Non-Hodgkin lymphoma of the descending colon in a child with obstruction; a case report and review of literature
P Srivastava, A Gangopadhyay, D Gupta, V Upadhyaya, S Sharma
Keywords
descending colon, non-hodgkin lymphoma, obstruction
Citation
P Srivastava, A Gangopadhyay, D Gupta, V Upadhyaya, S Sharma. Non-Hodgkin lymphoma of the descending colon in a child with obstruction; a case report and review of literature. The Internet Journal of Surgery. 2008 Volume 21 Number 2.
Abstract
Primary gastrointestinal non-Hodgkin lymphoma (NHL) accounts for 13-18% of all malignant tumours of small bowel and only 1% of large bowel tumours. The most common site of non-Hodgkin lymphoma of the intestinal tract in children is the terminal ileum and the ileocecal region. The non-Hodgkin lymphoma (NHL) of descending colon is extremely rare in children .We report non-Hodgkin lymphoma of the descending colon in an 8-year-old child with features of large bowel obstruction due to a large intraluminal single polyp. The diagnosis revealed non-Hodgkin lymphoma. Early diagnosis is mandatory and multimodality treatment is required to prevent complications and prolong survival.
Background
Primary alimentary tract malignancies, including primary gastrointestinal non-Hodgkin lymphoma (NHL), are rare in children [1]. The incidence of primary colorectal lymphomas is rare, comprising 10-20% of cases of non-Hodgkin lymphoma with limited stage and high-grade disease [2]. Colorectal involvement accounts for between 10% and 20% of the cases in most studies of gastrointestinal tract lymphoma. Primary lymphomas of the descending and sigmoid colon are extremely rare in children [3]. When NHL involves gastrointestinal disease, it usually arises in the submucosal lymphoid tissue of the ileocecal region, with transmural extension and extensive local involvement of mesenteric lymph nodes, forming a bulky abdominal mass. The ileocecal valve is most frequently involved (35.8%), followed by the small bowel (31.3%), large bowel (19.4%) and multiple gastrointestinal involvements (13.4%) [4]. Surgery is an important treatment modality for primary colo-rectal NHL because it is localized, and supplemented by systemic chemotherapy in the form of CHOP regime.
Case Report
An 8-year-old boy presented to us with a history of left lower abdominal pain for 15 days. There was off-and-on fever for ten days but the symptom did not subside with medication. Then, after 2-3 days, he had an episode of vomiting and distension of the abdomen associated with blood in stool. Per-rectal examination was not significant. Abdominal ultrasonography [figure-1] showed a single homogeneous solid mass measuring 5.6 × 4.8cm located in the descending colon. Colonoscopy showed a single polypoidal mass that occluded the entire lumen of the descending colon [figure-2]. CT scan of the abdomen was done to confirm the site and extent of the lesion [figure-3]. The results of all routine blood tests were within normal limits. Explorative laparotomy was done and there was the intraluminal polypoidal mass present in the descending colon. It was resected along with the bowel segment with end-to-end colonic anastomosis. .Histological examination revealed the diagnosis of B-cell lymphoma, diffuse large cell type, which was positive for pan-B cell marker CD20. After the patient recovered from surgery, chemotherapy (six cycles) in the form of cyclophosphamide, vincristine, doxorubicin and prenisolone (CHOP) was administered. He remains well and free of disease symptoms after 2 years.
Discussion
Non-Hodgkin lymphoma is the third most common malignancy in childhood. Along with Hodgkin lymphoma it constitutes 15% of all malignancies in childhood [5]. Non-Hodgkin lymphoma is more common than Hodgkin lymphoma in this age groups and it occurs in the abdomen in approximately 35% of cases. Children with certain immunodeficiency syndromes are known to have predisposing factors for development of Non-Hodgkin lymphoma [6]. In children, the most common gastrointestinal lymphoma is located in the ileocecal junction as compared with adults in which the most common site is stomach. Descending colon and sigmoid colon are very uncommon sites for involvement of Non-Hodgkin lymphoma and only one case in a child has been described in world literature [7]. Boys are 5 to 10 times more frequently affected than girls and the peak incidence is between 5 and 8 years of age [7]. Mucosa-associated lymphoid tissue (MALT) lymphoma is the most frequent colonic lymphoma [8] and usually appears as an isolated polyp [9]. Although a causal effect has been identified in adults as to the development of a mucosa-associated lymphoid tissue (MALT) lymphoma from infection with
The most common presentations of symptoms are abdominal pain and abdominal mass but bowel obstruction, intussusceptions, perforation, and/or bleeding can be present in the form of complications. Imaging tests can not detect lesions in early stages and tests are often nonspecific but ultrasound of the abdomen can detect the mass-like lesion. The correct preoperative diagnosis is obtained only by colonoscopic biopsy or exicisional biopsy of the lesion by laparotomy.
Surgery is an important treatment modality for primary intestinal NHL because resection can reduce tumor burden and prevent hemorrhage, perforation, and secondary infection caused by chemotherapy-induced tumor necrosis [12]. The early diagnosis is important in all cases of children with bleeding per rectum and colonoscopy with biopsy, supplemented by imaging study, should be done to confirm the lesion and prolong survival by surgery and chemotherapy.