IS Case 110: Castleman's Disease
Imaging Sciences URMC 2008
Publication Date: 2009-05-20
Frontal and lateral plain radiographs of the chest demonstrate a large subcarinal mass. The azygoesophageal line is displaced to the right. Post-contrast chest CT demonstrates a sizeable mass in the posterior mediastinum measuring up to 11 cm in diameter. The mass appears to be solid and well-circumscribed, deviating the esophagus towards the left and abutting the posterior carina, as well as the left and right main-stem bronchi. There is no obvious involvement of the lung parenchyma. There are considerable feeding vessels.
Castleman's disease is a rare, benign disorder which can be seen as abnormal enlargement of the lymph nodes. Two subdivisions are commonly noted including localized hyaline-vascular disease (majority) versus disseminated plasma cell type.
The localized hyaline vascular type are hypervascular lesions and have a peak incidence in the fourth decade. This usually presents as an asymptomatic, enlarged, sharply marginated, intensely enhancing soft tissue mass in the middle mediastinum and hila but can be seen in any thoracic compartment, neck or abdomen. Calcification is uncommon but when present is coarse and in a central location. T1-weighted images show heterogeneous lesions that are increased in signal intensity versus skeletal muscle. T2-weighted images show hyperintensity with occasional low signal intensity septa. Post gadolinium images show enhancement. These lesions are usually completely surgically resected. Recurrence is rare.
The less common disseminated plasma cell type describes systemic disease and can occur in lymph node tissues through the body.This had an association with POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal proteinemia and skin changes) syndrome. Common findings include diffuse lymphadenopathy (1-6cm diameter) involving all mediastinal compartments. Focal mediastinal masses are rare. Symptoms can include fever, weight loss, skin rash, hemolytic anemia, and hypergammaglobulinemia.
- McAdams HP, Rosado-de-Christenson M, Fishback NF, Templeton PA. Castleman disease of the thorax: radiologic features with clinical and histopathologic correlation. Radiology. 1998 Oct;209(1):221-8. [PMID: 9769835]