What is the recommended management for a patient with extranodal marginal‑zone (MALT) lymphoma of the gastro‑oesophageal junction presenting with obstructive symptoms?

Management of Extranodal Marginal Zone Lymphoma of the Gastroesophageal Junction Presenting with Obstruction

For MALT lymphoma of the gastroesophageal junction causing obstruction, immediate relief of obstruction takes priority through endoscopic stent placement, followed by definitive lymphoma-directed therapy based on H. pylori status and disease stage. 1

Immediate Management of Obstruction

The acute obstructive symptoms must be addressed first to restore oral intake and reduce nausea/vomiting:

• Endoscopic esophageal stent placement is the preferred initial intervention for gastroesophageal junction obstruction, providing rapid symptom relief while allowing time for lymphoma staging and treatment planning 1
• If endoscopic stenting is unsuccessful or contraindicated, consider venting gastrostomy for gastric decompression 1
• Feeding jejunostomy tubes can be placed if the patient cannot maintain oral intake after stent placement 1

Critical pitfall: Do not proceed directly to surgical gastrojejunostomy or gastrectomy before completing lymphoma staging, as MALT lymphoma is highly responsive to non-surgical therapies and surgery has not shown superior outcomes compared to conservative approaches 1

Comprehensive Staging After Obstruction Relief

Once obstruction is managed, complete the following workup:

• Multiple biopsies from all gastric regions, duodenum, and gastroesophageal junction with histopathologic evaluation by an expert hematopathologist 1
• Immunohistochemistry panel including CD20, CD10, CD5, and cyclin D1 to confirm MALT lymphoma diagnosis 1
• H. pylori testing via histochemistry, urea breath test, stool antigen test, or serology - this is mandatory regardless of obstruction 1, 2
• FISH or PCR for t(11;18) translocation to identify patients unlikely to respond to antibiotics 1, 2
• Endoscopic ultrasound to evaluate gastric wall depth and regional lymph nodes 1
• CT chest/abdomen/pelvis for systemic staging 1
• Bone marrow aspirate and biopsy 1
• Complete blood count, LDH, and β2-microglobulin 1

Definitive Treatment Algorithm

If H. pylori-Positive and Localized Disease (Stage I-II):

• Initiate H. pylori eradication with PPI-based triple therapy (clarithromycin plus amoxicillin or metronidazole) for 10-14 days, even in the presence of obstruction 1, 2, 3
• Confirm eradication with urea breath test at least 6 weeks after therapy and 2 weeks after stopping PPI 1, 2
• If eradication fails, attempt second-line triple or quadruple therapy 1, 2
• Wait at least 12 months after successful eradication before considering alternative therapy, even if histologic lymphoma persists, provided clinical and endoscopic remission is achieved 1, 2

Important consideration: The stent may need to remain in place during the observation period if obstruction recurs, though many patients experience tumor regression sufficient to restore luminal patency 1

If H. pylori-Negative, Antibiotic-Refractory, or t(11;18)-Positive Localized Disease:

• Involved-field radiotherapy is the optimal choice: 24-30 Gy delivered over 3-4 weeks to the stomach and perigastric nodes 2, 4
• This achieves excellent disease control with minimal morbidity and preserves gastric function 4
• Radiation can be delivered with the esophageal stent in place if needed 1

Critical warning: Patients with t(11;18) translocation will not respond to antibiotics alone and are likely resistant to alkylating agents as monotherapy 2, 4, 3

If Systemic Disease (Stage IV) or Symptomatic Disseminated Disease:

• Rituximab-based chemoimmunotherapy is first-line treatment for symptomatic stage IV disease 2, 3
• Rituximab plus chlorambucil has the highest level of evidence from randomized trials and is well-tolerated 2
• Alternative regimens include rituximab plus bendamustine, or oral alkylating agents (cyclophosphamide or chlorambucil) with or without rituximab 2, 3
• Purine nucleoside analogues (fludarabine, cladribine) are options but carry higher risk of secondary myelodysplasia 2

Do not use aggressive anthracycline-containing regimens (R-CHOP) unless there is histologic transformation to diffuse large B-cell lymphoma 2

If Asymptomatic Stage IV Disease:

• Watchful waiting is appropriate even with disseminated disease 2
• Still eradicate H. pylori if present, as this can contribute to disease control 2
• Monitor with endoscopy and imaging every 6 months 2

Role of External Beam Radiation for Obstruction

• Radiation therapy can be used as palliative treatment for obstruction in addition to its role as definitive lymphoma therapy 1
• This dual benefit makes radiation particularly attractive for obstructing gastroesophageal junction MALT lymphoma 1, 4

What NOT to Do

• Never perform gastrectomy or gastrojejunostomy as initial treatment - surgery has not shown superior results and impairs quality of life 1, 2, 4
• Do not skip H. pylori testing even in obstructing disease - eradication therapy may still be curative 2, 3
• Do not use R-CHOP routinely - reserve for transformation or aggressive behavior 2
• Do not delay obstruction relief while awaiting biopsy results - stent first, then stage 1
• Do not assume obstruction requires immediate surgery - most cases can be managed endoscopically 1

Follow-Up After Treatment

• Endoscopy with multiple biopsies at 2-3 months to document H. pylori eradication 2, 3
• Repeat endoscopy with biopsies twice yearly for 2 years to monitor histologic regression 2, 3
• Annual blood counts and appropriate imaging long-term 2
• Persistent monoclonal B cells after histologic regression warrant continued surveillance, not immediate treatment 1, 2