Pulmonary embolism or pulmonary artery intimal sarcoma? Diagnostic pitfalls in a rare vascular malignancy

Pulmonary artery obstruction is commonly attributed to pulmonary embolism; however, rare malignant conditions may present with similar clinical and radiological findings. Failure to recognize these alternative diagnoses may result in inappropriate treatment and delayed management. We report the case of a 48-year-old man presenting with acute dyspnoea and cough. Computed tomography angiography revealed a filling defect in the right main pulmonary artery, and pulmonary embolism was diagnosed. Despite appropriate anticoagulant therapy, the patient's clinical condition progressively deteriorated. The absence of deep vein thrombosis and the lack of right heart strain prompted diagnostic reconsideration. Endobronchial ultrasound-guided fine-needle aspiration revealed malignant spindle cell proliferation, and immunohistochemistry confirmed pulmonary artery intimal sarcoma. This case highlights the importance of reconsidering the diagnosis when clinical improvement does not occur despite adequate anticoagulation. Pulmonary artery obstruction should not automatically be attributed to thromboembolism, and malignancy must be considered in the differential diagnosis. LEARNING POINTS: Pulmonary artery obstruction should not automatically be attributed to thromboembolism, as rare malignancies may present with similar findings.Lack of clinical improvement despite appropriate anticoagulant therapy should prompt immediate diagnostic reconsideration.

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Publication Details

Journal
European Journal of Case Reports in Internal Medicine
Published
2026-04-07
DOI
https://doi.org/10.12890/2026_006384
Primary Topic
Cardiac tumors and thrombi
Type
article
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article

Pulmonary embolism or pulmonary artery intimal sarcoma? Diagnostic pitfalls in a rare vascular malignancy

Sara Mangiacapra, Mariagrazia Nunziata, Natalina Iuliano, Valeria Iorio et al.
European Journal of Case Reports in Internal Medicine
Cardiac tumors and thrombi
article

Pulmonary embolism or pulmonary artery intimal sarcoma? Diagnostic pitfalls in a rare vascular malignancy

Sara Mangiacapra, Mariagrazia Nunziata, Natalina Iuliano, Valeria Iorio, M. Amitrano, Raffaele Natale, Lidia Santarpia
article en

Abstract

Pulmonary artery obstruction is commonly attributed to pulmonary embolism; however, rare malignant conditions may present with similar clinical and radiological findings. Failure to recognize these alternative diagnoses may result in inappropriate treatment and delayed management. We report the case of a 48-year-old man presenting with acute dyspnoea and cough. Computed tomography angiography revealed a filling defect in the right main pulmonary artery, and pulmonary embolism was diagnosed. Despite appropriate anticoagulant therapy, the patient's clinical condition progressively deteriorated. The absence of deep vein thrombosis and the lack of right heart strain prompted diagnostic reconsideration. Endobronchial ultrasound-guided fine-needle aspiration revealed malignant spindle cell proliferation, and immunohistochemistry confirmed pulmonary artery intimal sarcoma. This case highlights the importance of reconsidering the diagnosis when clinical improvement does not occur despite adequate anticoagulation. Pulmonary artery obstruction should not automatically be attributed to thromboembolism, and malignancy must be considered in the differential diagnosis. LEARNING POINTS: Pulmonary artery obstruction should not automatically be attributed to thromboembolism, as rare malignancies may present with similar findings.Lack of clinical improvement despite appropriate anticoagulant therapy should prompt immediate diagnostic reconsideration.

European Journal of Case Reports in Internal MedicineVol. 13(4)
Azienda Ospedaliera S.Giuseppe Moscati (IT), Federico II University Hospital (IT)
Good health and well-being
Openalex Percentile: Top 30%
Cardiac tumors and thrombi
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