Early, Isolated Duodenal Mucosa-Associated Lymphoid Tissue Lymphoma Presenting without Symptoms or Grossly Apparent Endoscopic Lesions and Diagnosed by Random Duodenal Biopsies

Abstract

Clinical data regarding mucosa-associated lymphoid tissue lymphoma (MALToma) solely involving the duodenum are sparse because of the relative rarity of the disease. A comprehensive literature review revealed only 17 cases reported until 2004, and only a moderate number of cases have been reported since. MALToma can be asymptomatic in its very early stages but frequently produces localized or nonspecific symptoms, including early satiety, abdominal pain, vomiting, and involuntary weight loss in later stages. While gastric MALToma is strongly associated with gastric Helicobactor pylori infection, duodenal MALToma is often unassociated with H. pylori infection. A 74-year-old female presented with only dysphagia (without symptoms referable to a duodenal lesion), without systemic ‘B’ symptoms, and with no evident duodenal lesions at esophagogastroduodenoscopy; however, she was diagnosed with duodenal MALToma by pathologic examination of random duodenal biopsies performed to exclude celiac disease. An important clinical feature of this case is that duodenal MALToma was diagnosed by pathologic analysis of duodenal biopsies despite (1) no endoscopically apparent duodenal lesions; (2) duodenal involvement without gastric involvement; (3) lack of symptoms attributable to duodenal MALToma, and (4) absence of evident H. pylori infection. This work shows that early duodenal MALToma can be difficult to diagnose because of absent symptoms, absence of gastric involvement, absence of endoscopic abnormalities, and absence of H. pylori infection; it may require random duodenal biopsies for diagnosis.

Primary Gastric and Duodenal Mucosa-Associated Lymphoid Tissue Lymphoma With Symptomatic Anemia

“…8 Patients with duodenal MALT lymphoma can present with symptoms ranging from no symptoms early in the course of the disease to abdominal pain, anemia, protein-losing enteropathy, and more serious presentations such as gastric outlet obstruction and acute pancreatitis. 5,[12][13][14][15] The rarity and variation in the reported cases make the management of duodenal MALT lymphoma challenging. A comprehensive analysis of all duodenal MALT lymphoma case reports, amounting to 26 cases, was documented from 1995 to 2008.…”

“… 8 Patients with duodenal MALT lymphoma can present with symptoms ranging from no symptoms early in the course of the disease to abdominal pain, anemia, protein-losing enteropathy, and more serious presentations such as gastric outlet obstruction and acute pancreatitis. 5 , 12 – 15 …”

Clinicopathological characteristics and prognostic factors of small bowel lymphomas: a retrospective single-center study

“…1), consistent with the published literature. [23][24][25] Although we found endoscopic abnormalities in all patients, it is important to note that small bowel lymphomas may also present with normal mucosa 26,27 and may mimic other conditions such as Crohn disease, 28 therefore requiring a high index of suspicion. Endoscopic treatment of complications has also been occasionally described, for example endoscopic ultrasound-guided gastrojejunostomy and choledocoduodenostomy for malignant intestinal and biliary obstruction, 29 endoscopic balloon dilation for duodenal stenosis, 30 or placement of covered self-expanding metal stent for duodenal-ileal fistula healing.…”