Pulmonary Artery Intimal Sarcoma versus Pulmonary Artery Thromboembolism: CT and Clinical Findings

To describe CT and clinical findings of pulmonary artery intimal sarcoma (PAIS) compared with those of pulmonary thromboembolism (PTE), to investigate MRI and positron emission tomography (PET)-CT findings of PAIS, and to evaluate the effect of delayed diagnosis of PAIS on survival outcomes.

Twenty-six patients with PAIS were retrospectively identified and matched for sex, with patients with PTE at a ratio of 1:2. CT and clinical findings of the two groups were compared using Student's t test or chi-square test. The effect of delayed diagnosis on survival was investigated using Kaplan-Meier analysis.

The most common tumor pattern in PAIS was tumoral impaction. Heterogeneous attenuation, wall eclipse signs, intratumoral vessels, acute interphase angles, single location, presence of lung ischemia, and central location were significantly more common in PAIS than in PTE (all p < 0.01). Levels of D-dimers and brain natriuretic peptide were lower in PAIS than in PTE (p < 0.05). In three patients of PAIS, long inversion time sequence MRI showed intermingled dark signal intensity foci suggestive of intermingled thrombi. All nine patients who had undergone PET-CT displayed hypermetabolism. Diagnosis was delayed in 42.3% of the PAIS patients and those patients had a significantly shorter overall survival than patients whose diagnosis was not delayed (p < 0.05).

The characteristic CT and clinical findings of PAIS may help achieve early diagnosis of PAIS and make better survival outcomes of patients. MRI and PET-CT can be used as second-line imaging modalities and could help distinguish PAIS from PTE and to plan clinical management.