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Neuroradiology Case of the Week
Case 415
June 2009
Gurshawn Singh, MS2, and
Rajiv Mangla, MD
Clinical
Presentation: Patient is a 20-year-old female with lymphadenopathy.
Imaging Findings: On CT, multiple enlarged well-defined discrete lymph nodes were seen in the neck which showed homogenous enhancement on post-contrast images. Chest CT showed axillary lymphadenopathy. Mediastinal lymph nodes were not enlarged. The abdominal CT revealed hepatosplenomegaly with few enlarged paraaortic lymph nodes.
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Figures 1A&B: Axial post-contrast CT of neck shows multiple homogeneously enlarged enhancing lymph nodes in the neck.
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Figure 2: Axial CT of upper abdomen shows marked splenomegaly.
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| Figure 3: Axial CT of upper chest shows bilateral axillary lymph nodes. The mediastinal lymph nodes were not enlarged.
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Diagnosis: Multicentric Castleman's disease
Discussion: Castleman’s disease is a uncommon benign lymphoproliferative disorder also known as angiofollicular hyperplasia because of the increased interfollicular vascularity within follicular hyperplastic lymph nodes. There are two separate types of the disease, unicentric and multicentric. This disease can occur at any age with unicentric being more common in third or forth decade and multicentric in older age group.
Unicentric Castleman’s disease is slow growing benign mass in the mediastinum with no classic symptoms. Multicentric Castleman’s disease is characterized by lymphadenopathy involving peripheral lymph-nodes and involvement of multiple organs systems, such as hepatosplenomegaly in some cases due to overproduction of acute phase reactants, most notably IL-6. Often, multicentric Castleman’s disease is symptomatic and can be mistaken for lymphoma.
It can rarely be associated with Kaposi’s sarcoma, non-Hodgkin’s lymphoma, and Hodgkin lymphoma. Castleman’s disease patients present with hyperplastic mediastinal lymph nodes with small germinal centers. For multicentric Castleman’s disease, radiographs will show marginating masses with possible bilateral mediastinal widening. Smooth lobulated lesions may also be present along with axillary masses. CT usually shows enlarged lymph nodes with some soft-tissue homogenous attenuation without contrast, and with contrast will show nodal enhancement. MR T1 images are usually heterogeneous with more signal intensity when compared to skeletal muscle. MR T2 images show lesions that are hyperintense.
References:
- Menezes BF, Morgan R, Azad M. Multicentric Castleman's disease: a case report. J Med Case Reports. 2007 Sep 5;1:78. [PubMed]
- 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. [PubMed]
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