UNICENTRIC CASTLEMAN DISEASE (LOCALIZED)
Unicentric or “localized” CD is the most common form of Castleman disease. It generally affects children and young adults.
* It triggers the development of benign tumours in the lymph nodes.
* It is not a type of cancer.
* This disease is not contagious or hereditary.
Discovery
The disease is usually discovered when an enlarged lymph node (adenopathy) is found at the side of the neck, the underarm or groin area. The lymph node may be large and uncomfortable but is not generally painful. The enlarged lymph node can be internal and may be discovered by chance during a radiological examination (X-ray, ultrasound, scan) of the chest or abdomen.
The disease is sometimes detected by signs of inflammation: fever, sweats, weight loss, failure to thrivein children or unexplained anaemia.
Diagnosis
A diagnosis is made after examining a sample (biopsy) taken from the affected lymph node.
This sample may be taken by a surgeon (minor operation under local anaesthesia) or by a radiologist who inserts a needle into the lymph node.
The lesions characteristic of Castleman disease can be detected by analysing this sample.
The doctor in charge of this analysis (anatomopathologist) will then indicate or confirm the diagnosis.
Initial assessment
Based on the biopsy report which indicates this diagnosis, the doctor in charge will propose various tests to confirm the diagnosis and define type of Castleman disease concerned.
- The doctor will be systematically looking for anaemia and signs of inflammation by examining a blood sample. The patient will also be screened for HIV infection. These examinations are generally normal or negative in this form of Castleman disease.
- A radiological examination of the whole body (scan or PET scan) can confirm whether a single lymph node is affected or whether it is localised with a number of other lymph nodes in the same region.
Treatment
The standard treatment is surgery. Complete recovery is usually possible if the entire lymph node is removed during surgery. The risk of relapse may be greater in forms associated withfever or signs of inflammation.
In some cases, surgery is not possible or too risky, particularly when the lymph node is in a difficult position. In this case, it may be possible to try to reduce the size of the lymph node. There are several possible treatments, but the results are uncertain and variable (see “treatment” sheet):
- Corticosteroids
- Rituximab
- Siltuximab or Tocilizumab
- Cyclophosphamide
- Embolization, or the occlusion of the blood vessel which irrigates the lymph node by means of a probe inserted into this vessel by a specialist radiologist, may be carried out before surgery to make it easier or as stand-alone treatment if surgery is not possible.
- Radiotherapy is also an option. The results are promising, but it is important to assess the potential long-term risk depending on age, location and radiation field.
- For stable, non-symptomatic lesions, it would seem that simple monitoring may also be envisaged as an alternative to close monitoring.
Outcome
The general prognosis is excellent, with no impact on life expectancy.