JPGN reports · 4 authors, 1 centre
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This case report describes a 14-year-old boy with no prior medical history who presented with acute esophageal food impaction and progressive dysphagia over four weeks. Imaging and biopsy revealed esophageal stricture caused by non-Hodgkin's lymphoma, an extremely rare presentation in children. The case highlights that atypical features—such as a long stricture without inflammation or eosinophilic esophagitis risk factors—should prompt further imaging to identify underlying malignancy.
**Background:** Primary involvement of the esophagus is a rare manifestation of lymphoma, with few published cases in adults and even fewer in children. Less than 1% of gastrointestinal lymphoma cases involve the esophagus, and only 0.2% of patients present with primary esophageal presentation. Pediatric incidence rates are unknown. Presenting symptoms typically include dysphagia, abdominal pain, and weight loss. Reported complications of associated strictures include perforation and tracheoesophageal fistula.
**Methods:** This is a single case report of a 14-year-old previously healthy male who presented with acute onset of esophageal food impaction. The patient had progressively worsening dysphagia for 4 weeks, initially with solids and then with liquids. He denied fevers, weight loss, and had no history of allergies, eczema, or asthma. An esophagram at an outside hospital revealed a 7-cm narrowing. Due to worsening symptoms, he presented to the emergency department. Upper endoscopy revealed a proximal esophageal stricture that could not be traversed by a standard-sized endoscope. A neonatal endoscope successfully passed the measured 7-cm stricture. Endoscopic evaluation of mucosa proximal and distal to the stricture was unremarkable, and biopsies were normal. Because of the short duration of symptoms with a long stricture, lack of endoscopically visualized inflammation, and normal biopsies, a chest radiograph was performed and revealed possible mediastinal widening. Magnetic resonance imaging of the chest confirmed circumferential esophageal wall thickening of the upper thoracic esophagus involving up to 8 cm in length and enlarged surrounding lymph nodes suspicious for lymphoma versus primary esophageal malignancy. Computed tomography of the chest confirmed these findings. Endoscopic ultrasound with fine needle aspiration of one of the lymph nodes was performed and an esophageal stent was placed.
**Key Results:** Biopsies revealed atypical B cells consistent with malignant lymphoma. The patient was ultimately diagnosed with non-Hodgkin's lymphoma. The patient had no classic symptoms of lymphoma; however, due to the lack of risk factors associated with eosinophilic esophagitis (EOE), lack of endoscopic/histologic evidence of EOE, and short duration of symptoms with a lengthy stricture, another more insidious etiology was suspected, prompting further imaging studies.
**Clinical Implications:** This case demonstrates that primary esophageal lymphoma, though extremely rare, should be considered in pediatric patients presenting with esophageal stricture when typical causes such as eosinophilic esophagitis are absent. Key red flags include a long stricture (7–8 cm), short duration of symptoms, absence of endoscopic inflammation, and normal mucosal biopsies. Careful history-taking and a low threshold for cross-sectional imaging (chest radiograph, MRI, CT) can help distinguish unusual diagnoses and expedite necessary treatment. The patient is doing well following initiation of chemotherapy.