Introduction

Unicentric Castleman disease (UCD) is a rare condition in which a single lymph node, or a small cluster in one area, becomes enlarged. It is not cancer, but the swollen node can press on nearby organs and sometimes cause health problems. In many cases, people may not notice any symptoms until it is picked up during a scan.¹ 

The positive news is that UCD is often treatable, and surgery can provide a complete cure for many people. When surgery is not possible, alternative treatments like radiotherapy or medication may be used. This article looks at the different treatment options and what they involve. 

Understanding Unicentric Castleman Disease? 

What is Unicentric Castleman Disease? 

Castleman’s disease is a rare condition that is characterised by the unusual overgrowth of tissue within the lymph nodes. For some individuals, it develops in multiple lymph nodes throughout the body, while for others, it is limited to a single area.¹ When the condition is limited to a single lymph node or a small cluster in one location, it is called unicentric Castleman disease (UCD). The enlarged lymph nodes usually develop in the chest, abdomen, neck, or pelvis, but they may also occur in other parts of the body.² 

Many people with UCD do not notice any symptoms, and the swollen lymph node is often found by accident during a scan or check-up for something else. If the node becomes large enough, it can press on nearby organs or tissues, causing problems such as pain, pressure, or discomfort. Less commonly, people may notice weight loss, night sweats, or fever.⁴ 

To determine if an enlarged lymph node is UCD, doctors typically start with imaging tests such as CT scans, MRI scans, ultrasounds, or PET scans. Scans are useful for identifying the affected lymph node and planning follow-up tests, but a biopsy is the most reliable way of confirming UCD. A biopsy involves removing a tiny sample from the lymph node, which doctors examine under a microscope to rule out other conditions such as lymphoma.⁵  

Treatment Options In Unicentric Castleman Disease 

Other Treatment Options Before Surgery 

Medication-Based Treatments 

If surgery cannot be carried out straight away, doctors may use medications to help manage UCD. While these medicines can be helpful, their effects are usually temporary. They can help to stabilise the condition or reduce symptoms until surgery is possible.

One option is rituximab, a type of immunotherapy that targets specific immune cells. In some cases, it can shrink the enlarged lymph node, which makes surgery easier and safer.⁶ Other medicines, such as anti-inflammatory drugs, can also be used to help with any pain or discomfort from the swollen lymph node.  

While treatments like rituximab or anti-inflammatory medicationscan help shrink the node or ease discomfort, they do not cure UCD. Their main purpose is to manage symptoms and keep things under control until surgery can be done. Research shows that people respond differently to these medicines, so their effects can be limited.³

Radiation Therapy

Radiation therapy is considered for patients who are not suitable for surgery or for those whose previous surgery did not completely remove the lymph node.⁷

In radiation therapy, targeted beams of energy are aimed at the lymph node. This can shrink the node by damaging the overgrowing cells and relieve any related discomfort or pressure. Radiation therapy has been shown to be effective for UCD, often shrinking the lymph node, and in nearly half of reported cases, the node disappeared completely after treatment.⁸ 

Radiation therapy is usually well-tolerated, but it can sometimes cause side effects like fatigue, skin changes, or soreness in the area being treated. Rarely, it can lead to longer-term effects, like swelling or irritation in nearby tissues.⁹ Even so, radiation can be a very effective way to reduce or remove the lymph node when surgery is not an option. 

Surgery: The Curative Option 

Why Surgery Is Usually Recommended 

Surgery is the optimal treatment for UCD and is often recommended as the first option, as it removes the problem at its source: the enlarged lymph node. Other treatments, like medicines or radiation mentioned above, can help to shrink the node and ease discomfort, but they rarely provide a permanent solution. Surgery, however, allows doctors to completely remove the node, which is why it is generally the first choice when the node can be reached safely.¹⁰ 

In some situations, surgery may not be the immediate option. If the lymph node is in a sensitive position, such as close to a large blood vessel, doctors may suggest using medication or radiation first to make the procedure safer. Even in these cases, surgery is usually the end goal, as it gives the best chance of long-term recovery.¹¹ 

How the Procedure Is Done

Surgery for UCD involves removing the affected lymph node. The surgical approach can vary depending on where the node is located in the body and how easily it can be reached. In most cases, doctors perform the surgery through a regular surgical cut. For nodes in areas more difficult to reach, minimally invasive techniques such as laparoscopy or thoracoscopy may be used.¹¹ The goal is always to remove the entire lymph node safely, which is usually straightforward and well-tolerated. 

Benefits of Surgery

Removing the affected lymph node usually leads to a full recovery for patients with UCD, with a low likelihood of the disease recurring. Many patients notice that their symptoms, like swelling or fatigue, improve soon after surgery. Removing the affected lymph node also removes the source of the problem. Although recurrence is rare, regular follow-ups are important to continue monitoring your health and catch any changes early. Research shows that removing the lymph node completely leads to long-lasting results and maintains patients’ well-being over time.¹¹ 

Recovery and Follow-Up 

Recovery After Surgery

Most people recover quickly after the lymph node is removed. Pain and fatigue are common symptoms, but they typically resolve within days. Light activities can be resumed soon, while heavier tasks may take a bit longer.¹²

Managing Discomfort and Strength 

Follow your doctor’s instructions, rest as needed, stay hydrated, and eat a balanced diet. Watch out for any unusual swelling, redness, or fever, and contact your healthcare provider if these symptoms occur.  

Monitoring Your Health 

Attend regular follow-ups so your doctor can check your recovery and ensure the disease has not returned. 

FAQs

Can UCD come back after surgery? 

UCD rarely recurs once the lymph node is completely removed, but it is still important to attend regular follow-ups so your doctor can check for any changes early. 

Is surgery safe for everyone? 

Surgery is usually safe, but your doctor will need to assess factors such as your age and other health conditions to decide if it is safe and the right option for you. 

How long is recovery? 

This can vary from person to person. However, most people recover within a few days to a couple of weeks. Some fatigue or mild discomfort may persist. Following the postoperative advice provided by your healthcare team can help speed your recovery. 

Are there support groups or resources for people affected by Castleman disease? 

Yes, there are organisations and online communities that are set up to offer guidance and support. Your healthcare providers are also available to support you. Connecting with these groups can provide you with useful information and support throughout your treatment and recovery process.

Summary 

Unicentric Castleman disease can be managed in several ways, but surgery to remove the affected lymph node is often the most effective approach and usually curative. Other treatments, like medicines or radiation therapy, can help to manage patients' symptoms or to shrink the lymph node before surgery, but they alone rarely provide a full cure. Recovery after surgery is generally a smooth process, though some discomfort and fatigue may be experienced in the first few days. Attending follow-up appointments is crucial to ensure the disease does not return and to monitor overall health. Understanding your treatment options and staying in close contact with your healthcare team can significantly impact in your recovery and long-term well-being. With careful management and the right support, most people affected by UCD can return to their normal activities and maintain a good quality of life.