Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Intrapulmonary castleman disease
- Radiologic Findings
- (A) Chest posteroanterior (PA) radiograph demonstrates a left paramediastinal mass. (B) Axial non-enhanced chest computed tomography (CT) image reveals a hyperenhancing mass in the superior segment of the left lower lobe (LLL) and enlarged left interlobar lymph node. (C) Axial contrast-enhanced CT image shows prominent enhancement of the mass and lymph node. (D) Coronal CT image depicts the mass with a small left pleural effusion.
- Brief Review
- Castleman disease (CD) is a rare lymphoproliferative disorder classified into unicentric (UCD) and multicentric (MCD) forms. UCD typically presents in the 4th decade as a solitary, enlarging mass, often discovered incidentally or due to compression symptoms, while MCD manifests in older patients with systemic symptoms like fever and weight loss. The two primary histopathologic subtypes are hyaline vascular and plasma cell variants. The hyaline vascular type is characterized by vascular proliferation, hyalinized walls, and unique lymph node architecture, including "onion skinning" and the "lollipop appearance." UCD is most commonly located in the thorax, particularly in the mediastinum or hilar region, but it may uncommonly arise in locations such as the lung, pleura, or pericardium.
Radiologically, UCD masses appear as hypervascular, round lesions, with noncontrast CT showing homogeneous hypo- or isoattenuation relative to muscle. After contrast administration, smaller thoracic lesions (<5 cm) exhibit homogeneous enhancement, while larger or extrathoracic lesions show heterogeneous patterns due to fibrosis or necrosis. Uncommon cases in the lung, pleura, or pericardium demonstrate imaging findings similar to those of UCD arising in lymph nodes. Rare calcifications (10%) may appear nonspecific. Prominent feeding vessels, a relatively specific feature, are often identified and assist in surgical planning. UCD lesions are typically larger than those in MCD, with a mean size of 5.5 cm.
- References
- 1. Radiographics. 2023 Aug;43(8):e220210.
2. Ann Thorac Cardiovasc Surg. 2014;20
- Keywords