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ORIGINAL ARTICLE

Primary pediatric gastrointestinal lymphoma

Ranjana Bandyopadhyay, A B S T R A C T
Swapan Kumar Sinha1, Uttara
Background: Primary non-Hodgkin’s lymphoma (NHL) of the gastrointestinal (GI) tract
Chatterjee2, Dipanwita Nag1, is the most common extranodal lymphoma in pediatric age group. Yet, the overall
Subhalakshmi Mukhopadhyay1, incidence is very low. The rarity of the disease as well as variable clinical presentation
Supriyo Roy Chowdhury1, prevents early detection when the possibility of cure exists. Materials and Methods:
We studied six cases of primary GI NHL in pediatric age group with reference to their
Pranab K. Biswas1 clinical presentation, anatomic distribution and histopathologic characteristics. Results:
Department of Pathology, Burdwan All were males except one. Intestinal obstruction was the presenting feature in 50%.
Medical College, 1Medical College, Half the cases showed ileocaecal involvement, while large bowel was involved in 16%.
Kolkata, 2Institute of Postgraduate Histology showed four cases of diffuse large B-cell lymphoma (DLBCL), one case of
Medical Education & Research, Burkitt lymphoma, and one Burkitt-like lymphoma. Immunohistochemistry for Tdt, CD20,
Kolkata, West Bengal, India CD3, CD30, bcl2, bcl6 confirmed the morphological diagnosis. Conclusion: Pediatric
GI lymphoma commonly involves the ileocaecal region and presents with intestinal
Address for correspondence: obstruction. A higher prevalence of DLBCL is found compared to other series. A high
Dr. Ranjana Bandyopadhyay, proliferative index is useful in differentiating Burkitt-like lymphoma from DLBCL.
1B/3, Uttarpara Housing Estate,
88 B, G T Road, P.O. Bhadrakali,
Dist. Hooghly, Pin - 712 232,
West Bengal, India. Key words: Gastrointestinal tract, non-Hodgkin’s lymphoma, pediatric
E-mail: drranjanapatho@[Link]

adenopathy at diagnosis, no mediastinal adenopathy at chest

INTRODUCTION
radiography, normal blood cell counts, no involvement of
Primary tumors of the gastrointestinal (GI) tract are rare liver and spleen, and involvement of only regional lymph
in children and represent less than 5% of all pediatric nodes at laparotomy.[3] The presenting symptoms were
neoplasms.[1] The rarity of the disease and variable clinical registered; extent of disease was determined by history,
presentation prevent early detection when the possibility physical examination, baseline complete hemogram, liver
of cure exists. Non-Hodgkin’s lymphoma (NHL) remains function tests, lactate dehydrogenase (LDH) as a tumor
the most common malignancy of the GI tract in children.[2] bulk indicator, uric acid, serum electrolytes, bone marrow
They usually have different anatomic distribution and biopsy, abdominal ultrasound and/or contrast-enhanced
histologic appearance compared to common patterns in computed tomography (CECT) scan of the abdomen.
adult cases. We studied six cases of primary GI lymphoma All pathologic specimens were reviewed and classified
in pediatric age group with reference to clinical presentation, according to World Health Organization (WHO) –
anatomic distribution and histopathologic characteristics. REAL classification. Five-micron thick sections were
cut and stained with Hematoxylin and Eosin (H and E).
Immunohistochemical staining for CD20, CD3, Tdt, Bcl2
MATERIALS AND METHODS and Bcl6 was performed. Ki67 index were determined in
selected cases. The tumors were staged according to St.
We studied six pediatric patients with provisional diagnosis of Jude’s staging system.[4]
extranodal NHL in GI tract over a 3-year period. According
to criteria developed by Dawson and colleagues, primary
lymphoma of GI tract includes cases with no superficial RESULTS

Access this article online Six cases of pediatric GI lymphoma were identified over
Quick Response Code: a period of 3 years. The age at presentation ranged from
Website:
[Link] 1 to 8 years. All of these patients were boys except one
girl child who presented at a very early age (1 year). The
DOI:
commonest presentation was intestinal obstruction present
10.4103/0971-5851.89786 in three of the six (50%) patients. Other presentations
included asymptomatic abdominal lump, abdominal

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Bandyopadhyay, et al.: Pediatric gastrointestinal lymphoma

pain, and pain along with vomiting. Ileocecal region was

Limited resection followed by chemotherapy

Limited resection followed by chemotherapy

Radical resection followed by chemotherapy
Radical resection followed by chemotherapy

Almost Radical resection followed by chemotherapy

Radical resection followed by chemotherapy

the most common site of involvement (50%) followed
by terminal ileum (33%). Only one patient (16%) had
involvement of the large gut in the region of sigmoid colon
[Table 1].

Surgical resection was performed in all six patients and

the gross specimen showed nodular growth ranging from
2.5 to 5 cm in diameter in four cases, and the remaining
two had infiltrative growth involving cecum and sigmoid

Treatment
colon, respectively, with thickening of their walls. One
of the lesions in the terminal ileum was accompanied by
regional lymphadenopathy.

Ki67

100
(%)
99
46

56
54
72
Histology of the lesions showed classical Burkitt lymphoma
(BL) in one case with diffuse sheets of small tumor

Tdt CD20 CD3 CD30 Bcl2 Bcl6

+
+
+

+
+

-
cells having round to oval nuclei and several prominent
basophilic nucleoli. The chromatin was coarse and nuclear

+
-

-
membrane was thick. This case showed a prominent starry-

Table 1: Clinical data, histopathology and staging of pediatric gastrointestinal lymphoma

sky pattern [Figure 1]. The tumor was positive for B-cell

+
-
-

-
-

-
marker CD20, negative for CD3, Tdt and showed a high
proliferative index [Figure 2]. In the second case from

-
-
-

-
-

-
terminal ileum, the cells were larger and more atypical with
few binucleate forms. This tumor also showed a very high

+
+
+

+
+

+
proliferative index and was diagnosed as atypical Burkitt.

-
-
-

-
-

-
The remaining four cases showed histology of a diffuse
large cell lymphoma and were positive for B-cell marker St. Jude’s
CD20 and negative for CD3 [Figure 3]. stage
III

III
II
II

II

II
None of these patients showed generalized lymphadenopathy
Atypical Burkitt

or involvement of bone marrow. Cutaneous nodules at a

distant site were observed in two (33%) cases. The patient
Histology

DLBCL
DLBCL

DLBCL

DLBCL
Burkitt
having atypical BL on histology developed a nodule on the
lateral side of the back, approximately 2 cm in diameter,
15 days after the operative procedure. Another patient
Abdominal pain and vomiting

with primary ileocecal mass showed diffuse large B-cell

lymphoma (DLBCL) on histology and presented with a
Intestinal obstruction

Intestinal obstruction

skin nodule over the abdominal wall, 1 month after surgery.

On histology, this skin nodule was seen to be composed
Abdominal pain
Intussusception

Ileocecal mass
Presentation

of diffuse sheets of tumor cells infiltrating the dermis,

M – Male; F – Female; DLBCL – diffuse large B-cell lymphoma

subcutaneous tissue and skeletal muscle [Figure 4]. LDH

levels were increased in all the cases. The tumor staging
was done according to the St. Jude’s system for childhood
M Appendix, mesentry

NHL.[4] Four patients (66%) presented in stage II and the

and terminal ileum

remaining two (33%) had stage III disease. Stage I and II

M Terminal ileum

Terminal ileum
M Sigmoid colon

tumors were subjected to primary radical resection followed

M Ileocecal

M Ileocecal

by multiagent CHOP-like chemotherapy (six cycles).

Age Sex Site

Patients in stage III were subjected to conservative surgery

followed by chemotherapy. In the 3-year follow-up period,
F

two of our patients died (one DLBCL and one atypical

(years)

Burkitt). Both of them had stage III disease. The remaining

4½
2½

8
5

1
7

four patients fared well during this 3-year period without

any relapse. None of these patients developed tumor lysis
Case

syndrome during chemotherapy.

6
4
2

5
3
1

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Bandyopadhyay, et al.: Pediatric gastrointestinal lymphoma

Figure 1: Photomicrograph showing the typical starry-sky appearance Figure 2: Photomicrograph showing high Ki67 index in a case of
of a case of Burkitt lymphoma (H and E, ×400) atypical Burkitt lymphoma (Ki67 immunostaining, ×400)

Figure 3: Photomicrograph showing CD20 positive cells in a case of Figure 4: Photomicrograph showing diffuse infiltration of large lymphoid
diffuse large B-cell lymphoma (CD20 immunostaining, ×400) cells in between skeletal muscle fibers (H and E, ×400)

intussusception. The commonest presenting symptom

DISCUSSION
is reported to be abdominal pain (81.4%), followed by
abdominal swelling, vomiting, constipation, diarrhea,
Malignant lymphomas are the third most common type
and intestinal obstruction.[9] In our patients, intestinal
of childhood cancer.[5] It is important to identify the
obstruction was the commonest mode of presentation.
distinction between NHLs of adults and those of children.[6]
Children typically present with diffuse extranodal disease Nearly 50% of children with GI NHL have tumor infiltrates
in contrast to adults among whom primary nodal disease is confined to GI tract with possible regional lymph node
common. Primary GI malignancies are a rarity in children, involvement.[2] In the present series, all six patients had
with limited information from Asian population.[7] intestinal growth with two of them showing regional lymph
node involvement. Two patients developed cutaneous
The peak age for NHL of GI tract in children is 5–15 nodules, one over the back and other over the abdominal wall.
years.[8] Three patients in this present series presented Subcutaneous nodules are very rare in BL and DLBCL.[10,11]
below 5 years, with one of them presenting at 1 year. The Seeding of skin and subcutaneous tissue after surgical
patients presenting below 5 years are reported to have only procedure with subsequent rapid growth have been reported
marginally better outcome (5-year survival 82.9% compared in BL cases. Seeding has been documented in one case
to 76.9%).[9] Unlike adult patients in whom stomach is the of abdominal BL. However, satellite lesions and regional
most frequent site, small and large intestines are the most metastasis not directly communicating with the main tumor
commonly involved sites in pediatric age group.[10] Five have also been reported in an area distal to the previous
out of six patients in the present study had involvement surgical field.[10] Similarly, in our case, the site of the nodule
of small intestine with colonic involvement in only one was distant from the site of surgery. Recently, a case of gastric
case. The male to female ratio of childhood GI NHL is DLBCL with cutaneous involvement has also been reported.[12]
reported to be from 7:1 to 1.8–2.5: 1.[2,9] There was only one According to St. Jude’s staging system, four of them had
female child amongst the six cases in our series. Clinically, stage II and two had stage III disease. Other studies have
the patients presented with varied symptoms ranging from also found tumors presenting in stage II as well as in stage III
abdominal mass to acute abdominal emergency caused by and IV disease with significant survival advantage in stage II

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Bandyopadhyay, et al.: Pediatric gastrointestinal lymphoma

compared to stage III and IV.[13] All six patients in this series like lymphoma from cases of DLBCL. Detailed study
did well in the postoperative period and all of them were of morphology, immunohistochemistry and proliferative
given systemic chemotherapy. Histologically, four of our cases index would help in solving that issue.
were diagnosed as DLBCL based on cellular morphology and
immunohistochemistry. In a study from Pakistan, they have REFERENCES
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How to cite this article: Bandyopadhyay R, Sinha SK, Chatterjee U,
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Source of Support: Nil, Conflict of Interest: None declared.
addition, one should be careful to differentiate Burkitt-

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