Section: UGI Sub-section: Gastric Curriculum: Curriculum, page 96
Lymphoma overview
Incidence
- Stomach most common site of lymphomas (50-75%) of GI tract
- Stomach 75%
- Small Bowel 9%
- Ileo-caecal 7%
- 6% have multiple gastrointestinal sites
- Also, the most common extra-nodal primary site for Non-Hodgkin’s Lymphoma
- Accounts for 5-10% of gastric malignancies
- Accounts for 2% of all lymphomas
- Age 60-70
- M > F - 2:1
Presentation
- 50% present with anaemia
- Dyspepsia symptoms
- Mostly in antrum
Risk Factors
- H. pylori
- Immunodeficiency
- HIV
- Coeliac disease
- Autoimmune diseases
Pathology
- Diffuse Large B-Cell (55%)
- Extranodal marginal zone lymphoma (MALT) (40%)
- Burkitt’s (3%)
- Related to EBV, aggressive
- Mantle Cell (<1%)
- Follicular (<1%)
- T-cell
- Liked with enteropathy/Coeliacs
- Prognosis not as good as B-Cell
Investigations
- Needs fresh tissue
- Fluorescence-activated cell sorting
- Immunohistochemistry
- Genetic analysis
Staging
- CT C/A/P
- EUS – best staging for depth of invasion and regional node involvement
- Bone Marrow Aspirate
There is the Lugano and Paris staging systems for gastrointestinal lymphomas
Revised staging system for primary nodal lymphomas (Lugano classification)
| Limited | Involvement | Extranodal status |
|---|---|---|
| I | One node or a group of adjacent nodes | Single extranodal lesions without nodal involvement |
| II | Two or more nodal groups on the same side of the diaphragm | Stage I or II by nodal extent with limited contiguous extranodal involvement |
| II bulky* | II as above with “bulky” disease | Not applicable |
| Advance | Involvement | Extranodal status |
| III | Nodes on both sides of the diaphragm; nodes above the diaphragm with spleen involvement | Not applicable |
| IV | Additional noncontiguous extralymphatic involvement | Not applicable |
Diffuse Large B-Cell Lymphoma
- Could represent a progressive change from less aggressive lymphoma
- (CLL, MALT)
- B cells with nucleus size of macrophages
- Can also be associated with H. pylori + EBV
- Appears as a large, ulcerated mass
- Mimics advanced gastric carcinoma
Management
- CHOP or R-CHOP Chemotherapy
- R – Rituximab
- C – Cyclophosphamide
- H – Doxorubicin (Hydroxydaunorubicin)
- O – Vincristine (which used to be called Oncovin)
- P – Prednisolone
- Radiotherapy controversial
- Not good for large tumours
- Prognosis
- 5YS 25%
Extranodal marginal zone lymphoma (MALT)
- Related to chronic inflammation, H. pylori
- Mucosa associated lymphoid tissue
- Arise in organs usually devoid of lymphoid tissue, presumably in response to antigenic stimuli
- Occur in stomach, thyroid, lung, salivary gland
- Arise in organs usually devoid of lymphoid tissue, presumably in response to antigenic stimuli
-
50 years, M=F
- Ill-defined thickening of mucosa with occasional ulcerations
- Composed of neoplastic B cells
- Increased risk with chronic inflammation
- H. pylori (70% remission with treatment)
Management
- H. pylori eradication +/- chemotherapy +/ radiotherapy
- H. pylori eradication can lead to complete regression of low-grade MALT (MALT, B-cell)
- Occasionally radiation or surgical resection
- 5-yr survival for localized disease = 90%
- Associated with synchronous/metachronous adenocarcinoma