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Hodgkin's Lymphoma
Hodgkin's lymphoma is a cancer of the lymphatic system that primarily affects adolescents and young adults. The vast majority of young patients are cured. The challenge is therefore twofold: to treat the disease effectively and to minimize long-term effects as much as possible.
Hodgkin lymphoma develops from B lymphocytes, which are cells of the immune system. It is characterized by the presence of specific tumor cells, called Hodgkin and Reed-Sternberg cells, within an environment composed of numerous inflammatory cells.
The disease most often affects the lymph nodes, particularly in the neck and chest. It can also affect the spleen and, more rarely, other organs. In children, it is rare before the age of 5 and becomes more common during adolescence.
Key Takeaways
More than 90% of children and adolescents with Hodgkin lymphoma are cured. Treatment is tailored to the extent of the disease and the early response as assessed by PET, in order to avoid unnecessary treatment intensity.
Why does Hodgkin's lymphoma develop?
In most cases, no specific cause is identified. A previous infection with the Epstein-Barr virus may be associated with certain types of Hodgkin lymphoma, but the vast majority of people who have been exposed to this virus will never develop lymphoma.
Factors related to the functioning of the immune system and genetic susceptibility may play a role. Hereditary forms of the disease remain rare. The disease is not caused by lifestyle, diet, upbringing, or psychological factors.
Symptoms
The most common symptom is a persistent enlargement of one or more lymph nodes, often in the neck. These lymph nodes are generally painless and develop gradually.
A lump in the neck, above the collarbone, under the armpit, or, more rarely, in another area. A lump that persists or grows should be evaluated.
Prolonged unexplained fever, profuse night sweats, and significant unintentional weight loss are factors in the classification of the disease.
Enlarged lymph nodes in the mediastinum can cause coughing, shortness of breath, chest pain, or difficulty breathing.
Fatigue, itching, loss of appetite, or discomfort related to enlarged lymph nodes may be present, though these symptoms are not specific.
When to Seek Immediate Medical Attention
Difficulty breathing, swelling of the face or neck, chest pain, feeling unwell, or a rapid worsening of symptoms require urgent medical evaluation. A single swollen lymph node is most often related to an infection, but if it persists or grows larger, you should seek medical advice.
The Diagnosis
Lymph Node Biopsy
The diagnosis is based on the analysis of a lymph node or tissue sample. Whenever possible, a biopsy that allows for examination of the lymph node’s architecture is preferred. The pathologist looks for Hodgkin and Reed-Sternberg cells and determines the lymphoma subtype.
A simple blood test cannot confirm or rule out the diagnosis. However, it assesses the patient’s overall condition, checks for an inflammatory syndrome, and provides useful information prior to treatment.
Staging Evaluation
Fluorodeoxyglucose (FDG) PET-CT plays a central role in identifying active disease sites, determining the stage of the disease, and providing a baseline examination prior to treatment. A diagnostic CT scan or other imaging tests may be used to supplement this, depending on the situation.
The disease is classified based on the affected lymph node regions, their position relative to the diaphragm, any organ involvement, tumor volume, and the presence or absence of B symptoms. A bone marrow aspiration or biopsy is no longer routinely performed when PET provides the necessary information.
Prepare the treatment
Before certain chemotherapy treatments, the medical team may perform a cardiac evaluation and other tests tailored to the planned medications. For patients who have reached puberty, the issue of fertility preservation is discussed before treatment begins, when circumstances permit.
Treatments
European pediatric treatment guidelines are organized by treatment groups. They tailor the number and type of chemotherapy cycles to the initial extent of the disease, risk factors, and early response. The goal is to achieve a cure while reducing the risk of cardiac, endocrine, pulmonary, and gonadal complications, as well as the risk of secondary cancers.
Chemotherapy
Chemotherapy is the primary treatment. The drug combinations used in children and adolescents differ from those used in adults and are selected to maintain efficacy while minimizing certain toxicities, particularly gonadal toxicity. The number of treatment cycles depends on the treatment group and the response.
Early Response Assessed by PET
After the first few cycles, a PET scan assesses the metabolic response. This step is critical: a sufficiently good response may make it possible to avoid radiation therapy in certain situations. An insufficient response leads to a discussion about adjusting the treatment and the indication for targeted radiation therapy.
Radiation Therapy
Radiation therapy is not routinely administered. When indicated, it targets the initially affected sites that require additional treatment, using appropriate treatment volumes and doses. Modern techniques aim to provide the best possible protection for the heart, lungs, thyroid, breasts, and other healthy tissues.
Healing While Minimizing Long-Term Effects
The EuroNet protocols are based on a risk- and response-adaptive strategy. Their primary goal is to limit radiation therapy to patients who need it, without compromising the chances of cure.
In cases of refractory disease or relapse
When a lymphoma does not respond adequately to initial treatment or recurs, a new biopsy may be considered, and the treatment strategy depends on the time to relapse, the treatments already received, the affected sites, and the response to salvage therapy.
Treatment may include salvage chemotherapy, brentuximab vedotin—which targets CD30—or anti-PD-1 immunotherapy such as nivolumab. In certain situations, high-dose chemotherapy followed by an autologous stem cell transplant remains the recommended approach. Other patients, particularly those with certain low-risk relapses, may benefit from tailored strategies evaluated in specialized clinical trials.
Treatments that are now much more than just experimental
Brentuximab vedotin and PD-1 inhibitors are among the treatment options used or being evaluated in children, adolescents, and young adults with relapsed or refractory disease, depending on the specific situation and clinical trial protocols.
Follow-up After Treatment
After treatment ends, follow-up care ensures that remission is maintained, monitors for any late effects, and supports the patient’s return to daily life. It is primarily based on follow-up visits, clinical examinations, and symptoms. Imaging tests are ordered according to the protocol and the patient’s situation, in order to avoid unnecessary radiation exposure.
Follow-up depends on the anthracyclines administered and whether thoracic radiation therapy was given.
Radiation therapy to the neck or mediastinum may warrant long-term thyroid monitoring.
The risk varies depending on the medications, the doses, and radiation therapy. Information and, when possible, preservation options are offered before treatment.
The risk depends, in particular, on the treatments received. Appropriate monitoring and preventive advice are part of long-term follow-up.
Certain therapeutic exposures may require specific evaluations.
Psychological support, adapted physical activities, educational services, and social support are provided as needed.
Long-term, personalized follow-up
The frequency of follow-up visits and tests varies from patient to patient. It depends on the initial stage of the disease, the patient’s response to treatment, medications, radiation therapy doses, age, and the patient’s specific needs.
Patient Care at Gustave Roussy
The Department of Pediatric and Adolescent Oncology treats patients up to age 25 and provides care tailored to children, adolescents, and young adults. Each case is discussed during a multidisciplinary team meeting, drawing on the expertise of pediatric oncologists, hematologists, pathologists, radiologists, nuclear medicine specialists, radiation oncologists, and palliative care professionals.
The care plan may include psychological support, pain and nutrition management, adapted physical activity, support for continuing school or college, social support, fertility preservation, and long-term follow-up.
Research and Clinical Trials
The research aims to maintain very high cure rates while reducing toxicity. In particular, it evaluates the use of PET-guided treatment, the reduction of radiation therapy, the integration of targeted drugs and immunotherapies, as well as less toxic catch-up strategies.
Key Points
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A highly treatable disease
The vast majority of children and adolescents recover, with an overall survival rate of over 90%.
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The biopsy confirms the diagnosis
The histopathological analysis identifies Hodgkin and Reed-Sternberg cells.
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PET guides the strategy
It contributes to the initial assessment and early evaluation of the response.
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Non-routine radiation therapy
It is reserved for situations where it is expected to be beneficial.
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New Options for Relapse
Brentuximab vedotin and anti-PD-1 immunotherapies now play a significant role.
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Long-term follow-up
It focuses in particular on the heart, the thyroid, fertility, secondary cancers, and quality of life.