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The clinicopathological features and survival of Castleman disease: a multicenter Turkish study
1Department of Hematology, Yenimahalle Training and Research Hospital, Yildirim Beyazit University, Ankara, Turkey. dr.jalevardi@hotmail.com.
Insights
Castleman disease (CD) subtypes, namely unicentric (UCD) and multicentric (MCD), present distinct clinical features. Both UCD and MCD show promising survival rates with appropriate treatment, though further research is needed.
Area of Science:
- Oncology
- Pathology
- Internal Medicine
Background:
- Castleman disease (CD) is a rare lymphoproliferative disorder with poorly understood clinicopathological features.
- Understanding the differences between unicentric Castleman disease (UCD) and multicentric Castleman disease (MCD) is crucial for effective management.
Purpose of the Study:
- To investigate the clinicopathological characteristics and survival outcomes of patients with Castleman disease.
- To differentiate between the clinical presentations and prognoses of UCD and MCD.
Main Methods:
- Retrospective analysis of 33 patients diagnosed with CD across six Turkish centers.
- Evaluation of patient demographics, subtype (UCD/MCD), histological variant (hyaline vascular), and site of involvement.
Main Results:
- The study included 33 patients (51.5% female, median age 49). The hyaline vascular subtype was most common (54.5%), with UCD being the predominant subtype (60.6%).
- Head and neck involvement was most frequent (57.5%). UCD patients were younger than MCD patients (p=0.027).
- MCD showed higher rates of visceral lymph node involvement, hepatomegaly, and splenomegaly compared to UCD (p=0.001, p=0.035, p=0.013, respectively). No deaths occurred during a median follow-up of 19.5 months.
Conclusions:
- Unicentric CD and multicentric CD represent distinct clinical entities.
- Surgical and systemic treatments offer promising survival outcomes for both UCD and MCD subtypes.
- Well-designed prospective studies are needed to further validate these findings for this rare disease.
Objective:
In this study, we aimed to investigate the clinicopathological features and survival of CD, which is quite rare and has many unknowns.
Patients And Methods:
This study was conducted by retrospectively evaluating patients diagnosed with CD in six different centers in Turkey.
Results:
The median age of 33 patients included in the study was 49 and 51.5% (n = 17) of these patients were women. 18 (54.5%) patients were in the hyaline vascular subtype and most of the patients were UCD (n = 20, 60.6%). The most common involvement region was head and neck (n = 19, 57.5%). The UCD group was younger than the MCD group (p=0.027). Visceral lymph node involvement was higher in MCD than in UCD (p=0.001). Similarly, it was observed that there was more hepatomegaly (p=0.035) and splenomegaly (p=0.013) in the MCD group. During the median 19.5 months follow-up period, there were no patients who died.
Conclusions:
It was observed that UCD and MCD are different clinical entities. Promising survival times can be achieved with surgical and systemic treatments in both subtypes of this extremely rare disease. However, this result should be supported by well-designed prospective comprehensive studies.
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