Chronic ileocolic intussusception due to transmural infiltration of diffuse large B cell lymphoma in a 14-year-old boy: a case report

Intussusception is usually considered to be a readily diagnosable disease in terms
of the susceptible age (under 2 years old) and characteristic symptoms, including
intermittent abdominal pain, vomiting, bloody stools and palpable masses. The development
of symptoms depends on the degree of strangulation and obstruction of the bowel.

Chronic intussusception, lasting over 14 days, is a rare condition (Rees and Lari
1976]). The incidence of chronic intussusception has been reported to be 5.2% in all cases
of intussusception and is higher in patients age above 1 year of age (10.3%) than
in those with an age below 1 year (3.1%) (Macaulay and Moore 1955]). Chronic intussusception is non-strangulated and incompletely obstructing. Non-strangulating
intussusception may reduce spontaneously, progress to strangulation or remain stable
(Schulman et al. 1998]). In cases of ‘chronic’ intussusception, nonspecific symptoms, including diarrhea,
anorexia and weight loss, may be present, in addition to typical symptoms associated
with ‘acute’ intussusception (Macaulay and Moore 1955]; Reijnen et al. 1989]). These factors associated with chronic intussusception may make confirming the definitive
diagnosis of ‘chronic’ intussusception challenging, particularly when the initial
treatment is aimed to treat gastroenteritis, as described in our case (Shekhawat et
al. 1992]).

Two mechanisms of chronic intussusception can be assumed, that is: (1) reduction and
invagination may repeat spontaneously or (2) the intussusception may remain unchanged.
In the current case, the latter is an acceptable explanation because (1) we detected
findings compatible with intussusception on the CT scan obtained at the former clinic,
(2) the mucosa of the terminal ileum was severely erosive and edematous and (3) manual
reduction was impossible even after the excision. Although achieving hydrostatic reduction
of chronic intussusception is difficult (Rees and Lari 1976]; Macaulay and Moore 1955]), contrast enemas with or without low hydrostatic pressure may be useful for obtaining
the diagnosis. In this case, we performed contrast enema with low hydrostatic pressure
to obtain any information of leading point, which resulted in failure. Contrast enema
was not necessarily required in previously diagnosed chronic intussusception same
as our case. Because an organic lead point may frequently be present in patients with
chronic intussusception, early surgical intervention should be applied (Schulman et
al. 1998]; Reijnen et al. 1989]).

Although cases of intussusception in children are usually ‘idiopathic’, approximately
5% of patients have a pathological lead point, including Meckel’s diverticulum, duplication
cysts, polyps or lymphoma (Applegate 2009]). Intussusception occurring in older children and adults is accompanied by a significantly
higher incidence of coexisting neoplasms (Hsiao et al. 2013]). Although pathological lead points in the small intestine are usually benign, around
30% of cases are secondary to malignant lesions, including malignant lymphomas (Akbulut
2012]).

The gastrointestinal tract is one of the most common extranodal sites for non-Hodgkin
lymphoma. Approximately 80–90% of primary gastrointestinal tract lymphomas are of
B-cell origin (Li et al. 2008]). Lymphomas arising in the gastrointestinal tract present with various symptoms,
including abdominal pain, anorexia, weight loss, diarrhea and ileus (Koch et al. 2001]) and can be a rare pathological lead point of intussusception, as in our case (Shakya
et al. 2009]). Sometimes pediatric lymphomas may be related to underlying abnormalities of immunity
associated with either congenital causes, infections, such as with the Epstein Barr
virus, autoimmunity or inflammatory bowel diseases. In the present case, there were
no clinical findings indicating innate or acquired immunodeficiency. Furthermore,
using in situ hybridization, the tumor cells were found to be negative for Epstein
Barr virus-encoded small RNA (data not shown).

Although there are some reports of the non-operative management of lymphoma presenting
with intussusception (Lerner et al. 2011]; Kang et al. 2014]), the administration of chemotherapy under conditions of intussusception due to lymphoma
may have the potential risk of causing tumor lysis syndrome and perforation of the
intestine. If the patient may tolerate surgical intervention, resection of the affected
intestine is a reasonable strategy for reducing the tumor volume and making the correct
histopathological diagnosis. Gupta et al. reported that pediatric patients with Burkitt’s
lymphoma presenting with intussusception often have completely resectable disease
and assumed that the detection of intussusception may lead to an early diagnosis (Gupta
et al. 2007]). We assume that when the size of enteric lymphoma is small, the tumor tends to cause
intussusception rather than obstruction and that when the tumor grows too to be large
to cause invagination, the lesion may cause intestinal obstruction.