burkitts lymphoma

1. BURKITT’S LYMPHOMA
ABDOMINAL MASS
Pediatrics

2. Introduction
• Lymphoma’s are cancer originating from the lymphatic
system
• There 2 types of Lymphoma based on how they appear
microscopically
• Hodgkins lymphoma
• Non Hodgkins lymphoma
• Burkitt’s Lymphoma is a Non Hodgkin lymphoma of B-
lymphocytes.
• It is a common childhood malignancy in Africa.

3. Types of Burkitt’s Lymphoma
• AFRICAN (ENDEMIC) BURKITT’S LYMPHOMA
• SPORADIC (NON ENDEMIC) BURKITT’S LYMPHOMA
• HIV ASSOCIATED BURKITT’S LYMPHOMA

4. Equatorial and Malaria endemic belt

5. Endemic type of BL
• Endemic Burkitt's lymphoma is
seen among the young
children of equatorial Africa.
• It frequently affects the jaws
of children with developing
molar teeth;
• experts suggest that growth
factors may be responsible for
the site-specific nature of this
tumor.

6. Sporadic BL
• Sporadic Burkitt's lymphoma
often involves the abdomen,
and it spreads to the bone
marrow in about 20% of
patients
• When Burkitt's lymphoma
involves the abdomen, it can
cause symptoms such as
abdominal pain, swelling,
nausea, vomiting, and
changes in bowel habits.
• It is a highly malignant
lymphoma, but it is potentially
curable with very aggressive
therapy.

7. Immunodefecient BL
• Immunodeficient Burkitt's lymphoma affects patients with
abnormal or suppressed immune systems (e.g., patients
with HIV).

8. Pathogenesis
• All forms of Burkitt’s lymphoma are associated with a
translocation of the c-myc gene on chromosome 8

9. Pathogenesis
• EBV is found in all African Burkitt’s Lymphomas and in
25% of the HIV associated Burkitt’s lymphomas but is
rare in Non-endemic or sporadic type of Burkitt’s
• EBV in it self how ever does not appear to be the only
factor in causing the development of Burkitt’s lymphoma
thus not every one who gets infected with EBV develops
Burkitt’s lymphoma

10. Pathogenesis

11. Other associated causes of BL
• Chronic malaria infection has been identified as one of the
core factor’s for the development of Burkitt’s lymphoma
since it favors sustained proliferation of B cells
immortalized by EBV
• A weakened immune system fails to control EBV infection
and thus favors proliferation of the infected cells and thus
the high incidence of EBV associated Burkitt’s
lymphomas in immunocompromised patients

13. Common sites involved
• Bones of the jaw and other facial bones,
• Kidneys,
• Gastrointestinaltract, The abdomen, especially the
ileocecal area, is the mostcommon site of involvement;
• Ovaries,
• Breast,
• Other extranodal sites

14. • Bilateral involvement of the breasts may occur in
association with the onset of puberty or with lactation
• Lymph node involvement is more common
among adults than among children
• Patients may also have malignant pleural effusions or
ascites

15. Staging
Group A – Low Risk:
• A single facial mass (excluding retro-orbital masses) less than 10cm
Group B – High Risk:
• Any disease more than Group A but without CNS disease
Group C – CNS disease present:
• Any child with clinical or laboratory evidence of CNS disease
associated with any other stage of BL disease; all retro-orbital masses
are considered as high risk for CNS disease and therefore included in
this group.
Group D – Bone Marrow Involvement:
• Any child with laboratory evidence of Bone Marrow involvement
associated with any other stage of BL disease.

16. Investigations
• Clinical
• Radiological
• Cytology
Other investigations
• FBP
• Serology for HIV
• USS of swelling
• CSF examination
• RFT and Electrolytes
• Bone marrow aspirate and biopsy

17. Management
• Chemotherapy
• Radiation
• Surgery

18. Chemotherapy
• chemotherapy is the treatment of choice
• Alternative name is cytotoxic drugs
• Chemotherapy uses anticancer drugs that are given IV, IT
or orally
• These drugs enter the bloodstream and reach all areas of
the child's body, making this treatment useful for cancer
that has spread widely

19. Chemotherapy
Cyclophosphamide
1-1.2gm/m² or 40mg/kg/dose
given iv in 100mls of 5%glucose
vincristine
1.5-2mg/m² or 0.05-0.07mg/kg iv by slow push
Methotrexate
20-60mg/m or 0.7-2mg/kg IT (can also be given
oraly,IV)

20. National TX Protocol
Group Number and name of
intravenous (IV)
cycles of
chemotherapy
Number of cycles of
intrathecal (IT)
chemotherapy
A: Low Risk 3 COM 3
B: High Risk 6 COM 3
C: CNS positive 6 COM 6
D: BM Positive 6 alternating COM/
EMIC
6

21. Sugery
• Surgery should not be done simply to remove a tumor, as
normal organs might be damaged in the process.
• Other reasons for surgery includes:
To obtain tissue for diagnostic tests when other procedures
could not obtain enough tissue
On an emergence basis, to relieve a blockage (obstruction)
in the intestine caused by the tumor mass

22. Radiation
• Was once the main treatment for children with NHL,
however it is no longer used in the initial Rx of NHL,
except perhaps in an emergency to treat pressure on the
trachea or SC.
• For palliation during late presentation

23. Side effects
• Nausea, vomiting, diarrhea, loss of appetite
• Alopecia
• Tumor Lysis syndrome-after a rapid destruction of the
tumor » Hyperuricemia
with renal failure (Prevented by allopurinol and plenty
of fluids). Breakdown products may affect kidneys, heart,
CNS.

24. • Damage to other normal cells that are dividing rapidly-
bone marow,linings of mouth (mouth sores),
GIT,reproductive cells
• Hemorrhagic cystitis
• Cardiotoxic
• Vincristine is neurotoxic

25. Prognosis
• International Prognostic Index
The International Prognostic Index (IPI) was designed to
further clarify lymphoma staging.
• The IPI predicts the risk of disease recurrence and
overall survival by taking into account factors such as
• age,
• stage of disease,
• general health (also known as performance status),
• number of extranodal (other than the lymph nodes)
• sites,
• presence or absence of an elevated serum enzyme
named lactate dehydrogenase (LDH).

26. Differential diagnosis of Abdominal
Masses
• Wilm’s tumour- Nephroblastoma
• Embryonal tumours
• Arising from the kidneys
• They do not cross the midline
• Good prognosis
• Neuroblastoma’s
• Tumour arising from the neuro crest
• Located/ arising from the adrenal glands
• Crosses the midline
• Poor prognosis
• Elevated Urine VMA
• Hepatoblastoma
• Arising from the liver
• Embryonal tumours
• Elevated alpha pheto-protein

27. DDX
• Splenomegaly
• Sequestration crisis in SCD
• Severe/complicated Malaria
• Leukemia’s
NOTE:
Cautious on your palpation.

28. Done!!!