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Multimodality imaging and clinical features in Castleman disease: single institute experience in 30 patients
A J Hill1, S H Tirumani, M H Rosenthal
11 Department of Radiology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.
Insights
Imaging features of Castleman disease (CD) subtypes were analyzed to differentiate from lymphoma. Calcification and heterogeneous enhancement on CT were key differentiating features, though assessing lymphoma risk in CD patients remains challenging.
Area of Science:
- Radiology
- Oncology
- Pathology
Background:
- Castleman disease (CD) is a rare lymphoproliferative disorder with diverse subtypes and clinical presentations.
- Distinguishing CD from lymphoma, particularly in multicentric forms, can be challenging based solely on imaging.
- Understanding imaging characteristics is crucial for accurate diagnosis and patient management.
Purpose of the Study:
- To analyze the imaging features of various Castleman disease (CD) subtypes.
- To identify differentiating imaging characteristics between CD and lymphoma.
- To explore imaging findings suggestive of lymphoma development in CD patients.
Main Methods:
- Retrospective analysis of pre-treatment imaging (CT, PET/CT, MR, ultrasound) in 30 histopathologically confirmed CD patients.
- Review of imaging in three cases that subsequently developed lymphoma.
- Consensus review by two radiologists to assess nodal distribution, calcification, and enhancement patterns.
Main Results:
- Hyaline-vascular subtype was most common (n=18).
- Unicentric CD involved thoracic, abdominal, and cervical nodes; multicentric CD showed broader distribution including inguinal and axillary.
- CT revealed calcification (26.7%) and heterogeneous enhancement (19.2%) as differentiating features from lymphoma. PET/CT showed FDG avidity. Ultrasound showed hypoechoic nodes. MR showed variable signal intensity and homogeneous enhancement.
- Three patients developed non-Hodgkin's lymphoma, with splenomegaly, effusions, or ascites noted in these cases.
Conclusions:
- Castleman disease can present as unicentric or multicentric, affecting lymph nodes above and below the diaphragm.
- Imaging features like calcification and heterogeneous enhancement can aid in differentiating CD from lymphoma.
- While challenging, the development of lymphoma in CD patients may be suggested by splenomegaly, pleural effusion, and ascites.
Objective:
To analyse imaging features of subtypes of Castleman disease (CD), emphasizing differentiating features from lymphoma.
Methods:
Institutional review board-approved, Health Insurance Portability and Accountability Act compliant, retrospective study examined 30 patients with CD. 30 patients (females, 20; mean age, 46 years; range, 22-87 years) with histopathologically confirmed CD and pre-treatment imaging formed the analytic cohort. Imaging at presentation in all patients [CT, 30; positron emission tomography (PET)/CT, 5; MR, 4; ultrasound, 3] and subsequent imaging in three cases that developed lymphoma was reviewed by two radiologists in consensus.
Results:
Subtypes: hyaline-vascular (n = 18); multicentric not otherwise specified (NOS) (n = 6); human herpesvirus 8 associated (n = 2); mixed unicentric (n = 2); pure plasma-cell variant (n = 1); and unicentric NOS (n = 1). Distribution: unicentric (n = 17); and multicentric (n = 13). Nodal sites-unicentric: 13 thoracic, 3 abdominal and 1 cervical; multicentric: 9 abdominal, 8 thoracic, 6 cervical, 5 inguinal, 4 axillary and 4 supraclavicular. On CT, differentiating features from lymphoma were calcification (n = 8; 26.7%) and heterogeneous enhancement (n = 5; 19.2%). No association between CD subtype, degree or enhancement pattern, or calcification was noted. On PET/CT (n = 5), nodes were typically fluorine-18 fludeoxyglucose avid (n = 4). On ultrasound (n = 3), nodes were hypoechoic, homogeneous with posterior acoustic enhancement. On MR (n = 4), nodes were hypointense (n = 2) to isointense (n = 2) on T1 weighted images and isointense (n = 1) to hyperintense (n = 3) on T2 weighted images. All (n = 4) demonstrated homogeneous enhancement. Three cases developed non-Hodgkin's lymphoma, two of the three had larger spleens, and these cases had effusions/ascites.
Conclusion:
CD can be unicentric or multicentric and involve nodes above and below the diaphragm. Patients with CD can develop lymphoma.
Advances In Knowledge:
Assessing individual risk of developing lymphoma in patients with CD is difficult, although the findings of splenomegaly, pleural effusion and ascites may be suggestive.

