Unicentric Castleman's Disease Arising from an Intrapulmonary Lymph Node

Abstract

Castleman's disease is an uncommon lymphoproliferative disorder of unknown etiology, most often involving the mediastinum. It has 2 distinct clinical forms: unicentric and multicentric. Unicentric Castleman's disease arising from an intrapulmonary lymph node is rare, and establishing a preoperative diagnosis of this disease is very difficult mainly due to a lack of specific imaging features. We report a case of intrapulmonary unicentric Castleman's disease in an asymptomatic 19-year-old male patient who was accurately diagnosed by preoperative computed tomography (CT). The mass was incidentally found on a routine chest X-ray. A subsequent dynamic CT showed a well-defined, hypervascular, soft-tissue mass with small calcifications located in the perihilar area of the right lower lung. Three-dimensional CT (3D-CT) angiography indicated that the mass was receiving its blood supply through a vascular network at its surface that originated from 2 right bronchial arteries. The clinical history and CT findings were consistent with a diagnosis of unicentric Castleman's disease, and we safely and successfully removed the tumor via video-assisted thoracoscopic surgical lobectomy. This case shows that the imaging characteristics of these rare tumors on contrast-enhanced CT combined with 3D-CT angiography can be helpful in reliably establishing a correct preoperative diagnosis.

Figure 1

Chest radiograph shows a mass lesion located in the right infrahilar region.

Figure 2

Dynamic CT scans of the chest, in plane (a), arterial (b), portal in-flow (c), and equilibrium (d), show a well-defined, lobulated, soft-tissue mass with small calcifications in the right lower lobe. The periphery of the mass was strongly enhanced in the arterial phase, and the parenchyma of the mass was heterogeneously enhanced in the portal in-flow to equilibrium phase.

Figure 3

3D-CT angiography demonstrated the enlarged feeding vessels at the surface of the mass (arrowheads) originating from 2 bronchial arteries (arrows).

Figure 4

Histology shows the typical appearance of hyaline vascular type Castleman's disease.