Diffuse large B-cell lymphoma associated ileocecal intussusception in adulthood

Summary

Background: Intussusception in adults is considered a rare condition, accounting for 5% of all cases of intussusceptions and approx. 1–5% of bowel obstruction. Almost half intussusceptions of the bowel are associated with malignant disease; thus, we should also treat the underlying malignancy. Case description: A 52-year-old male presented with colicky right lower abdominal pain for a 6-month period. He had a weight loss of 20 kg within 6 months. Physical examination revealed a tender right lower abdominal mass. Colonoscopy showed a mass that filled the ileocecal. The digestive surgeon performed laparoscopic right hemicolectomy with end-to-end anastomosis. Histopathology examination showed diffuse proliferation of large tumor cells with centroblastic-like features prominently in submucosal area, with normal epithelial mucosa. The immunohistochemistry result concluded the final dia­gnosis of diffuse large B-cell lymphoma. RCHOP chemotherapy regimens were administered every 3 weeks for 6 cycles. The response was complete remission. Discussion: Intussusception was preoperatively dia­gnosed by multi-slice spiral CT scans with the characteristic target or sausage sign, edematous bowel wall and mesentery in the lumen. After surgery, approximately 90% of adult intussusception cases have a demonstrable etiology. Malignant lymphoma, especially diffuse large B-cell lymphoma, of the ileocecal is one cause of the adult intussusception. Conclusion: Adult bowel intussusception is a rare clinical entity. Abdominal CT is considered as the most sensitive imaging modality in the dia­gnosis of intussusception. Diffuse large B-cell lymphoma is the most common cause of ileocecal intussusception.

Keywords:

Diffuse large B-cell lymphoma – Intussusception – bowel obstruction – malignant lymphoma

Introduction

Intussusception of the bowel is defined as the telescoping of a proximal segment of the gastrointestinal tract within the lumen of the adjacent segment. If we compare to children, bowel intussusception in adults is considered a rare condition, accounting for 5% of all cases of intussusceptions and approx. 1–5% of bowel obstruction [1]. It can present with a variety of acute, intermittent, and chronic vague and nonspecific symptoms, thus making its preoperative dia­gnosis difficult. CT scan of the whole abdomen proved to be the most useful dia­gnostic radiologic method. The treatment option of adult intussusception is surgical resection. Almost half intussusceptions of the bowel are associated with a malignant disease; thus, we should also treat the underlying malignancy.

Case description

A 52-year-old male presented with colicky right lower abdominal pain for a 6-month period. He also complained constipation, nausea, and vomiting. He had a weight loss of 20 kg within 6 months. Physical examination revealed a tender right lower abdominal mass. Laboratory findings showed mild anemia with a hemoglobin level of 11 g/dL, leukocytosis with white blood cells count 14,620/µL, and normal thrombocyte with platelet count 280,000/µL. Renal, liver function, and blood glucose were within normal limits. Lactate dehydrogenase (LDH) was also normal – 329 U/L (norm: 240–480 U/L). The level of carcinoembryonic antigen (CEA) was normal. Colonoscopy showed a mass that filled the ileocecal and did not allow the scope to enter (Fig. 1). CT scan of the whole abdomen showed the structure of ileum, mesenteric fat, and blood vessels invaginated into the structure of the cecum extension to the proximal of colon transversum with multiple lymphadenopathies around the mesenterium without sign of obstructive ileus (Fig. 2).

The digestive surgeon performed laparoscopic right hemicolectomy with end-to-end anastomosis. The resected colon section can be seen in Fig. 3. The result of histopathology examination showed diffuse proliferation of large tumor cells with centroblastic-like features prominently in submucosal area, with normal epithelial mucosa. The immunohistochemistry result (Fig. 4) showed positive result for B-cell marker CD20 and negative for T-cell marker CD3, epithelial marker AE1/3 and high Ki67 proliferation index (60%). The final dia­gnosis was diffuse large B-cell lymphoma.

We administered the RCHOP regimen every 3 weeks for 6 cycles. The response was complete remission (Fig. 5). The patient tolerated the chemotherapy well, although he developed neutropenia, dyspepsia, nausea and vomiting, diarrhea, alopecia, and peripheral neuropathy during chemotherapy.

Discussion

Intussusception occurs if a proximal portion of the bowel invaginates into the distal bowel [2]. It can be classified into three types based on its location: (1) enteroenteric, when confined to the small bowel; (2) colocolonic, when involving the large bowel; (3) enterocolonic, which can be ileocecal or ileocaeco-colonic [3]. It looks like that this patient suffered from ileocecal intussusception. The pre­sentation of adult intussusception can be acute, subacute, or chronic non-specific symptoms therefore the initial dia­gnosis is often delayed. Yakan et al reported a retrospective review of adult patients with a dia­gnosis of intestinal intussusception that pain was the most common presenting symptoms (85%) followed by nausea, vomiting, constipation, rectal bleeding, and diarrhea [4]. This patient presented with chronic colicky right lower abdominal pain, constipation, nausea, vomiting, rectal bleeding, and weight loss.

Intussusception was preoperatively dia­gnosed by multi-slice spiral CT scans with the characteristic target or sausage sign, edematous bowel wall and mesentery in the lumen. Abdominal CT scan has been reported to be the most useful imaging technique to dia­gnose intussusception, with a dia­gnostic accuracy is 58–100%. Additional valuable information, such as metastasis or lymphadenopathy, is readily obtained by CT and may point to an underlying pathology. CT scan of the whole abdomen in this patient showed a characteristic finding of intussusception.

Treatment of adult intussusception usually requires resection of the involved bowel segment with primary anastomosis [4,5]. After surgery, approximately 90% of adult intussusception cases have a demonstrable etiology. Malignant lymphoma, especially diffuse large B-cell lymphoma, of the ileocecal is one cause of the adult intussusception [5]. The digestive surgeon performed laparoscopic right hemicolectomy with primary anastomosis. Since the result of histopathology and immunohistochemistry was diffuse large B-cell lymphoma, we administered chemotherapy RCHOP to the patient resulting in a good response.

Conclusion

Adult bowel intussusception is a rare clinical entity. Its dia­gnosis is usually delayed due to nonspecific symptoms. Abdominal CT is considered as the most sensitive imaging modality in the dia­g­­- nosis of intussusception. Diffuse large B-cell lymphoma is the most common cause of ileocecal intussusception. Laparoscopic hemicolectomy of the segmental intussusception followed by chemotherapy RCHOP is the preferred treatment.

Acknowledgements

This paper is self-funded.

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.
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Submitted/Obdrženo: 30. 11. 2021
Accepted/Přijato: 11. 2. 2022

Dimas Priantono, MD
Division of Hematology-Medical
Oncology
Department of Internal Medicine
Dr. Cipto Mangunkusumo General
Hospital
Jl. Diponegoro No. 71
Jakarta Pusat
DKI Jakarta 10430
Indonesia
e-mail: dimas.priantono@gmail.com