Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Multicentric Castleman's Disease with Lung Involvement (Lymphocytic Interstitial Pneumonitis)
- Radiologic Findings
- Chest radiograph shows diffuse coarse reticulonodular opacities in both lung fields. Chest CT shows multiple enlarged lymph nodes in the mediastinum, both axillary areas, and upper abdomen, which was associated with splenomegaly. And also there are poorly defined centrilobular nodules, ground-glass opacities and diffuse interstitial thickening in both lungs.
The histopathological diagnosis of Castleman's disease (plasma cell type) and lymphocytic interstitial pneumonia were made by mediastinal lymph node biopsy and wedge resection of the right middle and lower lobes.
- Brief Review
- Castleman’s disease(CD) is an uncommon benign lymphoproliferative disorder characterized by hyperplasia of lymphoid follicles. Two histological types are described: hyaline-vascular type and plasma cell type. From a clinical stand point, CD is also classified as localized or multicentric.
The hyaline-vascular type of CD occurs in up to 90% of cases. Patients with the hyaline-vascular type are usually children or young adults and up to 70% of them have an asymptomatic, localized mediastinal mass. On the other hand, the plasma cell variety often presents as a multicentric process, associated with generalized lymphadenopathy and hepatosplenomegaly. It often results in a systemic illness, associated with fever, anemia, infections, and malignancies such as lymphoma or Kaposi’s sarcoma.
On contrast-enhanced CT, localized CD typically shows dense contrast enhancement; any mediastinal compartment can be involved. Central, dense, or flocculent lymph node calcifications are occasionally seen. Patients with multicentric CD have shown early, dense, uniform enhancement of enlarged mediastinal lymph nodes. Axillary, abdominal, and other lymph node groups may also be involved.
Histologic findings obtained from lung biopsy in patients who had multicentric CD have been shown lymphocytic interstitial pneumonitis(LIP). CT findings of LIP include ground-glass attenuation, air-space consolidation, ill-defined nodules, extensive thickening of bronchovascular bundles and interlobular septa, and cysts. Multicentric CD had a much worse prognosis than localized CD and currently regarded as a potentially malignant lymphoproliferative disorder.
- References
- 1. Thoracic Imaging: Pulmonary and Cardiovascular Radiology, North American Edition. W. Richard Webb, Charles B. Higgins. Lippincott Williams & Wilkins, 2010 : 255
2. Takeshi J, Nestor LM, Kazuya I et al. Intrathoracic multicentric Castleman disease: CT Findings in 12 patients. Radiology 1998;209:477-481
- Keywords
- lung, lymphoproliferative disease,