Key result
Occult pulmonary adenocarcinoma can cause rapidly progressive PH, RV failure, and death via tumor embolism.
Why the study?
Pulmonary lymphangitic carcinomatosis and pulmonary tumor embolism are rare manifestations of metastatic malignancy that may mimic common causes of dyspnea and pulmonary hypertension, often leading to delayed diagnosis.
Case Report (n=1)
Pulmonary lymphangitic carcinomatosis and tumor embolism should be considered in patients with rapidly progressive pulmonary hypertension and right ventricular failure despite negative macrovascular pulmonary embolism imaging.
Answers are grounded in this paper and cite their sources.
Consider occult malignancy in unexplained rapidly progressive PH; case report leaves open questions on prevalence and detection.
Pulmonary lymphangitic carcinomatosis (PLC) and pulmonary tumor embolism (PTE) are rare manifestations of metastatic malignancy that may mimic more common causes of dyspnea and pulmonary hypertension, often leading to delayed diagnosis. Both conditions are associated with poor prognosis and may present with rapidly progressive right ventricular failure. A 45-year-old female lifelong non-smoker with no significant past medical history presented with a two-week history of cough, pleuritic chest pain, and worsening fatigue. CT pulmonary angiography was negative for central macrovascular pulmonary embolism but demonstrated interlobular septal thickening, ground-glass opacities, pulmonary nodules, and vertebral lesions. Initial echocardiography showed mild to moderate pulmonary hypertension with preserved right ventricular function. Despite supportive management, the patient experienced rapid clinical deterioration, progressing to severe pulmonary hypertension, right ventricular failure, cardiogenic shock, and pulseless electrical activity arrest. Post-mortem examination revealed invasive pulmonary adenocarcinoma with extensive lymphovascular invasion and metastatic disease, consistent with PLC and PTE. This case emphasizes the need to take into account PLC and PTE in patients with rapidly progressive pulmonary hypertension despite negative pulmonary embolism imaging. Recognition of subtle radiographic clues may facilitate earlier diagnosis, although prognosis remains poor once right ventricular failure develops.
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Shahane et al. (2026) conducted a case report in Pulmonary lymphangitic carcinomatosis and pulmonary tumor embolism (n=1). Pulmonary lymphangitic carcinomatosis and pulmonary tumor embolism was evaluated. Occult pulmonary adenocarcinoma presenting as pulmonary lymphangitic carcinomatosis and tumor embolism can cause rapidly progressive pulmonary hypertension, right ventricular failure, and death.
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