PMC3420614_01
Pulmonary angiography suggestive for chronic thromboembolic pulmonary hypertension (CTEPH) with unexplained multiple dilatations of the branches of the right upper lobar artery.
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Chest X-ray showed enlarged pulmonary arteries with a right parahilar nodule, perfusion scan multiple lobar and segmental defects, and pulmonary angiography amputations of the right lower lobe and left upper lobe arteries, with multiple aneurysmal dilatations of the branches of the right upper lobe artery (Figure 1).
(a) Initial PET scan. Metabolic active lesions in the right upper lobe which diameter increased over time, with appearance of a metabolically active cavity in the right lower lobe. (b) Follow-up PET scan after 2 months of oral corticosteroids. The diameter of the metabolic active lesions in the right upper lobe increased over time, with appearance of a metabolically active cavity in the right lower lobe.
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CT of the chest confirmed the presence of a nodule in the right upper lobe, which was metabolically active on PET scan (Figure 2(a)). After 2 months of therapy, evolution was characterized by clinical deterioration with appearance of cough, brown and sometimes bloody expectorations and intermittent fever, by a slightly increased CRP (19 mg/L, <5), and by progression of the lesions on Chest X-ray (Figure 3), CT (Figure 4), and PET (Figure 2(b)) scans.
Follow-up chest X-ray shows a thin-walled cavitary lesion in the right lower lobe (white arrow).
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fig3
After 2 months of therapy, evolution was characterized by clinical deterioration with appearance of cough, brown and sometimes bloody expectorations and intermittent fever, by a slightly increased CRP (19 mg/L, <5), and by progression of the lesions on Chest X-ray (Figure 3), CT (Figure 4), and PET (Figure 2(b)) scans.
CT thorax shows an initial lung infiltrate (a) evolving into a large thin-walled cavitary lesion in the right lower lobe (b).
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fig4
After 2 months of therapy, evolution was characterized by clinical deterioration with appearance of cough, brown and sometimes bloody expectorations and intermittent fever, by a slightly increased CRP (19 mg/L, <5), and by progression of the lesions on Chest X-ray (Figure 3), CT (Figure 4), and PET (Figure 2(b)) scans.
This section of the lung by autopsy showed a dilated vessel with tumor (intima sarcoma, rectangle) and extravascular extension of the tumor (circle). (Hematoxylin and eosin, x12.5).
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Autopsy showed (i) residual intima sarcoma in the peripheral branches of both pulmonary arteries, with complete or partial vessel wall invasion and extravascular extension (Figure 5), (ii) a myxoid nodular mass, 3 cm in diameter, in the right middle lobe, (iii) a large "cavity" with myxoid center, more specifically formed by oedematous tumoral tissue forming a lattice-like network responsible for radiolucency, in the right lower lobe.