Angiosarcoma in the chest: radiologic-pathologic correlation: Case report

Abstract

Rationale: Angiosarcomas are rare, malignant vascular tumors.

Patient concerns: They represents about 2% of all soft tissue sarcoma, which can often metastasize through the hematogenous route. The radiological features have been analyzed in 4 patients with metastatic angiosarcoma in the chest.

Diagnoses: The main radiologic findings included nodules, cysts, nodules with halo sign, and vascular tree-in-bud. Morphologic features, as observed in the histologic specimen, have been correlated with radiologic appearance.

Lessons: Metastatic angiosarcomas to the lung are characterized by a wide variety of radiologic appearances that can be very characteristic. Computed tomographic findings observed include bilateral solid nodules, cystic, and bullous lesions sometimes associated with spontaneous hemopneumothoraces.

Figure 1

Patient 1. CT scan shows numerous bilateral tiny nodules mainly in the middle and lower zones of hemithoraces. Many of these nodules show a vascular tree-in-bud pattern related to neoplastic thrombotic microangiopathy (red circle). Moreover vascular branches are focally enlarged. This finding is particularly evident in the left lower lobe (red arrow). A mild smooth thickening of interlobular septa is also present in both lower lobes.

Figure 2

Patient 1. CT-guided biopsy of sacrum. H&E, midpower. Dissecting vascular channels lined by endothelial cells showing various degrees of cytologic pleomorphism and nuclear atypia.

Figure 3

Patient 2. PA Chest X ray. Presence of 2 contiguous opacities in the middle zone of right hemithorax, both subpleural and with an obtuse angle with the pleural margin, suggesting the feature of pleural lesions.

Figure 4

Patient 2. CT scan confirms the presence of 2 pleural lesions, the biggest one measures 2.6 × 3.5 cm and it is in the right middle lobe.

Figure 5

Patient 2. CT-guided biopsy of the pleural lesions. (A) Atypical, multilyering pleomorphic cells often forming tuft small papillae (H&E, midpower). (B) These cells are positive with the vascular marker CD31.

Figure 6

Patient 2. CT shows bilateral pulmonary metastatic lesions characterized by nodules with halo sign (the biggest measuring about 1 cm) and ground glass lesions.

Figure 7

Patient 2. CT scan 2 months after the evidence of pulmonary progression shows the increase of pulmonary nodules, bilateral pleural effusion and wide ground glass attenuation, mainly in the right upper lobe.

Figure 8

Patient 3. CT scan shows several consolidations with halo sign, the biggest in the right middle lobe. Some ground glass lesions are also present; the biggest in size is in the posterior segment of right upper lobe.

Figure 9

Patient 3. Surgical lung biopsy. (A) A small pulmonary artery has the lumen completely obliterated by neoplastic cells. The surrounding airspaces are filled by red cells and hemosiderin laden macrophages (H&E, midpower). (B) These cells show strong CD 31 positivity.

Figure 10

Patient 4. CT scan shows bilateral cysts, some of these with thick wall as in the anterior segment of left upper lobe. Some solid nodules are also present, particularly in right middle and lower lobe. Both the cystic lesions and nodules are suspicious of metastatic lesions. Bilateral hydropneumothorax is present.

Figure 11

Patient 4. CT scan shows left plural drainage and the onset of bilateral severe subcutaneous emphysema. Pneumothorax is significantly reduced, remaining bilaterally in a small size. A small bilateral pneumothorax and pneumomediastinum are also present.

Figure 12

Patient 4. Comparison between baseline CT scan and control 3 weeks later shows that the cystic lesions are bigger and more numerous than the prior, suggesting a rapid disease progression. Moreover, nodules that were visible in the prior examination now have a cystic shape (blue circle). Informed consent from patients was obtained.