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Castleman disease. Histopathological and immunohistochemical analysis of 39 cases
Diana Brisa Sevilla-Lizcano1, Christian Lizette Frias-Soria1, Carlos Ortiz-Hidalgo1,2
1Departamento de Patología Quirúrgica y Molecular, Centro Médico ABC.
Insights
Castleman disease (CD) is a rare lymphoproliferative disorder with distinct subtypes. This study analyzed 39 cases, revealing histopathologic and immunohistochemical differences that aid in classifying CD for varied outcomes.
Area of Science:
- Pathology
- Immunohistochemistry
- Oncology
Background:
- Castleman disease (CD) is a rare lymphoproliferative disorder with unicentric and multicentric clinical subtypes.
- Histopathologic patterns include hyaline-vascular (HV) and plasma-cell (PC) types, with some multicentric PC cases linked to HHV-8 infection.
Purpose of the Study:
- To characterize the histopathologic and immunohistochemical features of 39 Castleman disease cases.
- To evaluate specific markers for dendritic cell populations in CD subtypes.
Main Methods:
- Retrospective review of 39 Castleman disease cases diagnosed at ABC Medical Centre.
- Detailed paraffin immunophenotypic analysis of 9 cases using antibodies for desmin, cytokeratin OSCAR (CO), and Epidermal growth factor receptor (EGFR).
- Immunostaining for CD20, CD3, and CD21 to assess immune cell distribution.
Main Results:
- Of 39 cases, 24 were HV (all unicentric) and 15 were PC (one multicentric). Lymph nodes were the most common site for both subtypes.
- Immunohistochemistry revealed characteristic immune cell staining patterns (CD20, CD3, CD21) and differential expression of EGFR, desmin, and CO in FRC/FDC.
- One PC case tested positive for HHV-8. EGFR was expressed in FDC in most cases, while desmin was positive in FRC.
Conclusions:
- Histopathological and immunohistochemical findings, including marker expression (EGFR, desmin, CO), differentiate CD subtypes.
- Clinical, histopathological, and viral status markers are crucial for classifying CD into prognostically distinct groups.
Introduction:
Castleman disease (CD) is a rare lymphoproliferative that comprises two distinct clinical subtypes (unicentric and multicentric) and has two basic histopathology patterns that are hyaline-vascular (HV) and plasma-cell (PC) type. Some cases of multicentric PC disease are associated with HHV-8 infection.
Objective:
To present the histopathologic and immunohistochemical characteristics of 39 cases of CD.
Methods:
A review of cases with the diagnosis CD from the files of the Department of Pathology of the ABC Medical Centre in Mexico City was performed. Thirty-nine cases of CD were identified, and a detailed paraffin immunophenotypic study of 9 of them was completed using desmin, cytokeratin OSCAR (CO) and Epidermal growth factor receptor (EGFR), to evaluate the dendritic cell population.
Results And Conclusions:
Of the 39 cases of CD, 24 were HV and 15 CP. All HV cases were unicentric and only one case of CP was multicentric. The most frequent localization in both subtypes was in lymph nodes; 21/24 cases in HV and 15 cases of CP. All cases were immunostained with CD20 that was expressed in the germinal centers (CGs), CD3 in the paracortical zone, and CD21 in follicular dendritic cells (CDF) within CGs, with expansion towards the area of the hyperplastic mantle zone (only in the HV variant). One case of CD CP was positive for HHV-8. Of the nine cases (6 HV and 3 PC cases) that were detailed with IHC, we found EGFR expression in FDC in all but one of the 9 cases studied and desmin was positive in fibroblastic reticulum cells (FRC) in all, but one of the cases of CD. CO was positive FRC in 3 of 6 cases of HV type and all (3) of the PC type. Clinical, histopathological and HIV and HHV-8 status markers, allow for the classification of CD into groups with markedly different outcomes and disease associations.
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