Cecal obstruction: a rare presentation of childhood intestinal lymphoma in a South Sudanese child: a case report

Abstract

Introduction and importance:

Primary colonic lymphoma, a rare disease, is seldom encountered in children, infrequently causing an acute abdomen that requires surgical intervention. The true incidence is unknown, and early diagnosis is uncommon due to its rarity and variable clinical presentation. We report a case of a pediatric cecal lymphoma incidentally discovered during laparotomy.

Case presentation

A 5-year-old boy presented with severe abdominal pain, distension, vomiting, and fever. His clinical presentation and imaging suggested intestinal obstruction. Laparotomy revealed a large cecal mass, mesenteric lymphadenopathy, and a histopathologic finding of primary non-Hodgkin’s lymphoma (NHL).

Clinical discussion

Primary lymphoma of gastrointestinal (GI) tumors is rare in children, with a peak age of 5–15 years. NHL in children includes lymphoblastic, Burkitt, diffuse, and anaplastic types. Symptoms include abdominal pain, abdominal distension, vomiting, constipation, diarrhea, and sometimes bowel obstruction. Case series from India and Bangladesh reported that bowel lymphoma in children preferentially involves the ileum, followed by the ileocolic region, and is rarely limited to the cecum. Emergency surgery is often necessary for palliation and diagnosis through biopsy, as well as for removing symptomatic limited disease from an affected organ. There is still no consensus on the best treatment for symptomatic limited disease affecting an organ or the timing of chemotherapy.

Conclusion

The case report emphasizes the importance of pediatric cecal obstruction due to B-cell NHL as a differential diagnosis for intestinal obstruction in children. Thus, pediatricians should also keep in mind the rare causes, especially in children presenting beyond the typical age-group of idiopathic intussusceptions, as GI lymphoma needs to be ruled out.

Introduction

Primary colonic lymphoma is a rare entity, comprising about 0.2–1% of all colonic malignancies^[1^]. Primary cecal non-Hodgkin’s lymphoma (NHL) is a rarely encountered but frequent cause of acute abdomen that requires surgical intervention in children^[2^]. The true incidence is unknown, and existing literature consists only of case reports and small case series^[2^]. The scarcity of the disease and its variable clinical presentation preclude early diagnosis when the likelihood of cure exists. For this reason, the correct preoperative diagnosis is rarely made, and surgeons tend to be confronted with such a rare condition at the time of laparotomy. This study documents the experience of pediatric bowel lymphoma in a tertiary care institute, which was incidentally encountered intraoperatively in the differential diagnosis of acute abdomen.

HIGHLIGHTS

• Primary colonic lymphoma, a rare disease, is seldom encountered in children and infrequently causes an acute abdomen requiring surgical intervention.

• The true incidence is unknown, and early diagnosis is rare due to its scarcity and variable clinical presentation.

• We report a case of a pediatric cecal lymphoma, incidentally encountered during laparotomy.

• The case highlights the importance of considering lymphoma as a possible cause of intestinal obstruction in children, especially those older than the usual age for idiopathic intussusception.

• We find fewer such cases where the submission of this work to this journal may build awareness among the professional community and medical students up on dissemination.

This case report is submitted in line with the SCARE GUIDELINE checklist^[3^].

Case presentation

A 5-year-old boy presented to the surgical emergency department with a complaint of worsening abdominal pain of 3 months’ duration. At presentation, the pain was associated with multiple episodes of bilious vomiting, abdominal distension, failure to pass feces and flatus, low-grade fever, and anorexia. Prior to his emergency presentation, he had experienced progressive weight loss, poor feeding habits, and night fevers. The parents noticed that the boy was not himself, frequently reporting fatigue and body aches. He had no medical or surgical history and no family history of any specific chronic disease.

At admission, acutely sick-looking on a chronic basis, irritable but alert. He had dry mucous membranes and slightly pale conjunctivae. Febrile to touch, with an axillary temperature of 38.5°C, tachycardia with a feeble pulse of 145/min, a respiratory rate of 35 breaths per minute, and an SaO₂ of 95% on room air.

Abdominal examination revealed a tender, moderately distended abdomen, a palpable right iliac fossa mass with limited mobility measuring 5 cm × 6 cm grossly, voluntary guarding with no rebound, exaggerated small bowel sounds, and an empty rectum on digital per rectal examination. An erect abdominal X-ray revealed dilated small bowel loops, no air–fluid levels, and a paucity of rectal shadow (refer to Fig. 1). Ultrasound scan of abdomen: Grossly dilated colonic hepatic flexure, heterogeneous solid mass located at the ileocecal region measuring ~5.5 cm × 6 cm in size, and moderate ascites.

Figure 1.

Both (A) erect abdominal X-ray and (B) supine abdominal X-ray show dilated small bowel loops extending from the central to the left upper quadrant (red arrows).

Laboratory parameters showed white blood cell count of 7500/mm³, left shift with 77.8% neutrophils, 19.3% lymphocytes, Hg 10.2 g/dL, and platelets 812 000/UL. Blood chemistry showed creatinine 0.9 mg/dL, urea 40.3 mg/dL, Na⁺ 135 mmol/L, K⁺ 4.40 mmol/L, and Cl 93.2 mmol/L.

The initial assessment indicated a small bowel obstruction (SBO) attributed to an obstructing cecal mass, intussusception, and tuberculosis (TB). Resuscitation commenced with a fluid bolus of 20 mL/kg of normal saline (0.9%), which was administered over a 20-minute period. Following this, maintenance fluids were provided on an hourly basis. Empirical antibiotics were administered: intravenous ceftriaxone at 50 mg/kg/day in divided doses twice daily, intravenous metronidazole at 15 mg/kg/day in divided doses thrice daily, and intravenous paracetamol at 15 mg/kg/day thrice daily. He produced adequate urine output (~40 mL/h), which was collected in the urine bag over a 2-h period.

Subsequently, exploratory laparotomy revealed a huge obstructing cecal mass measuring ~6 cm × 5 cm, entirely involving the cecum with surrounding mesenteric LAP limited to the cecal and terminal ileum mesentery, along with involved ileocecal regional nodes, dilated proximal small bowel loops, and collapsed large bowel loops. The liver and spleen were free of secondary deposits, and other viscera grossly appeared normal (refer to Fig. 2).

Figure 2.

(A): Intraoperative finding of cecal mass (black arrow), and (B): serosal slough due to tumor involvement (green arrow), mesenteric lymphadenopathy (LAP) (blue arrow).

The patient under went right hemicolectomy and ileo-transverse end-to-side anastomosis. Histopathologic examination revealed a primary NHL of the cecum, limited to the cecal region, with negative resection surgical margins. Postoperatively, the patient had an uneventful course and was linked to the pediatric oncology unit at a children’s hospital. BCL and CD20 markers were not feasible due to resource limitations.

Clinical discussion

Primary tumors of the gastrointestinal (GI) tract are rare in children and represent less than 5% of all pediatric neoplasms^[4^]. The reported peak age is 5–15 years for NHL of the GI tract in children^[5^]. The male-to-female ratio of childhood GI NHL is reported to range from 7:1 to 1.8–2.5: ^[6,7^],. In children, NHL comprises four broad categories: lymphoblastic lymphoma, Burkitt lymphoma, diffuse large B-cell lymphoma, and anaplastic large cell lymphoma^[8^]. Case series reported from Indian children indicate that bowel lymphoma in children preferentially involves the ileum (81%), followed by the ileocolic 23%^[4^]. Abdominal pain is the most common presenting symptom (81.4%), followed by abdominal swelling, vomiting, constipation, diarrhea, and intestinal obstruction^[5^]. In the present study, the patient presented with localized disease (stage II-Ann Arbor). Primary cecal lymphoma presenting as acute abdomen often poses a challenge to optimal management due to the requirement of urgent intervention, mostly with misdiagnosis^[2^]. These pathologies are commonly misdiagnosed as they often mimic other common childhood acute abdominal pathologies. Surgeons may encounter unexpected pathologies during surgery due to preoperative misdiagnosis, which may increase mortality and morbidity and sometimes lead to unnecessary surgical procedures, as there is no standard treatment. Due to its symptoms mimicking TB and with TB still being a serious problem in developing countries, this patient’s diagnosis was initially presumed to be abdominal TB. There is, as yet, no consensus on the optimum treatment for symptomatic limited disease affecting an organ and the timing of chemotherapy perioperatively. Emergency surgery for complications of NHLs is mainly for palliation and diagnosis^[9^]. However, surgery may be useful in confirming or refuting equivocal radiological diagnoses through biopsy and removing symptomatic limited disease from an affected organ^[9^]. The diagnosis of NHL is suspected based on ultrasound or computed tomography scans, but the definitive diagnosis of GI lymphoma is made by biopsy of the tissue and histopathologic examination. In this case, we finally performed a biopsy and confirmed the diagnosis as diffuse NHL through histopathologic examination. There is as yet no consensus as to the optimum treatment for symptomatic limited disease affecting an organ and timing of chemotherapy perioperatively. Emergency surgery for complications of NHL is mainly for palliation and diagnosis. The patient was subsequently linked to an oncology center after a smooth postoperative course.

Conclusion

In a nutshell, we describe the diagnosis of a pediatric cecal obstruction due to B-cell NHL. Surgery is not often used as a treatment because of the efficacy of chemotherapy. However, surgery may be useful in relieving symptomatic, limited disease in an affected organ. Although recurrent abdominal pain is a common symptom for many common pediatric problems, it may be the only presentation of primary GI lymphoma. Thus, in cases of recurrent abdominal pain, pediatricians should also keep the rare causes in mind, especially in children presenting beyond the typical age-group for idiopathic intussusception (3 years); GI lymphoma must be ruled out.

Ethical approval

Ethical approval is deemed unnecessary by the ethical committee, as this is a single case encountered during practice, and it doesn’t involve human or animal experiments. The Institutional Review Board waived ethical approval for this particular study.

Consent

Written informed consent was obtained from the patient’s mother, who is the primary care giver, in their native language, for the publication of non-identifying information including accompanying intraoperative images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Sources of funding

No funding was provided for this case report.

Author contributions

L.D.L.: Primary operating surgeon, study concept and plan, writing the paper and literature review. C.M.: Editing the manuscript, patient management, data collection, and critical review.

Conflicts of interest disclosure

No conflict of interest among the authors.

Research registration unique identifying number (UIN)

Not applicable.

Guarantor

Lohide Daniel Lopura.

Provenance and peer review

Not commissioned; externally peer reviewed.

Data availability statement

The data are deposited in public repository and can be accessed by anyone.