BURKITTS LYMPHOMA

Aetiology
The aetiology of Burkitts lymphoma is
unknown.
It is associated with Epstein Barr Virus
It is prevalent in endemic malaria areas.

Pathology
Burkitts lymphoma arises as tumour of:
1.Maxilla or mandible producing gross
swelling and deformity of the face.
This is the commonest presentation.
2.Other sites which include:
a.Abdomen
Kidneys
Ovaries

 Lymph nodes (para-aortic

lymph nodes)
Peripheral lymph nodes rarely
affected (unlike leukemia and
other malignant lymphomas)
b. CNS
c. Bone marrow/ in advanced stages
Burkitts lymphoma is a B
lymphocyte malignancy

Symptoms and signs

Burkitt,s lymphoma presents as:
1.Facial tumour. This is the commonest
presentation causing deformity of the face,
loosening of the teeth, proptosis of the
eyes, encroachment within the mouth or
pharynx
2.Abdominal tumour
3.CNS symptoms in advanced cases

Burkitts lymphoma of left maxilla and proptosis

Diagnosis
1.Clinical from symptoms and signs
2.Needle aspiration of the tumour for
cytology shows characteristic
appearance of abnormal vacuolised
lymphoid cells, often described as
starry sky picture;
3.Other investigations: X-ray, abdominal
ultrasound and complete blood count
4.Screening for associated infections

5. Staging of Burtitts lympoma

The diagnosis of the tumour is not
complete without staging of the
disease.
Staging is the process of determining
whether cancer has spread and, if so,
how far.
The staging and evaluation of the extent
of the tumour is very valuable but the
size is important even in single
abdominal site involvement.

Special Staging System for Burkitts

Lymphoma
(WHO Classification of Tumours 2001)
Stage I
A single extra nodal tumor or single
anatomic nodal area, except
mediastinum or abdomen

Stage II
1.A single extranodal tumor with
regional node involvement
2.Two or more nodal areas on the
same side of the diaphragm
3.Two single extranodal tumors with or
without regional node involvement
on the same side of the diaphragm

4. Primary GI tumor, with or without

involvement of associated mesenteric
nodes only

Stage IIR
Completely resected abdominal disease

Stage III
1.Two single extranodal tumors above
and below the diaphragm
2.Two or more nodal areas above and
below the diaphragm.
3.All primary intrathoracic tumors
4.All extensive primary intra-abdominal
disease.

Stage IIIA
Localized but non-resectable
abdominal disease.
Stage IIIB
Widespread multiorgan abdominal
disease
Stage IV
Any of above with initial CNS and/or
bone marrow involvement (25%)

Prognosis
1. Early detection and treatment
Outcome
80% complete response
10% partial response
10% no response
50% those who show complete
response relapse

Treatment
1. Surgery
In case of an abdominal tumour with
an extra abdominal involvement. This
is followed by chemotherapy.
2. Chemotherapy
Success or failure depends upon
how early it is started.

Drugs
1.Cyclophosphomide 40mg/dose (11.2g/m2)
2.Vincristine 0.05-0.07mg/kg (1-1.2mg/m2
body surface) I.V. once a week for 6
weeks
3.Methotraxate 0.7-2mg/kg (20-60mg/m2
body of surface area) po or iv once a
week for 6 weeks.

For CNS involvement

Methotraxate intrathecally once a week
Dose according to age is as follows:
< 2year

- 8mg

2-3 years - 10mg

>3 years - 12mg

Other more aggressive option

CODOX-M/IVAC regimen (Magrath
protocol) which consists of:
Two cycles of CODOX-M
(cyclophosphamide, vincristine,
doxorubicin, high-dose metho-trexate, and
intrathecal therapy) alternating with
IVAC (ifosfamide, etoposide, high-dose
cytarabine, and intrathecal therapy)

Allopurinol to prevent tumour lysis

syndrome
Dose 10mg/kg n 3 dose 1-2 days.

END