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Gastric lymphoma

From Surgopaedia

Classification

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  • Described as primary gastric lymphoma if the stomach is the exclusive or predominant site of disease

Epidemiology

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  • Stomach is most common extra-nodal site of lymphoma
  • Overall accounts for 3% of gastric cancers

Risk factors

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  • Peak incidence 50-60yo
  • Slight male predominance
  • H. pylori infection
  • Rheumatoid arthritis
  • SLE
  • Immunosuppression
  • Coeliac disease

Pathophysiology

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  • Diffuse large B cell lymphoma - DLBCL (45-60%)
    • Generally primary lesions, but can also occur as progression from less aggressive lymphomas such CLL, small lymphocytic lymphoma, follicular lymphoma, or MALT lymphoma
    • Associated with immunodeficiency and H. pylori infection
    • Fairly high-grade
  • Gastric Mucosa-Associated Lymphoid Tissue (MALT) lymphoma (40-50%)
    • Lower grade than DLBCL
    • B cell lineage
    • Usually preceded by H. pylori gastritis (in almost all cases). B cells are chronically activated in stomach, forming a MALToma, which can transform into lymphoma.
    • Characterised by translocations at:
      • T(1;14)
      • T(3;14)
      • T(11;18)
      • T(14;18)
  • Peripheral T cell lymphoma (1-4%)
  • Mantle cell lymphoma (<1%)
  • Follicular lymphoma (<1%)
  • Usually occurs in gastric antrum, but can arise from anywhere

Presentation

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  • Vague symptoms
    • Epigastric pain
    • Early satiety
    • Fatigue
    • B symptoms - only about 10% of patients

Workup

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  • Endoscopy
    • Biopsy is indicated for all patients
    • Test for H. pylori (probably need to also confirm with serologic testing)
    • EUS can be used to determine depth of invasion and evaluate for lymph node involvement
  • Upper airway examination
    • Look for distant disease - Waldeyer tonsillar ring
  • Bone marrow biopsy
  • CT chest/abdo/pelvis
    • Routine staging
  • PET/CT (FDG)
    • Dependent on subtype
    • Usually done for DLBCL staging

Staging

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Treatment

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  • Chemotherapy
    • Most commonly R-CHOP
    • Associated with a 5% rate of gastric perforation and GI bleeding
  • Indications for surgery
    • Symptomatic recurrence after treatment failure
    • Complications (bleeding, gastric outlet obstruction, perforation)
  • MALT
    • Must evaluate for H. pylori infection
    • Early-stage MALT and active H. pylori infection may be treated by eradication alone - successful eradication results in remission for 75%, but need repeat endoscopy in 2 months and 6 monthly endoscopy for 3 years to document regression
    • Predictors of failure from eradication alone:
      • Trans-mural tumour extension
      • Nodal involvement
      • Transformation into a large cell phenotype
      • Nuclear BCL-10 expression
    • MALT and H. pylori negative:
      • Consider radiotherapy if all involved sites can be encompassed in a single field
      • Otherwise chemotherapy
    • Risk factors for poor prognosis:
      • Age >=70
      • Stage IV disease
      • Elevated LDH
      • 5 year survival of 98.7% for no risk factors, 93.1% for one risk factor and 64.3% for two or three risk factors