A Rare Case of Primary Pancreatic Burkitt

Srinivas S Nistala, Niraj R Sawalakhe, Narendhran R Thiruvengadam, Pravin M Rathi
Department of Gastroenterology, T.N. Medical College and B.Y.L. Nair Charitable Hospital.
Mumbai, India
ABSTRACT
Context Lymphomas of the gastrointestinal system are usually of a non-Hodgkin’s type. Primary lymphomas of the pancreas are
uncommon and Burkitt lymphoma involving the pancreas is very rare. It is important to recognize this entity because it can mimic
adenocarcinoma but its management is entirely different. Case report We present the case of a young Indian male who presented
with rapidly progressing obstructive jaundice, gastric outlet obstruction and severe weight loss. Conclusion Early diagnosis of this
aggressive tumor and prompt induction of chemotherapy dramatically improved the patient’s condition and avoided unnecessary
surgical intervention.
INTRODUCTION
In the middle of the last century, Denis Burkitt,
working in central Africa, noted children with grossly
distorted faces, and lesions involving the face, and
upper and lower jaws. This malignancy, initially
thought to be a sarcoma and later established to be a
lymphoma, was given the name Burkitt lymphoma [1].
Burkitt lymphoma is a high-grade B-cell neoplasm of a
non-Hodgkin lymphoma type. Primary pancreatic
lymphomas, usually non-Hodgkin lymphomas, made
up fewer than 2% of extranodal malignant lymphomas
and 0.5% of all pancreatic masses [2]. The frequency
of Burkitt lymphoma among primary pancreatic
lymphoma is not known. A PubMed search for primary
pancreatic Burkitt lymphoma and pancreatic Burkitt
lymphoma revealed 17 case reports/series, six of them
in the pediatric age group.
This tumor is very rarely seen primarily involving the
pancreas. Burkitt lymphoma is one of the fastest
growing malignancies in humans. Because of its rapid
proliferation, early diagnosis and treatment offers the
best chance of survival.
CASE REPORT
We report the case of a 21-year-old male who
presented with jaundice of a 25-day duration followed
by increasing swelling in the epigastric region. This
was associated with severe abdominal pain, abdominal
distension, vomiting and fever. He also had significant
weight loss. There was no history of any major illness
in the past. Examination revealed a cachectic, febrile
patient with deep scleral icterus, without peripheral
lymphadenopathy. An abdominal examination revealed
a tender mass 7x7 cm in size in the epigastric region
with an enlarged liver, which was felt separately from
the mass. The spleen was not palpable. Free fluid was
present in the abdomen. The results of the clinical tests
carried out are as follows: hemoglobin: 10.8 g/dL
(reference range: 13.0-16.0 g/dL); MCV: 86 fl
(reference range: 78-100 fl); total leucocyte count: 12.8
x109/L (reference range: 4.5-11.0 x109/L) with 80%
neutrophils (reference range: 40-70%); erythrocyte
sedimentation rate: 32 mm/1st h (reference range: 0-17
mm/1st h); platelet count: 750 x109/L (reference range:
150-350 x109/L); total bilirubin: 646 µmol/L (reference
range: 5.1-17.1 µmol/L); direct bilirubin: 374 µmol/L
(reference range: 1.70-5.13 µmol/L); SGOT: 136 U/L
(reference range: 5-40 U/L); SGPT: 309 U/L (reference
range: 5-40 U/L); serum alkaline phosphatase: 2,220
U/L (reference range: 80-270 U/L); serum amylase:
325 U/L (reference range: 0-120 U/L); LDH: 480 U/L
(reference range: 70-240 U/L); prothrombin time: 14 s
(control: 11 s), random blood sugar: 61 mg/dL
(reference range: 70-100 mg/dL); CA 19-9: 656 U/mL
(reference range: 0-37 U/mL); calcium: 8.5 mg/dL
(reference range: 9-11 mg/dL); uric acid: 3.5 mg/dL
Received August 13th, 2009 - Accepted September 18th, 2009
Key words Burkitt Lymphoma; CVAD protocol; Jaundice,
Obstructive; Pancreas
Correspondence Pravin M Rathi
Department of Gastroenterology, TNM College and BYL Nair Ch.
 

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(reference range: 2.0-6.5 mg/dL); creatinine: 1.2 mg/dL
(reference range: 0.8-1.5 mg/dL). A CT scan of the
abdomen (Figure 1) showed a 12x11x10 cm solid mass
in the head of the pancreas, encasing the duodenum
and the cystic duct. It was compressing the common
bile duct and causing intrahepatic biliary duct dilation.
The mass also involved the portal vein and hepatic
artery. A CT scan of the thorax showed no
lymphadenopathy. Upper GI endoscopy showed tumor
infiltration of the first and second parts of the
duodenum. Biopsies were taken; the histopathological
examination was suggestive of high grade non-
Hodgkin’s lymphoma. Endoscopic ultrasound showed
a solid mass in the region of the pancreatic head with
involvement of the portal vein. EUS-guided FNA was
carried out on the mass; however, no conclusive
opinion was possible because the sample was
insufficient. US-guided percutaneous biopsy of the
mass showed a malignant round cell tumor suggestive
of non-Hodgkin’s lymphoma. On immunohisto-
chemistry (Figures 2 and 3), the tumor cells expressed
leukocyte common antigen CD20+, CD10+, bcl 6+, and
were CD5-. The Mib 1 index was 99%. These findings
were consistent with Burkitt lymphoma. The ascitic
fluid field was full of tumor cells. Bone marrow and
cerebrospinal fluid studies were normal. As a result of
the above investigations, the patient was diagnosed as
having Burkitt lymphoma of the pancreas and was
treated with 2 cycles of CHOP (cyclophosphamide,
adriamycin, vincristine and prednisolone) following
which his general condition improved dramatically.
The vomiting and pain subsided; the ascites
disappeared, and total bilirubin and alkaline
phosphatase levels decreased to 115.6 µmol/L and 680
U/L, respectively, over a period of one week. A CT
scan of the abdomen (Figure 4) was repeated and
showed a marked reduction in the size of the tumor
mass. He was started on a hyper CVAD
(cyclophosphamide,
vincristine,
doxorubucin,
dexamethasone) regimen as definitive therapy.
DISCUSSION
Lymphomas are the only tumors, other than
adenocarcinomas, which occur with some frequency in
the pancreas, although they are rarely found in the
pancreas. Primary pancreatic lymphomas represent less
than 2% of all NHLs, although nearly one-third of non-
Hodgkis lymphoma patients will develop pancreatic
Figure 1. CT scan of the abdomen before chemotherapy showing
arterial involvement by the mass.
Figure 2. Starry sky appearance on H&E staining (4x).
Figure 3. Mib1 staining showing 99% positivity (4x).
Figure 4. CT scan of the abdomen after chemotherapy showing a
marked reduction in tumor size.

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JOP. Journal of the Pancreas - http://www.joplink.net - Vol. 10, No. 6 - November 2009. [ISSN 1590-8577]
688
involvement at some point in their disease course.
Cases have been described in both pediatric and adult
population, presenting with obstructive jaundice [3, 4],
pancreatitis [5] or symptoms of gastric outlet
obstruction. In older individuals with elevated CA 19-9
levels, Burkitt lymphoma can be mistaken for
adenocarcinoma of the pancreas and they therefore
undergo major surgery [3].
In the World Health Organization (WHO)
classification, three clinical variants of Burkitt
lymphoma are described: endemic, sporadic, and
immunodeficiency-associated [6].
Endemic Burkitt lymphoma (eBL) refers to those cases
occurring in African children (usually 4-7 years of
age), with a male:female ratio of 2:1, involving the
bones of the jaw and other facial bones, as well as the
kidneys, gastrointestinal tract, ovaries, breast and other
extra-nodal sites. Epstein-Barr virus (EBV) positivity is
found in nearly all cases.
The sporadic or American variety (sBL) occurs
worldwide. It includes those cases occurring with no
specific geographic or climatic association. It accounts
for 1-2% of lymphomas in adults and up to 40% of
lymphomas in children in the U.S. and Western
Europe. The abdomen, especially the ileocecal area, is
the most common site of involvement. The ovaries,
kidneys, omentum and Waldeyer’s ring may also be
involved. Lymph node involvement is more common
in adults than in children. Neoplastic cells are EBV
positive in 15-30% of cases, or less.
Immunodeficiency-associated Burkitt lymphoma (iBL)
occurs mainly in patients infected with HIV and it is
frequently seen in patients with CD4counts greater
than 200 mm-3 unlike other HIV-related lymphomas
[7]. They are also seen in allograft recipients and
individuals with congenital immunodeficiency. Our
patient was negative for HIV serology.
Morphologically, classic Burkitt lymphoma consists of
medium-sized cells with abundant, basophilic
cytoplasm, often containing lipid vacuoles, round
nuclei with clumped chromatin and multiple nucleoli
with a diffuse, monotonous pattern of infiltration. A
“starry sky” appearance has been described in this type
of non-Hodgkin’s lymphoma because of its abundant
proliferative rate, frequent apoptoses and numerous
macrophages containing ingested apoptotic tumor cells.
Eighty percent of Burkitt lymphoma cases harbor
t(8;14) translocation [6], resulting in the juxtaposition
of the c-myc gene on chromosome 8 with IgH enhancer
elements on chromosome 14. In the remaining 20% of
cases, t(2;8) or t(8;22) are observed placing the c-myc
gene adjacent to either the kappa or lambda light chain
(IgL), respectively [6]. Patients with any of the three
clinical variants are at risk for diffusion to the central
nervous system (CNS) and bone marrow. The bone
marrow is positive in 30-38% and the CNS is involved
in 13-17% of adult cases [7].
The Ann Arbor and the St. Jude/Murphy staging
systems are the commonly used staging methods. Our
patient falls in the III B class of the St. Judes/Murphy
staging [7].
Features which have been associated with an adverse
outcome in adults and children include older age,
advanced stage, poor performance status, bulky
disease, high LDH, and CNS or bone marrow
involvement [6].
The cases reported from India [8, 9] are in the pediatric
age group, involving the stomach and the duodenum
with no direct pancreatic involvement. The important
clue to diagnosis in this case was the rapidity of
symptom progression and the elevated LDH levels.
Studies in pediatric populations have shown that a CT
scan is an important mode of investigation in
characterizing the lesion [10]. Two morphological
patterns were described on CT scan: a localized well-
circumscribed tumor and a diffuse enlargement
infiltrating the whole gland. A localized, well-
circumscribed form without obstructive jaundice is
described in the literature [11]. A CT scan of the
abdomen showed involvement of the head of the
pancreas in our case. In this instance, EUS FNA did
not yield a sufficient sample for a conclusive diagnosis.
This patient had low blood sugar levels. This was
different from other cases where temporary diabetes
mellitus requiring insulin was described [12]. This
could be due to the high tumor burden and poor
nutrition.
Finally, differentiation between diffuse large B cell
lymphoma (DLBCL) and Burkitt lymphoma is of
crucial importance since both prognosis and treatment
differ. The WHO criteria for Burkitt lymphoma include
classical/atypical histology, CD20+, bcl 6+, CD10+, Bcl
2-, CD5-, a Ki 67 score greater than 95% and IG-myc
+
[13]. Our patient met the above criteria to support the
diagnosis of Burkitt lymphoma.
CONCLUSION
Burkitt lymphoma of the pancreas is a rare, rapidly
progressing malignant neoplasia and can present with
varied clinical features. Its infrequent occurrence
among adults in India should not deter the clinician
from including it as a part of the differential diagnosis
in a patient with rapidly developing jaundice, an
abdominal mass and ascites. The importance of good
clinical acumen in the early diagnosis of the disease
cannot be overemphasized. The present case is a fitting
example of the importance of a timely diagnosis and
prompt treatment which will result in a favorable
clinical outcome.
Conflict of interest The authors have no potential
conflicts of interest
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689
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