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Referral and warning signs
When to refer
IMMEDIATE URGENT REFERRAL to Specialist Cancer Centre (UCI/RR) for ANY suspected case of Burkitt's Lymphoma.
Red flags
- Rapidly growing jaw or facial swelling in a child.
- Loosening of multiple teeth in affected area.
- Associated neurological signs (lip numbness, proptosis).
- Signs of airway compromise.
Signs and symptoms
- Rapidly growing swelling of the jaw (maxilla or mandible).
- Loosening and displacement of teeth in the affected area.
- Pain, may initially present as toothache.
- Facial asymmetry.
- Proptosis (if orbit involved).
- Nasal obstruction (if maxilla/sinuses involved).
- Numbness of lip/chin (nerve involvement).
- Lymphadenopathy (less common initially with jaw presentation).
- Systemic symptoms (fever, weight loss, night sweats) may be present.
- Often affects children (peak age 4-7 years in endemic form).
Risk factors
- Endemic Burkitt's (Africa):
- - Epstein-Barr Virus (EBV) infection.
- - Chronic malaria co-infection (immunosuppression).
- - Young age.
- Sporadic Burkitt's (Non-African): Usually abdominal presentation.
- Immunodeficiency-associated: HIV infection / AIDS.
Diagnosis
Clinical assessment
High index of suspicion for rapidly expanding jaw swelling, especially in a child in an endemic area, with associated tooth mobility. Often mistaken initially for dental abscess.
Differential diagnosis
- Dental abscess / Osteomyelitis.
- Ameloblastoma.
- Other childhood sarcomas (Rhabdomyosarcoma).
- Fibrous dysplasia.
- Giant cell granuloma.
- Metastatic tumour.
Investigations
- Biopsy: Incisional or excisional biopsy of the mass for histopathology (shows characteristic 'starry sky' appearance) and immunohistochemistry. ESSENTIAL for diagnosis.
- Imaging:
- - OPG / Facial X-rays: Show bone destruction, tooth displacement ('floating teeth').
- - CT / MRI (Head/Neck/Chest/Abdomen/Pelvis): Staging, assess extent of disease, CNS involvement.
- Blood Tests: FBC, LDH (often very high), Uric acid, Renal/Liver function (assess tumour lysis risk/organ function).
- Bone Marrow Aspiration and Biopsy: Check for involvement.
- Lumbar Puncture with CSF analysis: Check for CNS involvement.
- HIV testing.
Diagnostic criteria
Histopathological confirmation from biopsy.
Management
Non-pharmacological management
- Supportive care (hydration, nutrition).
- Management of complications (tumour lysis syndrome, infection).
Pharmacological treatment
- Requires IMMEDIATE Referral and Management at Specialist Cancer Centre (UCI/RR):
- Intensive Combination Chemotherapy: Backbone of treatment. Regimens vary (e.g., CODOX-M/IVAC). Very sensitive to chemotherapy.
- CNS Prophylaxis/Treatment: Intrathecal chemotherapy (e.g., Methotrexate).
- Tumour Lysis Syndrome Prophylaxis/Management: Allopurinol, IV hydration, Rasburicase (if available).
- Immunotherapy (Rituximab): Often added to chemotherapy.
- Radiotherapy: Limited role, mainly palliative or for residual disease.
- Surgery: Primarily for biopsy, limited role in primary treatment.
Monitoring
Close monitoring during chemotherapy for toxicity (myelosuppression, infection, tumour lysis). Monitor response to treatment (imaging, labs). Long-term follow-up for recurrence.
Complications
- Rapid tumour growth causing local destruction, airway compromise.
- Metastasis (CNS, bone marrow).
- Tumour Lysis Syndrome (metabolic emergency during initial treatment).
- Complications of chemotherapy (infection, infertility, secondary malignancies).
- Pathological fractures.
- Death if untreated or treatment fails.
Prevention
- Malaria control (may reduce risk of endemic form).
- Prevention/management of HIV.
Patient education
- Explanation of the diagnosis as a fast-growing cancer.
- Need for urgent specialized treatment (chemotherapy).
- Treatment plan overview, potential side effects.
- Importance of supportive care.
- Prognosis (often good with prompt, intensive treatment).
- Need for long-term follow-up.
Uganda-specific notes
Endemic Burkitt's lymphoma is the most common childhood cancer in Uganda. Jaw presentation is classic. Requires urgent referral to the Uganda Cancer Institute (UCI) or designated regional centres for diagnosis and intensive chemotherapy.
References
- Uganda Clinical Guidelines (UCG) 2023
Chapter 23, Section 23.5.1
- Uganda National Cancer Control Plan / Treatment Guidelines
(Specific oncology protocols for Burkitt's Lymphoma)