time without any treatment. (B) Large lobulated near-occlusive filling defect in the main pulmonary artery. (C) Filling defect extends into the left main pulmonary artery. (D, E) Another lobulated filling defect, within the right interlobular artery extending to the right lower lobe segmental pulmonary arteries scratch, which appears occlusive. (F-I) Equivalent section of images taken 5 weeks after surgery and before chemotherapy, showing clearing of filling defects.
Figure 2
Figure 2
(A) Hematoxylin and eosin staining demonstrating neoplastic growth arising from intimal lining of sampled pulmonary artery (×20 magnification). (B) Hematoxylin and eosin staining demonstrating poorly differentiated neoplasm composed of epithelioid and pleomorphic cells with abundant background inflammation and no convincing vascular formation (×100 magnification). (C) Staining for vimentin demonstrated strong positivity (×100 magnification). (D) Staining for mouse double minute 2 demonstrated strong positivity with nuclear pattern (×100 magnification).
See this image and copyright information in PMC

References

    1. Krüger I., Borowski A., Horst M. Symptoms, diagnosis, and therapy of primary sarcomas of the pulmonary artery. Thorac Cardiovasc Surg. 1990;38:91–95. - PubMed
    1. Simpson W.L., Jr., Mendelson D.S. Pulmonary artery and aortic sarcomas: cross-sectional imaging. J Thorac Imaging. 2000;15:290–294. - PubMed
    1. Kim C., Kim M.Y., Kang J.-W. Pulmonary artery intimal sarcoma versus pulmonary artery thromboembolism: CT and clinical findings. Korean J Radiol. 2018;19:792–802. - PMC - PubMed
    1. Wittram C., Maher M.M., Yoo A.J. CT angiography of pulmonary embolism: diagnostic criteria and causes of misdiagnosis. RadioGraphics. 2004;24:1219–1238. - PubMed
    1. Parish J.M., Rosenow E.C., 3rd, Swensen S.J., Crotty T.B. Pulmonary artery sarcoma. Clinical features. Chest. 1996;110:1480–1488. - PubMed

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