This site is targeted at medical and radiology professionals, contains user contributed content and material that may be confusing to a lay audience. Use of this site implies acceptance of our Terms of Use.

Castleman disease

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:

  • hyaline vascular
    • most common ~90%
    • more uni-centric
  • plasma cell
    • 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.


Radiographic features

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
  • hepatosplenomegaly
  • ascites

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.

Differential diagnosis

For thoracic lesions consider

Updating… Please wait.


Error Unable to process the form. Check for errors and try again.

Alert_accept Thank you for updating your details.