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Gastrointestinal lymphoma
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2. Dr. Varun
Introduction
• Anywhere outside the lymph node region
▫ Primary lymphoid organs: spleen, thymus,
waldeyer ring
▫ Organs or tissue devoid of lymphoid tissue: brain,
soft tissue
▫ Organs with significant lymphoid tissue
component: GIT
3. Dr. Varun
Introduction
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GIT – lymphoid elements seen in lamina propria and
submucosa
Secondary GIT involvement is common
Primary lymphomas involve only one site
5 criteria put forth by Dawson et al to diagnose
primary GI lymphoma
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No palpable superficial lymph nodes
Normal CXR
WBC count (TLC and DLC) are normal
At laparotomy, alimentary tract is involved with lymph node
involvement, if any, confined to drainage of involved gut
No involvement of liver/spleen
* advanced stages mimic secondary GI lymphoma
4. Dr. Varun
Introduction
• Primary GI lymphoma – MC extranodal
manifestation of NHL (20% of all cases)
• Association with HL – extremely rare
5. Dr. Varun
Incidence and pathogenesis
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Increasing NHL due to HIV
Extranodal NHL – 1.9 in 100,000
M:F – 3:2
<1% of GIT tumors
6th decade
MC GI tumor in children
6. Dr. Varun
Incidence and pathogenesis
• Risk factors
▫ H. pylori infection, celiac disease, IBD,
immunosuppression after solid organ
transplantation
• No lymphoid tissue normally in gastric mucosa.
H.pylori infection develops lymphoid tissue in
lamina propria defining the low grade tumor as
MALT (mucosa associated lymphoid tissue)
primary lymphoma
7. Dr. Varun
Incidence and pathogenesis
• Immunoproliferative small intestine disease special form of MALT lymphoma is suspected to
have an infectious etiology
• Celiac disease – risk factor for small bowel
adenocarcinomas, esophageal cancer, melanoma
and NHL
9. Dr. Varun
Incidence and pathogenesis
• HIV related cases have a B cell type lymphoma
with unusual morphological features high grade
and poor prognosis
10. Dr. Varun
Pathologic features
• Most are B cell type, though large B cell and
MALT are also reported – stomach
• T-cell: enteropathy in small intestine
• Burkitt, mantle cell and follicular – less common
• Order of incidence – stomach > small intestine*
> large intestine > esophagus
• (* - increasing with rise of HIV)
11. Dr. Varun
Staging
• Consensus conference in Luguano 1993
▫ Stage I – tumor confined to GIT, single primary
site and multiple non contiguous lesions
▫ Stage II – tumor extends into abdominal cavity
from primary GI site
II 1 – local nodal involvement
II 2 – distal nodal involvement
12. Dr. Varun
Staging
▫ Stage III – penetration through serosa to involve
adjacent organs or tissues
▫ Stage IV – disseminated extra nodal involvement
or GI lesion with supradiaphragmatic nodal
involvement
Most patients present with stage II
13. Dr. Varun
Radiologic appearances
Esophagus
• Cervical/mediastinal node invasion or
• Contiguous spread from gastric lymphoma
• <1% of primary GI lymphomas
• Predominantly B-cell, few MALT reported
• Predominantly submucosal infiltration; may manifest as
polypoidal mass, ulceration or nodularity.
• Subtle mucosal submucosal abnormalities better
delineated by barium, CT to assess extent. Perforation
and fistulization may be demonstrated
16. Dr. Varun
Radiologic appearances
Stomach
• 1-5% of gastric malignancies
• MC type of extranodal lymphoma; 50-70% of all
GI lymphomas
• H.pylori gastritis – low grade lymphoma
• Originates as low grade, then transforms into
intermediate/high grade
• Low grade 5yr survival -75-91%
• High grade <50%
17. Dr. Varun
• Barium
▫ DCBM – ulcerative, polypoid or infiltrative lesions
▫ Multiple polypoid tumors with central ulceration
(bull’s eye appearance), giant cavitating lesions,
or extensive infiltration with gastric fold
thickening
▫ Low grade – much varied appearance
18. Dr. Varun
• CT
▫ Low grade – less wall thickening, less abdominal
lymph nodes. Negative CT favors it
21. Dr. Varun
Small bowel
• MC malignancy of small bowel. Increasing incidence
due to HIV
• 20-30% of all GI lymphomas
• B cell, T cell, Burkitt, MALT and rarely Hodgkin’s
• Distal ileum – MC site due to abundance of
lymphoid tissue
• Circumferential bulky mass in intestinal wall,
associated with extension into small bowel
mesentery & regional nodes.
23. Dr. Varun
• May ulcerate/perforate forming a confined
sterile abscess
• Aneurysmal dilatation of the lumen may be seen
due to replacement of muscularis propria &
destruction of autonomic nerve plexus by
lymphoma
• Obstruction is uncommon in small bowel
• Peritoneal lymphomatosis – rare, if present
indistinguishable from peritoneal
carcinomatosis, TB
36. Dr. Varun
Large bowel
• 0.4% of all colon tumors
• 6-12% of GI lymphomas
• Cecum and rectum
• MALT, mantle cell and T cell
• Mantle cell – multiple polyps – lymphomatous
polyposis
37. Dr. Varun
• DCBE
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Polypoid massed near IC valve
Circumferential infiltration
Cavitary mass excavating into mesentery
Endoexoenteric tumors
Mucosal nodularity
Fold thickening
Focal strictures, aneurysmal dilatation
40. Dr. Varun
• Differentiating from adenocarcinoma
▫ Extension into terminal ileum
▫ Well defined margins with preservation of fat
planes
▫ No invasion into adjacent structures
▫ Perforation with no desmoplastic reaction
▫ Severe luminal narrowing with no obstruction
▫ D/D Kaposi