Extranodal Marginal Zone Lymphoma of the Stomach

Extranodal Marginal Zone Lymphoma, also known as mucosa-associated lymphoid tissue (MALT) lymphoma, frequently occurs within the stomach. It accounts for 30-50% of all gastrointestinal lymphomas.

For an excellent review of the histopathology, diagnosis, and management the European Gastro-Intestinal Lymphoma Study Group (EGILS) provides a consensus report released in 2011 ([[bibcite 1]]).

Diagnosis and Staging

Patients present with non-specific signs. These may include abdominal pain, dyspepsia, gastrointestinal bleeding or weight loss. Patients are diagnosed by gastroscopy for investigations of their symptoms.

History

History should focus on:

  • Presenting symptoms and their duration
    • Presence or absence of B symptoms
  • Treatment received so far (usually helicobacter pylori eradication)
  • Past history of malignancy or radiotherapy
  • Current medications and allergies (particularly to antibiotics)
  • Social history and ability to attend radiotherapy

Examination

Examination should evaluate:

  • Lymph node groups (inner/outer cervical, axillary, inguinal)
  • Hepatomegaly or splenomegaly
  • Evidence of systemic disease

Diagnostic Investigations

Most patients would have had gastroscopy and biopsy by presentation to the radiation oncologist. If not, then patients should undergo a gastroscopy with biopsy of suspicious lesions as well as of normal-appearing mucosa.

Special Histopathological Tests

The presence of a translocation of chromosomes 11 and 18 suggests more aggressive disease. Helicobacter pylori eradication is less successful when this mutation is present.

Bloods

General lymphoma bloods should be ordered (FBE, UEC, CMP, LDH, LFT).

CT

CT of the chest, abdomen and pelvis is mandatory for complete staging.

Staging

The Ann-Arbor system is used.

1. Ruskone-Fourmestraux, A. et al. EGILS consensus report. Gastric extranodal marginal zone B-cell lymphoma of MALT. Gut 60, 747–758 (2011).