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Castleman disease

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.  

Epidemiology

The condition can potentially present at any age but typically presents in the 3rd to 4th decades.

Pathology

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 subtypes based on histolopathology 1-2

  • hyaline vascular
    • most common ~ 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
Location

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.

Associations

Radiographic features

CT
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
  • hepatosplenomegaly
  • ascites
MRI

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
  • low values on ADC may be encountered 13-14
FDG - PET(/CT)

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 multi-centric 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.

Etymology

The condition was first published by Benjamin Castleman in 1954 4.

Differential diagnosis

For thoracic lesions consider

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