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Published on: December 15, 2011
Castleman's disease of the retroperitoneum: with special reference to IgG4-related disorder
Masaru Kojima1, Naoya Nakamura, Tadashi Motoori
1Department of Anatomic and Diagnostic Pathology, Dokkyo University School of Medicine. mkojima@dokkyomed.ac.jp
Insights
Localized Castleman
Area of Science:
- Pathology
- Immunology
- Oncology
Background:
- Localized Castleman's disease (CD) presents as hyaline vascular (HV) or plasma cell (PC) types.
- Recent findings link retroperitoneal PC-type CD to IgG4-related disorder.
- Further investigation of retroperitoneal CD clinicopathology is warranted.
Purpose of the Study:
- To clarify clinicopathological findings of Castleman's disease in the retroperitoneum.
- To investigate the association between retroperitoneal PC-type CD and IgG4-related disorder.
- To differentiate HV and PC types of retroperitoneal CD.
Main Methods:
- Retrospective analysis of eight retroperitoneal CD cases.
- Histopathological examination including immunohistochemistry for IgG4.
- Assessment of serum IgG4 and interleukin-6 levels.
Main Results:
- 50% of retroperitoneal CD cases were PC type, contrasting with the typical HV predominance.
- HV type lesions were primarily lymph node-based, while PC type presented as soft tissue masses.
- Three PC-type cases showed numerous IgG4+ plasma cells, and two had elevated serum IgG4.
Conclusions:
- Retroperitoneal PC-type Castleman's disease frequently exhibits features of IgG4-related disorder.
- Clinicopathological presentation of retroperitoneal CD differs significantly from previous descriptions.
- IgG4-related disorder should be considered in the differential diagnosis of retroperitoneal PC-type CD.
Abstract:
Localized Castleman's disease (CD) has been divided two types, the classical hyaline vascular (HV) type and the rare plasma cell (PC) type. Recently, we have reported two cases of IgG4-related disorder of the retroperitoneum showing PC type of CD. To further clarify the clinicopathological findings of CD of the retroperitoneum, eight such cases have been studied. A single lesion was located in the retroperitoneum (n=3), ureter (n=2) and renal hilum (n=2). One case had bilateral ureter lesions. The HV type of CD accounts for approximately 90% of cases. However, 50% (n=4) of our cases were the PC type of CD. Three of the four lesions of HV type had lymph node lesions, whereas all four PC type of CD were soft tissue masses. These clinicopathologic findings appear quite different from previous descriptions. Immunohistochemical study demonstrated numerous IgG4(+) plasma cells accounting for more that 50% of IgG4(+) cells in three cases of the four PC type of CD. Moreover, serum IgG4 concentration was increased in two of the four cases of PC type of CD that were examined. The serum interleukin-6 levels were within the normal range in two cases of PC type that were examined. The present study suggests that a majority of the PC type of CD arising in the retroperitoneum appears to be an IgG4-related disorder.
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