Castleman disease (CD) (also known as angiofollicular lymph node hyperplasia or giant lymph node hyperplasia ) is an uncommon benign lymphoproliferative condition. It can affect several regions of the body although commonly described as a solitary mediastinal mass.
The condition can potentially present at any age but typically presents in the 3rd to 4th decades.
The disease is of unknown etiology but the most widely accepted theory is that Castleman's disease is a chronic low-grade inflammatory process.
The disease is characterised by hypervascular lymphoid hyperplasia. There are two distinct sub-types of Castleman disease : uni-centric (UCD) and multi-centric (MCD). Uni-centric disease is more common
There are also several recognised sub types based on histolopathology 1-2
hyaline vascular :
- commoner ~ 90%
- more uni-centric
plasma cell :
- often multi-centric
- 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 %
Multi-centric disease may involve all of the above, and is associated with a more complex clinical course and poorer prognosis.
For mediastinal lesions : CT chest
- commonly seen as a mediastinal mass and rarely as matted lymphadenopathy (with or without a dominant mass) in a single mediastinal compartment
- typical arborising calcification may be seen within the mass
- typically shows intense homogeneous enhancement following contrast
- dynamic CT demonstrates early rapid enhancement with washout in the delayed phase
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 ( > 5cm) may demonstrate central hypo-attenuation consistent with necrosis.
- variable pattern of calcification, including arborising calcification.
For multi-centric disease : multi-region CT
- bilateral hilar and mediastinal lymphadenopathy
- centrilobular nodules
- diffuse abdominal lymphadenopathy
General signal characteristics include
- T1 - iso to hyper intense relative to skeletal muscle
- T1 C+ (Gd) - shows enhancement
- T2 - arborsing calcification may be seen as low signal
FDG - PET
There is some evidence that Castleman disease is FDG avid, and therefore 18F-FDG PET may be useful in identifying the extent of multi-centric disease and for monitoring disease progression 7.
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.
The condition was first published by Benjamin Castleman in 1954 4
For thoracic lesions consider
- If antero-superior : consider - differential for an antero-superior medistinal mass
- If posterior : consider - differential for a posterior medistinal 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
Synonyms & Alternative Spellings
|Synonyms or Alternative Spelling||Include in Listings?|
|Angiofollicular lymph node hyperplasia||✓|
|Giant lymph node hyperplasia||✓|