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Castleman disease

Dr MT Niknejad and Dr Yuranga Weerakkody et al.

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 3^rd to 4^th 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 two recognised sub types based on histology ^1,2

hyaline vascular :
- commoner ~ 90%
- more uni-centric
plasma cell :
- often multi-centric
- less enhancing
- may be more symptomatic ⁵

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

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.