Lymph Nodes, Castleman’s disease [LMP74593]

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Diagnostic Modality: 
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Disease Category: 
Diseases of the Immune System
Diagnosis: 
Castleman’s disease
Species: 
Human
Organ System: 
White Blood Cells, Lymph Nodes, Spleen, and Thymus
Gender: 
Female
Age: 
45 years
Diagnosis Code: 
ICD-10: D47.4
Slide Contributor Information
Papp, S., Lu, F.

Keywords

giant lymph node hyperplasia, angiofollicular hyperplasia

Clinical History

Retroperitoneal mass.

Clinical Discussion

Castleman’s disease is a rare lymphoproliferative disorder, which in itself is not malignant but may increase the risk of developing other malignancies. Histologically, Castleman’s disease may be classified as either the hyaline-vascular or plasma cell variant, with occasional cases demonstrating mixed features. It may be unicentric or multi-centric, and the hyaline-vascular histology accounts for most unicentric cases, with interleukin 6 (IL-6) playing a major role in its pathogenesis. Histopathologic features include: an increased proportion of follicular dendritic cells relative to lymphocytes within germinal centers, known as follicle regression; concentric arrangement of mantle zone lymphocytes in an "onion skin" pattern; and hypervascularity of follicles, some of which are radially penetrated by a hyalinized blood vessel, resembling a lollipop.