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Dr. Varun

Introduction
• Anywhere outside the lymph node region
▫ Primary lymphoid organs: spleen, thymus,
waldeyer ring
...
Dr. Varun

Introduction
•
•
•
•

GIT – lymphoid elements seen in lamina propria and
submucosa
Secondary GIT involvement is...
Dr. Varun

Introduction
• Primary GI lymphoma – MC extranodal
manifestation of NHL (20% of all cases)

• Association with ...
Dr. Varun

Incidence and pathogenesis
•
•
•
•
•
•

Increasing NHL due to HIV
Extranodal NHL – 1.9 in 100,000
M:F – 3:2
<1%...
Dr. Varun

Incidence and pathogenesis
• Risk factors
▫ H. pylori infection, celiac disease, IBD,
immunosuppression after s...
Dr. Varun

Incidence and pathogenesis
• Immunoproliferative small intestine disease special form of MALT lymphoma is suspe...
Dr. Varun
Dr. Varun

Incidence and pathogenesis
• HIV related cases have a B cell type lymphoma
with unusual morphological features ...
Dr. Varun

Pathologic features
• Most are B cell type, though large B cell and
MALT are also reported – stomach
• T-cell: ...
Dr. Varun

Staging
• Consensus conference in Luguano 1993
▫ Stage I – tumor confined to GIT, single primary
site and multi...
Dr. Varun

Staging
▫ Stage III – penetration through serosa to involve
adjacent organs or tissues
▫ Stage IV – disseminate...
Dr. Varun

Radiologic appearances
Esophagus
• Cervical/mediastinal node invasion or
• Contiguous spread from gastric lymph...
Dr. Varun
Dr. Varun
Dr. Varun

Radiologic appearances
Stomach
• 1-5% of gastric malignancies
• MC type of extranodal lymphoma; 50-70% of all
G...
Dr. Varun

• Barium
▫ DCBM – ulcerative, polypoid or infiltrative lesions
▫ Multiple polypoid tumors with central ulcerati...
Dr. Varun

• CT
▫ Low grade – less wall thickening, less abdominal
lymph nodes. Negative CT favors it
Dr. Varun
Dr. Varun
Dr. Varun

Small bowel
• MC malignancy of small bowel. Increasing incidence
due to HIV
• 20-30% of all GI lymphomas
• B ce...
Dr. Varun
Dr. Varun

• May ulcerate/perforate forming a confined
sterile abscess
• Aneurysmal dilatation of the lumen may be seen
du...
Dr. Varun
Dr. Varun
Dr. Varun
Dr. Varun
Dr. Varun
Dr. Varun
Dr. Varun
Dr. Varun
Dr. Varun
Dr. Varun
Dr. Varun

Malabsorption and intestinal recurrence are more in enteropathy
associated T – cell lymphoma
Dr. Varun
Dr. Varun

Large bowel
• 0.4% of all colon tumors
• 6-12% of GI lymphomas
• Cecum and rectum
• MALT, mantle cell and T cel...
Dr. Varun

• DCBE
▫
▫
▫
▫
▫
▫
▫

Polypoid massed near IC valve
Circumferential infiltration
Cavitary mass excavating into ...
Dr. Varun
Dr. Varun
Dr. Varun

• Differentiating from adenocarcinoma
▫ Extension into terminal ileum
▫ Well defined margins with preservation ...
Dr. Varun
Dr. Varun

• Rectal lymphoma
▫ Indistinguishable from rectal adenocarcinoma

• Appendix
▫
▫
▫
▫

Very rare
In children, Bu...
Dr. Varun
Dr. Varun
Dr. Varun
Gastrointestinal lymphoma
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Gastrointestinal lymphoma

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Compiled from radiographics, a review of features of GI lymphoma.

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Gastrointestinal lymphoma

  1. 1. 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
  2. 2. Dr. Varun Introduction • • • • 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 1. 2. 3. 4. – 5. 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
  3. 3. Dr. Varun Introduction • Primary GI lymphoma – MC extranodal manifestation of NHL (20% of all cases) • Association with HL – extremely rare
  4. 4. Dr. Varun Incidence and pathogenesis • • • • • • 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
  5. 5. 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
  6. 6. 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
  7. 7. Dr. Varun
  8. 8. Dr. Varun Incidence and pathogenesis • HIV related cases have a B cell type lymphoma with unusual morphological features high grade and poor prognosis
  9. 9. 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)
  10. 10. 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
  11. 11. 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
  12. 12. 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
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  15. 15. 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%
  16. 16. 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
  17. 17. Dr. Varun • CT ▫ Low grade – less wall thickening, less abdominal lymph nodes. Negative CT favors it
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  20. 20. 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.
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  22. 22. 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
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  33. 33. Dr. Varun Malabsorption and intestinal recurrence are more in enteropathy associated T – cell lymphoma
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  35. 35. 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
  36. 36. Dr. Varun • DCBE ▫ ▫ ▫ ▫ ▫ ▫ ▫ Polypoid massed near IC valve Circumferential infiltration Cavitary mass excavating into mesentery Endoexoenteric tumors Mucosal nodularity Fold thickening Focal strictures, aneurysmal dilatation
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  39. 39. 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
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  41. 41. Dr. Varun • Rectal lymphoma ▫ Indistinguishable from rectal adenocarcinoma • Appendix ▫ ▫ ▫ ▫ Very rare In children, Burkitts lymphoma Adults – large cell and MALT Maintained vermiform appearance with diffuse mural soft tissue thickening
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