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Pulmonary tumor thrombotic microangiopathy associated with right ventricular rupture and hemopericardium: A case
Abdallah Kamouh1, Andrew Nelson2, Shashank Vats1
1Division of Cardiology, University of Minnesota Medical Center, Minneapolis, MN, USA.
Abstract:
Pulmonary tumor thrombotic microangiopathy is a rare form of pulmonary tumor embolism clinically indistinguishable from pulmonary thromboembolic disease. It is characterized by fibrocellular intimal proliferation of small pulmonary arteries and arterioles in patients with intraluminal tumor embolization. We report the case of a 67-year-old Caucasian woman who had been treated for breast cancer 5 years prior to presentation and had been free of recurrence on routine clinical surveillance. She was admitted for evaluation of three witnessed syncopal events accompanied by tachypnea and hypotension. Transthoracic echocardiography revealed severe right ventricular dilatation and severely reduced right ventricular function. Shortly after admission, the patient became acutely unresponsive and expired despite aggressive resuscitation measures. The proximate cause of death was a rupture of the right ventricular free wall and a large hemopericardium. Histopathology revealed extensive microscopic occlusions of small pulmonary arteries and arterioles by metastatic breast carcinoma with superimposed acute thrombi. Pulmonary tumor thrombotic microangiopathy should be included in the differential diagnosis of patients presenting with symptoms of pulmonary thromboembolism and a history of malignancy. <Learning objective: Pulmonary tumor thrombotic microangiopathy is a rare form of pulmonary tumor embolism clinically indistinguishable from pulmonary thromboembolic disease. It is characterized by fibrocellular intimal proliferation of small pulmonary arteries and arterioles in patients with intraluminal tumor embolization. The disease has a fulminant course and poor prognosis. It should be included in the differential diagnosis of patients presenting with symptoms of pulmonary thromboembolism and a history of malignancy.>.
Insights
Pulmonary tumor thrombotic microangiopathy, a rare condition, presents like pulmonary embolism. It involves tumor emboli causing artery blockages and has a poor prognosis, especially in cancer patients.
Area of Science:
- Oncology
- Pathology
- Cardiology
Background:
- Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare vascular complication of cancer.
- It mimics pulmonary thromboembolic disease, posing diagnostic challenges.
Observation:
- A 67-year-old woman with a history of breast cancer presented with syncope, tachypnea, and hypotension.
- Echocardiography showed severe right ventricular dysfunction.
- The patient experienced rapid deterioration and expired due to right ventricular rupture and hemopericardium.
Findings:
- Histopathology confirmed extensive PTTM with metastatic breast carcinoma occluding small pulmonary arteries.
- Microscopic tumor emboli and intimal proliferation were evident.
- Superimposed acute thrombi contributed to the arterial occlusion.
Implications:
- PTTM is a critical differential diagnosis in patients with malignancy and symptoms of pulmonary embolism.
- Early recognition of PTTM is vital for appropriate management, despite its poor prognosis.
- This case highlights the aggressive nature and fatal potential of PTTM.
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