Korean J Radiol.  2018 Aug;19(4):792-802. 10.3348/kjr.2018.19.4.792.

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

• Kim C
• Kim MY
• Kang JW
• Song JS
• Lee KY
• Kim SS

• KMID: 2413709
• DOI: http://doi.org/10.3348/kjr.2018.19.4.792

Abstract

OBJECTIVE
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.
MATERIALS AND METHODS
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.
RESULTS
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).
CONCLUSION
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.

Keyword

Pulmonary artery intimal sarcoma; Pulmonary thromboembolism; Computed tomography; Magnetic resonance imaging; Positron emission tomography-computed tomography; PET

MeSH Terms

Delayed Diagnosis
Diagnosis
Early Diagnosis
Humans
Interphase
Ischemia
Kaplan-Meier Estimate
Lung
Magnetic Resonance Imaging
Natriuretic Peptide, Brain
Positron-Emission Tomography
Pulmonary Artery*
Pulmonary Embolism
Retrospective Studies
Sarcoma*
Thromboembolism*
Natriuretic Peptide, Brain

Figure

• 

  Fig. 1 Tumor extension patterns of CT in four patients (all 5-mm thick).A. Pattern I: cauliflower-like polypoid lesion. B. Pattern II: diffuse/focal wall thickening. C. Pattern III: tumoral impaction. D. Pattern IV: tubular-polypoid shape.

• 

  Fig. 2 ROC curve showing the D-dimer level predicting PAIS.Areas under ROC curve of D-dimers was 0.88 (95% confidence interval, 0.774–0.948). PAIS = pulmonary artery intimal sarcoma, ROC = receiver operating characteristic

• 

  Fig. 3 MR images obtained in 36-year-old male patient with PAIS (same patient as in Fig. 1A).A. T2-weighted axial image obtained at pulmonary trunk level. On MR image, index lesion has lobulated contour and high SI compared with pectoralis muscle. B. In late gadolinium enhancement image using inversion time of 600 msec (TI 600), lesion has intermingled dark SI, which reflects intermingled thrombosis (arrows). MR = magenetic resonance, SI = signal intensity

• 

  Fig. 4 FDG positron emission tomography-CT image obtained from 24-year-old patient with PAIS (same patient in Fig. 1C).Surgical resection was performed.A. Axial image shows high FDG uptake in right pulmonary artery (maxSUV: 12.9). B. Focal hemorrhage in mass of gross specimen (arrow); this is common in PAIS (arrow). High magnification elastic Van-Gieson-stained specimen (inset, × 100) shows mass of intimal origin detached from intimal layer (arrowheads). FDG = fluorodeoxyglucose

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  Fig. 5 Overall survival analyzed by Kaplan-Meier method.Median overall survival of patient with PAIS was 26 months (range, 0–200 months).

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  Fig. 6 Kaplan-Meier survival curve showing overall survival of patients with and without delayed diagnosis.Overall survival of patients with delayed diagnosis was shorter than that of patients with timely diagnosis, if initial patients who died due to immediate post-operative complication (< 7 days) were excluded (mean survival, 22.5 ± 2.6 vs. 122.0 ± 29.1 months, p = 0.038).

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