BURKITT LYMPHOMA

Dr Phillip L.Chalya
M.D.[Dar]; M.MEDsurg [Mak]
Specialist Surgeon- BMC
 INTRODUCTION
 BL is a high grade tumor of B-
lymphocytes, the lymphocytes that the
immune system uses to make antibodies
 It is the fastest growing tumor in man
that can double its in 24 hours
 BL was first named in 1952 by Denis
Parsons Burkitt, who mapped its peculiar
geographic distribution across Africa
 CLASSIFICATION

 Endemic [African] BL
 Non-endemic [Sporadic] BL
 Endemic [ African ] BL
 Endemic in highly malarial areas, mostly
in Africa
 It is associated with the Epsten-Barr
virus [EBV] in > 80% of tumors
 It is the most common type
 Non-endemic [Sporadic]
BL
 This is rare type seen all over the world
 It is not associated with EBV
 EPIDEMIOLOGY
 Incidence:
 BL is endemic in certain regions of
equatorial Africa and other tropical
locations between latitudes 10° south and
10° north
 Incidence in these areas of endemic
disease is 100 per million children
 BL is a very rare tumor in the United
States, with about 100 new cases occurring
each year
 Epidemiology [cont’d]
 Mortality/Morbidity:
 Before aggressive therapeutic programs, children
with BL died rapidly
 With combination chemotherapy and CNS
prophylaxis, the survival rate is now at least 60%
 Patients with local dx have a survival rate of 90%
 Patients with bone marrow and CNS involvement
have a poor prognosis
 Adults with the disease, especially those in the
advanced stage, do more poorly than affected
children.
 Epidemiology [cont’d]
 Race:
 BL is endemic in people living in central Africa
 It is sporadic in residents of the United States.
 Sex:
 The male-to-female ratio is 2-3:1.
 Age:
 BL is most common in children
 In Africa, the mean age is 7 years
 Outside Africa, the mean age is 11 years.
 AETIOLOGY
 The exact cause and mechanisms of
Burkitt lymphoma are not known
 Implicated factors include:-
 Chromosomal abnormalities:

Translocation of proto-oncogene c-myc from its
normal position on chromosome 8 to position 14
[90% of cases] or 2 or 22 [<10% of cases]
 Viral infection: EBV
 Malarial infection
 PATHOPHYSIOLOGY
 BL is characterized by the presence of a
"starry sky" appearance), imparted by
scattered macrophages with phagocytes cell
debris
 The African form most often involves the
maxilla or mandible
 The sporadic form usually involves abdominal
organs, with the most common involvement
of the distal ileum, cecum, or mesentery and
less common involvement of other abdominal
organs, pelvic organs, and facial bones
 Pathophysiology [cont’d]
 Most BLs carry a translocation of the c-
myc oncogene from chromosome 8 to
either the immunoglobulin (Ig) heavy-
chain region on chromosome 14 [t(8;14)]
or one of the light-chain loci on
chromosome 2 (kappa light chain)
[t(8;2)] or chromosome 22 (lambda light
chain) [t(8;22)]
 Pathophysiology [cont’d]
 The EBV has been implicated strongly in
the African form, while the relationship is
less clear in the sporadic form
 EBV is associated with about 20% of
sporadic cases
 Rare adult cases are associated with
immunodeficiency, particularly AIDS
 The lymphocytes have receptors for EBV
and are its specific target
 Pathophysiology [cont’d]
 In the African form, the hosts are
believed to be unable to mount an
appropriate immune response to primary
EBV infection, possibly because of
coexistent malaria or another infection
that is immunosuppressive
 Months to years later, excessive B cell
proliferation occurs.
 CLINICAL PRESENTATION
 History
 In the African form of Burkitt lymphoma,
patients most often present with swelling of
the affected jaw or other facial bones,
loosening of the teeth, and swelling of the
lymph nodes, which are nontender and
rapidly growing, in the neck or below the jaw
 Abdominal presentation is slightly less
common
 Clinical presentation
[cont’d]
 Patients with the sporadic form of Burkitt
lymphoma most commonly present with
abdominal tumors causing swelling and pain in
the affected area
 Some patients present with symptoms of
bowel obstruction secondary to an ileal-cecal
intussusception caused by tumor growth
 Because of the rapid growth of the Burkitt
tumor, patients may quickly manifest
significant metabolic derangement and renal
 Clinical presentation
[cont’d]
 Less common presentations of Burkitt
lymphoma include an epidural mass,
skin nodules, CNS symptoms, and bone
marrow involvement
 Rare cases of Burkitt lymphoma can
present as acute leukemia (L3-ALL) with
fever, anemia, bleeding, and adenopathy
 Clinical presentation [cont’d]
 Physical Examination [Signs]:
 Major signs of Burkitt lymphoma include a
soft tissue mass associated with the
involvement of the jaw or other facial bones,
enlarged cervical lymph nodes, abdominal
masses, and ascites
 Differential Diagnosis
 BL must be distinguished from other
primary abdominal tumors in childhood,
including Wilms tumor, neuroblastoma,
and peripheral neuroectodermal tumor
 In the bone marrow, it must be
differentiated from B and T precursor and
myeloid leukemias
 In peripheral B-cell lymphomas, the major
differential diagnosis is with diffuse large
 WORK UP
 Lab studies
 Imaging studies
 Diagnostic procedures
 Chromosomal analysis
 Lab studies
 CBC
 LFT
 RFT
 Serum electrolytes e.g. sodium, potassium,
calcium, phosphorus, magnesium etc
 Serum uric acid
 Cerebrospinal fluid (CSF) analysis
 Cytogenetic studies of peritoneal or pleural
fluid, when appropriate, should be performed
for diagnostic or staging purposes.
 Imaging studies
 Chest x-ray is necessary to rule out lung
metastasis and mediastinal involvement
 Chest CT should be performed if chest x-ray is
abnormal.
 CT scan or MRI of the primary site is important
for evaluation of the extent of the primary
disease.
 Abdominal CT scan is required to evaluate the
extent of disease such as involvement of
retroperitoneal and mesenteric lymph nodes,
liver, kidneys, ovaries, and other structures.
 Head or spinal CT scan or MRI is indicated if
neurological signs and symptoms are present.
 Imaging studies [cont’d]
 Bone scan and plain bone radiographs are needed
for patients with symptoms of bone involvement
 Fluorine 18-2-fluorodeoxyglucose (FDG)-PET is an
imaging modality using physiological tracer
glucose, whose uptake and metabolism is
increased in tumor cells
 FDG-PET scanning has been reported to be an
efficient, non-invasive method for staging and
monitoring of both Hodgkin and non-Hodgkin
lymphomas, including Burkitt lymphoma, and is
even more accurate than CT scan in detecting
lesions
 Diagnostic procedures
 Bone marrow aspiration should be
performed for every patient with BL
because the frequent presence of
unexpected bone marrow involvement
has important implications for treatment
planning.
 Lumbar puncture is necessary for
evaluation of CNS involvement.
 Paracentesis or thoracentesis is needed
for cytogenetic studies if ascites or
pleural effusion is present
 Diagnostic procedures
[cont]
 Biopsy of suspected lymph nodes or
other disease sites is essential to
confirm the diagnosisA starry sky
appearance [due to scattered
macrophages with phagocyte cell debris]
 STAGING
 Various staging systems have been
proposed
 The National Cancer Institute (NCI) system
 A - Single solitary extra-abdominal site
 AR - Intra-abdominal, more than 90% of tumor
resected
 B - Multiple extra-abdominal tumors
 C - Intra-abdominal tumor
 D -Intra-abdominal plus one or more extra-
abdominal sites
TREATMENT
 PROGNOSIS
 The prognosis in children correlates with
the bulk of disease at the time of
diagnosis
 With appropriate management of the
metabolic consequences of rapid cell
turnover and with combination
chemotherapy and CNS prophylaxis, the
survival rate has been improved
significantly.
 Patients with limited (A, AR) disease have
 Prognosis [cont’d]
 Patients with more extensive disease,
especially bone marrow and CNS
involvement, have a worse prognosis, but
long-term survival rates as high as 80%
can be achieved with more aggressive
chemotherapy regimens
 Adults with BL, particularly those with
advanced stage disease, do more poorly
than children with the disease