Castleman disease (CD), also known as angiofollicular lymph node hyperplasia or giant lymph node hyperplasia, is an uncommon benign B cell lymphoproliferative condition. It can affect several regions of the body although commonly described as a solitary mediastinal mass.
The hyaline vascular type typically occurs in children and young adults and plasma cell type in older population (5th and 6th decades).
The disease is of unknown aetiology but the most widely accepted theory is that Castleman disease is a chronic low-grade inflammatory process.
The disease is characterised by hypervascular lymphoid hyperplasia. There are two distinct subtypes of CD:
- unicentric disease (UCD) (more common)
- multicentric disease (MCD)
There are also several recognised subtypes based on histopathology 1-2:
- most common ~90%
- more uni-centric
- often multicentric
- less enhancing
- may be more symptomatic 5
- HHV-8–associated Castleman disease 8
- multicentric Castleman disease not otherwise specified
The distribution is as follows:
- thorax: ~70%
- abdomen/pelvis and retroperitoneum: 10-15%
- neck: 10-15%
Multicentric disease may involve all of the above, and is associated with a more complex clinical course with systemic symptoms including fever and organomegaly.
- POEMS syndrome
- osteosclerotic myeloma
- Kaposi sarcoma
- AIDS: especially multicentric 9
- amyloidosis 10
- chordoid meningioma
For mediastinal lesions: CT chest
- commonly seen as a solitary mediastinal mass, infiltrative mass, multiple lymph nodes or rarely as matted lymphadenopathy (with or without a dominant mass) in a single mediastinal compartment
- typical arborising calcification may be seen within the mass in 15% of cases
- hyalin vascular type typically shows intense homogeneous enhancement following contrast
- dynamic CT demonstrates early rapid enhancement with washout in the delayed phase
- plasma cell type are multicentric and are less enhancing 8
For abdominal lesions: CT abdomen
- most commonly, a single well defined abdominal mass
- location is variable, and includes retroperitoneum, mesentery and porta hepatis 3
- enhancement is homogeneous, or in larger lesions (> 5 cm) may demonstrate central hypoattenuation consistent with necrosis.
- variable pattern of calcification, including arborising calcification.
For multicentric disease: multi-region CT
- bilateral hilar and mediastinal lymphadenopathy
- centrilobular nodules
- diffuse abdominal lymphadenopathy
General signal characteristics include:
- T1: iso to hyperintense relative to skeletal muscle
- T1 C+ (Gd): shows enhancement
- T2: arborising calcification may be seen as low signal
- low values on ADC may be encountered 13-14
There is growing evidence that active Castleman disease is FDG avid, why 18F-FDG PET(/CT) is more sensitive than CT alone and may be an effective diagnostic tool, especially if performed with hybrid imaging part SOA contrast-enhanced CT 12. Discrimination between uni- and multicentric disease, mapping of the extent of disease and monitoring disease progression seems possible, especially if confirmed in larger series 7,12.
Treatment and prognosis
For unicentric Castleman disease treatment is surgical, with good prognosis (can be curative).
Multicentric Castleman disease may be treated with any combination of surgery, chemotherapy and prednisolone 6. Prognosis is relatively poorer.
History and etymology
The condition was first published by Benjamin Castleman in 1954 4.
For thoracic lesions consider
- if antero-superior, consider: differential for an anterosuperior mediastinal mass
- if posterior, consider: differential for a posterior mediastinal mass
- 1. Shin JH, Lee HK, Kim SY et-al. Castleman's disease in the retropharyngeal space: CT and MR imaging findings. AJNR Am J Neuroradiol. 2000;21 (7): 1337-9. AJNR Am J Neuroradiol (full text) - Pubmed citation
- 2. Ko SF, Hsieh MJ, Ng SH et-al. Imaging spectrum of Castleman's disease. AJR Am J Roentgenol. 2004;182 (3): 769-75. AJR Am J Roentgenol (full text) - Pubmed citation
- 3. Meador TL, Mclarney JK. CT features of Castleman disease of the abdomen and pelvis. AJR Am J Roentgenol. 2000;175 (1): 115-8. AJR Am J Roentgenol (full text) - Pubmed citation
- 4. Castleman B, Towne VW. Case records of the Massachusetts General Hospital: case 40011. N Engl J Med 1954;250:26 -30
- 5. Keller AR, Hochholzer L, Castleman B. Hyaline-vascular and plasma-cell types of giant lymph node hyperplasia of the mediastinum and other locations. Cancer. 1972;29 (3): 670-83. - Pubmed citation
- 6. Herrada J, Cabanillas F, Rice L et-al. The clinical behavior of localized and multicentric Castleman disease. Ann. Intern. Med. 1998;128 (8): 657-62. doi:10.1059/0003-4819-128-8-199804150-00010 - Pubmed citation
- 7. Enomoto K, Nakamichi I, Hamada K et-al. Unicentric and multicentric Castleman's disease. Br J Radiol. 2007;80 (949): e24-6. doi:10.1259/bjr/93847196 - Pubmed citation
- 8. Bonekamp D, Horton KM, Hruban RH et-al. Castleman disease: the great mimic. Radiographics. 2011;31 (6): 1793-807. doi:10.1148/rg.316115502 - Pubmed citation
- 9. Barker R, Kazmi F, Bower M. Imaging in multicentric Castleman's disease. J HIV Ther. 2008;13 (3): 72-4. - Pubmed citation
- 10. Haaga JR, Boll D. CT and MRI of the whole body. Mosby. (2009) ISBN:0323053750. Read it at Google Books - Find it at Amazon
- 11. Cronin DM, Warnke RA. Castleman disease: an update on classification and the spectrum of associated lesions. Adv Anat Pathol. 2009;16 (4): 236-46. doi:10.1097/PAP.0b013e3181a9d4d3 - Pubmed citation
- 12. Lee ES, Paeng JC, Park CM et-al. Metabolic characteristics of Castleman disease on 18F-FDG PET in relation to clinical implication. Clin Nucl Med. 2013;38 (5): 339-42. doi:10.1097/RLU.0b013e3182816730 - Pubmed citation
- 13. Oida Y, Shimizu K, Mukai M et-al. FDG-PET and diffusion-weighted MR imaging appearance in retroperitoneal Castleman's disease: a case report. Clin Imaging. 2008;32 (2): 144-6. doi:10.1016/j.clinimag.2007.08.020 - Pubmed citation
- 14. Nakayama T, Yoshimitsu K, Irie H et-al. Usefulness of the calculated apparent diffusion coefficient value in the differential diagnosis of retroperitoneal masses. J Magn Reson Imaging. 2004;20 (4): 735-42. doi:10.1002/jmri.20149 - Pubmed citation
Synonyms & Alternative Spellings
|Synonyms or Alternative Spelling||Include in Listings?|
|Angiofollicular lymph node hyperplasia||✓|
|Giant lymph node hyperplasia||✓|