Burkitt Lymphoma

Disclaimer

The information provided in this review is intended for general informational purposes only. It is not intended to be a substitute for professional medical advice, diagnosis, or treatment.

Always seek the advice of your physician or other qualified healthcare provider with any questions you may have regarding a medical condition. Feel free to share this information page with them.

Cancer Localization

Common sites of Burkitt lymphoma include:

• Most typically the abdomen (intestines, kidneys, ovaries)
• Jaw and facial bones
• Central nervous system
• Lymph nodes

Biology

Lymphomas are cancers that develop from white blood cells, which are important for fighting infections. In other words, lymphomas are cancers of the immune system. Burkitt lymphoma (BL) is a lymphoma – a cancer in a type of white blood cells called B cells.

BLs grow extremely fast. Almost all BL cancers involve a DNA mutation called a translocation where a growth gene called MYC is activated, leading to uncontrollable rapid tumor growth.

Characteristics

BL is a rare cancer, but it is one of the more common non-Hodgkin lymphomas in children. Most cases occur in children and teens, with boys often more affected. Currently, around 80-90% of all children with BL can be cured.

However, Although there are high cure rates for pediatric BL, it grows very quickly and treatment needs to start promptly for success.

Main Types of BL

There are three recognized clinical forms of BL:

1. Endemic – Seen mostly in equatorial Africa and some Pacific regions, the endemic form of BL is strongly linked to Epstein-Barr virus (EBV) infection and most often involves the jaw and facial bones.
2. Sporadic – This version is typically encountered in North America, Europe, and many other parts of the world. It typically presents in the abdomen and is less commonly associated with EBV.
3. Immunodeficiency-associated – Occurs in children with weakened immune systems (ex. those living with HIV or receiving immune suppressors after an organ transplant). Parent Tip: Alveolar RMS (ARMS) is a very aggressive and very violent type of cancer with the tendency to relapse. That’s why is crucial to defeating cancer on the first try. Do the best you can and be proactive early!

Although the biology behind these subtypes overlaps, their typical body sites and epidemiologic patterns differ.

Common Mutations in BL

The signature genetic event in Burkitt lymphoma is a MYC translocation. This genetic rearrangement places the MYC growth gene next to powerful gene enhancers, which drives relentless tumor growth. EBV infection acts as a co‑factor in many endemic cases.

Note: Unlike many other childhood cancers, BL does not usually carry a wide variety of different mutations; it is the speed and uniformity of the MYC translocation event that gives it its clinical personality.

Pediatric BL Noticeable Symptoms

Symptoms reflect where the tumor is and how fast it grows. A child might suddenly complain of belly pain or look distended because a mass has filled the abdomen. When BL starts in the jaw, parents may see swelling in the face or notice a tooth loosening. Enlarged lymph nodes can appear in the neck, armpit, or groin. General “B symptoms” such as fevers, night sweats, and weight loss can be present. Doctors should also watch closely for tumor lysis syndrome — the rapid breakdown of tumor cells that can upset blood chemistry.

Common signs to watch for as a parent:

• A swollen or painful abdomen, vomiting, constipation, or diarrhea
• Facial/jaw swelling or loose teeth (especially in endemic regions)
• Painless but fast‑growing lumps in the neck, armpit, or groin
• Fevers, drenching night sweats, and unexplained weight loss
• Sudden changes related to tumor lysis syndrome (handled preventively by the care team)

Childhood Cancer Diagnostic Methods

Diagnosis usually begins with a careful physical examination and history to locate any lumps, swelling, or systemic symptoms. Imaging tests — typically ultrasound in the abdomen or CT and MRI scans — are ordered to define the size of masses and to see whether disease has spread to nearby organs or lymph nodes. A biopsy is essential: a surgeon or interventional radiologist removes a small piece (or occasionally an entire node/mass) so a pathologist can look at the cells under a microscope, perform immunohistochemistry, and run flow cytometry to confirm that the tumor is a B‑cell lymphoma. Genetic testing is then used to demonstrate the MYC translocation. Because BL often spreads to the bone marrow and central nervous system, doctors may perform a bone marrow biopsy and a lumbar puncture to examine spinal fluid. Each of these steps helps “stage” the cancer, meaning we learn how extensive it is and which risk group your child falls into.

Note: Sarcomas and lymphomas are rare and can be tricky to diagnose correctly. Seeking a second opinion on pathology slides or the treatment plan is reasonable and, in good centers, welcomed.

Childhood Cancer Treatment

The same three pillars —surgery, radiation, and chemotherapy— appear in many pediatric cancers, but Burkitt lymphoma relies overwhelmingly on chemotherapy, with surgery and radiation playing limited, specific roles. Treatment is delivered according to protocols of deliberately short and intense chemotherapy: the idea is to hit the cancer hard, allow the body to recover, and repeat, rather than give low‑dose therapy for months on end.

Surgery

For BL, surgery is primarily diagnostic. A biopsy is needed to make the diagnosis, and occasionally surgeons intervene to relieve a bowel obstruction or repair a perforation caused by the tumor. Because BL is a systemic disease — even if only one mass is visible — trying to “cut it all out” does not improve cure rates and can delay life‑saving chemotherapy.

Radiation Therapy (RT)

Radiation is rarely part of first‑line BL care. Most children never receive external beam radiation for BL, which helps limit long‑term side effects.

However, RT may be considered when for any of the following cases:

• Spinal cord compression
• A life‑threatening airway issue
• Persistent disease in a site not fully controlled by chemotherapy

Chemotherapy

Chemotherapy is the cornerstone of pediatric BL treatment. Modern BL protocols use combinations of drugs such as cyclophosphamide, vincristine, prednisone, doxorubicin, high‑dose methotrexate, cytarabine, and others.

Rituximab, a monoclonal antibody that targets the CD20 protein on BL cells, is frequently added and has been shown to improve outcomes. Because BL cells can hide in the brain and spinal fluid, children receive intrathecal chemotherapy (medicine injected directly into the cerebrospinal fluid) to prevent or treat central nervous system disease. Treatments are risk‑adapted: children with less extensive disease may get fewer cycles or lower doses, while those with more advanced or relapsed disease receive more intensive regimens or salvage therapies such as R‑ICE (rituximab, ifosfamide, carboplatin, etoposide). Some children with recurrent disease may be candidates for stem cell transplant or novel targeted agents on clinical trials.

Parent Tip: Ask which protocol your team is using, whether rituximab is included, and how they are preventing tumor lysis syndrome and other early complications. Understanding the roadmap helps you anticipate hospital stays, transfusions, and supportive care needs.

Late Effects of the Treatment

Curing BL is the first goal, but attention must also turn to life after therapy. The same drugs that save lives can leave footprints.

Important late effects to look out for:

1. Anthracyclines like doxorubicin can affect heart muscle, so periodic EKG/ECGs may be needed. High‑dose methotrexate or cytarabine can influence learning or attention, particularly in very young children.
2. Cyclophosphamide can affect fertility in high cumulative doses.
3. Nerves, kidneys, and hearing can also be impacted depending on the specific regimen.

Most children will do very well, but careful, long‑term follow‑up is essential to catch and manage problems early.

Follow-up, Late Effects

Follow‑up schedules vary, but a common pattern is:

Year one: visits every two to three months during the first year

Years 2 and 3: every four to six months

Years 4 and 5: every six to twelve months

Year 6+: annually

At these visits, clinicians check growth, development, and organ function with blood tests; revisit any school or emotional concerns; and order imaging only when clinically indicated to limit radiation exposure.

Tests your child may have during follow‑up include:

• Blood tests to monitor organ function and blood counts
• Measurements of growth and development
• Ultrasound, CT, or MRI of the original tumor site when needed
• Chest X‑ray or CT scan if there was chest involvement
• EKG if heart‑affecting drugs were used

If the cancer returns, your team will discuss the next steps, which may include different chemotherapy combinations, targeted therapies, or clinical trials.

Review of Current Options for Clinical Trials

Review of Current Options for Clinical Trials

Below are several ongoing clinical trials focused specifically on pediatric Burkitt lymphoma. You can visit the links for full eligibility and contact details:

Epcoritamab in Pediatric Relapsed/Refractory Aggressive Mature B‑cell Lymphoma
A Phase I/II trial assessing safety, tolerability, and preliminary efficacy of epcoritamab — an anti‑CD3/CD20 bispecific antibody — in children with relapsed or refractory aggressive B‑cell lymphomas, including Burkitt lymphoma. Status: Recruiting.

https://clinicaltrials.gov/study/NCT05206357