A 5 Year Old African Child With Swelling Of The Jaw

A 5 year old African child is referred to a Pediatrician for the presenting symptoms of a progressive painless swelling of the jaw that has not responded to antibiotic treatment and is growing rapidly leading to disfiguring of the face. The child has low grade fever for over 4 months and has also lost weight. He has lost his appetite and interest in daily activities.

The picture is shown below:

Blood workup shows Anemia and low platelet count. Serum LDH was markedly elevated and serum uric acid levels were also high.
A biopsy was taken from the tumor and the case was diagnosed as Burkitt Lymphoma.

Case Discussion:

BURKITT LYMPHOMA
Burkitt lymphoma is a form of non Hodgkin's lymphoma in which the cancer cells start in the immune cells known as B cells.
It is associated with diminished immunity and if left untreated will lead to rapid death.
It is commonly seen in young children in Africa where it is believed to be caused by impaired immune response to Ebstein Barr virus that allows changes to occur in infected B cells to become cancerous cells. Outside Africa it is rare and mostly seen in patients infected with HIV.

Clinical Features:
The lymphoma cells divide very rapidly and so the symptoms appear within weeks. Patients may present with:

• swelling of one or more group of lymph nodes.
• Children in the endemic areas most commonly present with painless swelling of the jaw.
• weight loss
• Loss of appetite
• abdominal pain
• mass in the abdomen
• unexplained fever

The symptoms depend on the type of the Burkitt lymphoma.

Types Of Burkitt Lymphoma:

1. Endemic (African): most commonly affects children less than 12 years of age and is twice as more common in boys than in girls.

2. Sporadic (non- African): can occur worldwide but is very rare.

3. Immunodefeciency associated: Most commonly seen in patients with AIDS. 

Diagnosis: The definite diagnosis is made by biopsy of the tumor mass for histo pathology. Histologically Burkitt lymphoma is characterized by monoclonal proliferation of medium sized non cleaved B cells that are uniform in appearance. The cells have a scant basophillic cytoplasm along with numerous lipid vacuoles , round nuclei with stippled chromatin and multiple small nucleoli. Under the microscope the characteristic description is starry sky appearance. however it is not pathognomic for Burkitt lymphoma and may also be seen in other types of lymphomas. . The histologic picture is shown below:

Starry sky appearance ( the stars are the macrophages phagocyting the apoptotic tumor cells and the sky is the neoplastic lymphocytes.

Management: Burkitt lymphoma is a rapidly growing tumor and immediate diagnosis and treatment is needed. Chemotherapy is the mainstay of the treatment. There is no role of surgery or radiation therapy.