대한흉부영상의학회 Korean Society of Thoracic Radiology

Discussion

Diagnosis With Brief Discussion

Diagnosis

Multicentric Castleman's Disease with Lung Involvement (Lymphocytic Interstitial Pneumonitis)

Radiologic Findings

PA chest radiograph shows small and ill-defined nodular opacities, increased interstitial marking in both lungs and the thickened right minor fissure. Chest CT obtained on the same day shows small nodules, ground-glass opacities, interlobular septal thickening and thin walled cysts in both lungs. Bilateral hilar and subcarinal lymphadenopathies are also noted.
The diagnosis of Castleman’s disease, plasma cell type was made by biopsy of left inguinal lymph node.

Brief Review

Castleman’s disease, also known as angiofollicular hyperplasia or giant lymph node hyperplasia, is an uncommon benign lymphoproliferative disorder characterized by hyperplasia of lymphoid follicles. On the basis of histologic criteria, it has been divided into two types: hyaline vascular and plasma cell. The hyaline vascular type is characterized by hyperplasia of lymphoid follicles with germinal center formation and the presence of numerous capillaries with hyalinized walls. This type accounts for approximately 90% of cases and usually manifests as a solitary perihilar or mediastinal mass in asymptomatic patients. The plasma cell type is characterized by the presence of relatively few capillaries as well as the presence of mature plasma cells between the hyperplastic germinal centers. Although in the majority of Castleman’s disease, it is usually localized, a multicentric form has been recognized clinically and histologically. It affects individuals of all ages, but the peak incidence is in the fifth decade of life. Female patients are more commonly affected than male by a ratio of 2:1. Most patients presents with systemic complaints of fever, weight loss and anemia. The enlarged lymph nodes seen in the plasma cell type shows relatively low level of enhancement after an injection of contrast material than in the hyaline vascular type. Histologic findings obtained from lung biopsy in patients who had multicentric Castleman’s disease 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. The majority of multicentric Castleman’s disease was the plasma cell type, and those who affected by multicentric type typically had a much worse prognosis than those with localized Castleman’s disease. Multicentric Catleman disease is currently regarded as a potentially malignant lymphoproliferative disorder.

References

1. Takeshi J, Nestor LM, Kazuya I et al. Intrathoracic multicentric Castleman disease: CT Findings in 12 patients. Radiology 1998;209:477-481
2. McAdams HP, Christenson MR, Fishback NF, Templeton PA Castleman disease of the thorax: Radiologic features with clinical and histopathologic correlation. Radiology 1998;209:221-228

Keywords

Lung, Mediastinum, Lymphproliferative disorder, LIP,