Burkitt's lymphoma of intestine presenting as ileocolic intussusception: An atypical presentation

Preeti Sharma; Sufian Zaheer; Somshankar Chowdhury; Ashish Kumar Mandal

doi:10.4103/ijhas.IJHAS_86_17

CASE REPORT

Year : 2018 | Volume : 7 | Issue : 3 | Page : 207-209

Burkitt's lymphoma of intestine presenting as ileocolic intussusception: An atypical presentation

Preeti Sharma, Sufian Zaheer, Somshankar Chowdhury, Ashish Kumar Mandal
Department of Pathology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India

Date of Web Publication

20-Jul-2018

Correspondence Address:
Dr. Preeti Sharma
Department of Pathology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi - 110 029
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/ijhas.IJHAS_86_17

Abstract

Burkitt's lymphoma is the most common B-cell lymphoma of childhood rarely localized to the gastrointestinal tract. Further, intussusception secondary to Burkitt's lymphoma is an uncommon presentation. We describe an unusual case of intestinal Burkitt's lymphoma in a 4-year-old boy who presented to the emergency department with intermittent colicky pain. Imaging studies were suggestive of intussusception which was reduced on exploratory laparotomy. Gross examination of the resected bowel revealed a gray-white to pink fleshy tumor encompassing 90% of the ileocecal valve circumference. Histopathology supplemented with immunohistochemistry was suggestive of Burkitt's lymphoma. Owing to rather nonspecific clinicoradiological features, its preoperative diagnosis remains a challenging task for the pediatric surgeons as well as radiologists.

Keywords: Aggressive, Burkitt lymphoma, intestine, intussusception

How to cite this article:
Sharma P, Zaheer S, Chowdhury S, Mandal AK. Burkitt's lymphoma of intestine presenting as ileocolic intussusception: An atypical presentation. Int J Health Allied Sci 2018;7:207-9

How to cite this URL:
Sharma P, Zaheer S, Chowdhury S, Mandal AK. Burkitt's lymphoma of intestine presenting as ileocolic intussusception: An atypical presentation. Int J Health Allied Sci [serial online] 2018 [cited 2023 Jun 6];7:207-9. Available from: https://www.ijhas.in/text.asp?2018/7/3/207/237266

Introduction

Primary tumors of the gastrointestinal tract are rare in children and represent <5% of all pediatric neoplasms.^[1] Among these, Burkitt's lymphoma is an uncommon and aggressive form of non-Hodgkin's lymphoma (NHL) accounting for 40% of all childhood NHLs.^[2]

Burkitt's lymphoma is classified into three clinical forms, i.e., endemic, sporadic, and Human immunodeficiency virus acquired immune deficiency syndrome associated. Of these, the sporadic form occurs in the lymphoid tissues of the gut and most commonly presents as an abdominal mass with symptoms such as abdominal pain, nausea, and intestinal obstruction.^[3] Albeit rare, Burkitt's lymphoma may present as acute abdomen due to intussusception more so in the pediatric population.^[4] Thus, nonspecific and misleading symptoms of intestinal Burkitt's lymphoma make this condition a diagnostic dilemma for the clinicians and radiologists at times.

Keeping in view these considerations, we are presenting a case of pediatric intestinal Burkitt's lymphoma presenting with intussusception.

Case Report

A 4-year-old male child presented to the emergency department with sudden onset of severe lower abdominal pain, low-grade fever, and nonbilious vomiting for 1 day. There was a history of intermittent periumbilical colicky pain and loss of weight for 1 month. There was no history of any bladder or bowel disturbances. On examination, the child was mildly dehydrated and febrile with pulse rate 94/min and blood pressure 110/80 mmHg. Per abdomen examination revealed a distended and tender abdomen with guarding and rigidity. Baseline laboratory investigations showed that the child was anemic with hemoglobin 9.6% gm. All other routine laboratory investigations were within normal limits. Computed tomography (CT) revealed well-defined loop within loop appearance associated with the mesenteric vessels. With a clinicoradiological diagnosis of ileocecal intussusception patient was immediately taken up for exploratory laparotomy and reduction of the intussusception. On reduction, a firm mass was identified at the ileocecal junction. There were no palpable mesenteric lymph nodes identified. The resection of the affected area of ileocecal junction along with the mass was done with end-to-end ileocolic anastomosis. Gross examination of the resected bowel consisted of a 10.5 cm segment of ileum and cecum with appendix. A gray-white to pink fleshy tumor was identified measuring 4 cm × 3 cm × 2 cm and encompassing 90% of the ileocecal valve circumference [Figure 1]a. The overlying mucosa was ulcerated, and the tumor was involving full thickness of the bowel.

Figure 1: (a) Gross specimen of the resected bowel showing a gray-white to pink fleshy tumor with ulcerated mucosa and involving full thickness of the bowel. (b) Photomicrograph shows diffuse infiltration of muscularis propria by monomorphic lymphoid cells (H and E, ×100). (c) The lymphoid cells are medium sized with scant amount of eosinophilic cytoplasm and central nucleus showing increased nucleus: cytoplasm ratio and 2–3 nucleoli (H and E, ×400). (d) Interspersed in between the atypical lymphoid cells tumor cells are histiocytes giving a “starry-sky” appearance (H and E, ×100). On immunohistochemistry, lymphoid cells show positivity for (e) leukocyte common antigen (Immunostain, ×100), (f) CD20 (Immunostain, ×200), and (g) Bc-l6 (Immunostain, ×200). (h) The mean Ki-67 labeling index is 97% (Immunostain, ×200)

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Microscopy showed diffuse infiltration of mucosa, submucosa, and muscularis propria by monomorphic lymphoid cells [Figure 1]b. These cells were medium sized with scant amount of eosinophilic cytoplasm and central nucleus showing increased nucleus: cytoplasm ratio and 2–3 nucleoli [Figure 1]c. Brisk mitotic activity including numerous atypical forms was noted. Interspersed in between these tumor cells were histiocytes giving a “starry-sky” appearance [Figure 1]d. Areas of hemorrhage and necrosis were also seen. A preliminary pathological diagnosis of high-grade NHL possibly Burkitt's lymphoma was made which was further confirmed by immunohistochemical positivity for leukocyte common antigen [Figure 1]e, CD20 [Figure 1]f, Bcl-6 [Figure 1]g, and negative expression of CD3, CD5, CD15, CD30, and Bcl-2. Ki-67 labeling index was 97% [Figure 1]h.

The patient was subsequently subjected to adjuvant chemotherapy (cyclophosphamide, vincristine, prednisolone, and doxorubicin regime) and remained without any evidence of disease at 6-month follow-up.

Discussion

NHL is the third most common pediatric cancer and is subdivided into three histological subtypes, i. e., B-cell NHL (65%) including both Burkitt's lymphoma and diffuse large B-cell lymphoma, lymphoblastic (20%), and anaplastic large cell lymphoma (15%).^[5] Malabsorption syndromes and immunodeficiency states are well-established predisposing factors for NHLs of the intestine.^[6] Our patient, however, was previously healthy, and no predisposing factor was identified. Burkitt's lymphoma has a classical predilection for the head and neck region in children with the gastrointestinal tract and kidneys being potential sites of involvement. Ileocecal region is the most common site of involvement in the pediatric population owing to the high concentration of lymphoid tissue in this region.^[5] Presumably arising in lymphoid follicles or Peyer's patches, Burkitt's lymphoma grows submucosally underneath an intact mucosa. With advancement, ulceration of the mucosa occurs as seen in our case. Due to the aggressive behavior and volumetric doubling of this neoplasm, the patient may present with acute abdomen secondary to bowel obstruction, bleeding, or rarely intussusception.^[3]

An approximate incidence of 1–4/2000 children is reported for intussusception which is regarded as a pediatric surgical emergency. Children <2 years of age are most commonly affected in 75% of cases. A pathological lead point such as Meckel's diverticulum, polyp, hematoma, and lymphoma can be identified in 1.5%–12% of cases more so in older children.^[3] Lymphoma, although uncommon, arouses the most concern due to its malignant nature.^[7]

The most common presenting symptom as reported in the Indian literature is abdominal pain (81.4%), followed by abdominal swelling, vomiting, constipation, intestinal obstruction, and bleeding per rectum. Another study stated intestinal obstruction as the most common mode of presentation.^[8] Our patient presented predominantly with abdominal pain.

Infrequently intussusception is the first clinical sign in Burkitt's lymphoma. While on the one hand, it is a potentially life-threatening pediatric emergency, on the other, it leads to early detection of the disease.^[3] Imaging modalities especially doughnut sign on ultrasonography and target sign on CT scan in combination with clinical examination are nearly 100% diagnostic for intussusception. Dawson's criteria must be met for the diagnosis of gastrointestinal lymphomas. These criteria include (1) absence of peripheral lymphadenopathy at the time of presentation, (2) lack of enlarged mediastinal lymph nodes, (3) normal total and differential white blood cell count, (4) predominance of bowel lesion at the time of laparotomy with only lymph nodes obviously affected in the immediate vicinity, and (5) no lymphomatous involvement of the liver and spleen.^[9]

Various treatment modalities have been established including surgical excision, chemotherapy, radiotherapy, and radioimmunotherapy alone and in different combinations for the management of Burkitt's lymphoma. Being extremely chemo-sensitive, therapy courses of cyclophosphamide, methotrexate, cytarabine, and other chemotherapeutic drugs give excellent results. Approximately 3% patient population die from treatment complications, especially tumor lysis syndrome.Complete surgical excision of the tumor followed by chemotherapy can cure the disease in small localized tumors. An extensive involvement of the tumor in the abdomen needs CT guides biopsy for the diagnosis followed by multiagent chemotherapy.^[10]

Conclusion

Burkitt's lymphoma is a highly malignant and aggressive neoplasm owing to its volumetric doubling. It must be kept in mind as a differential diagnosis in children presenting with acute abdomen due to intussusception. Surgical excision followed by histomorphological and immunohistochemical assessment remains the gold standard for diagnosis. A multidisciplinary approach assures efficient therapeutic management.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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