Gastrointestinal lymphoma

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Review of gastrointestinal lymphoma

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Introduction :

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 Dr. Varun

Introduction:

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 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 Dr. Varun

Introduction:

Introduction Primary GI lymphoma – MC extranodal manifestation of NHL (20% of all cases) Association with HL – extremely rare Dr. Varun

Incidence and pathogenesis:

Incidence and pathogenesis Increasing NHL due to HIV Extranodal NHL – 1.9 in 100,000 M:F – 3:2 <1% of GIT tumors 6 th decade MC GI tumor in children Dr. Varun

Incidence and pathogenesis:

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 Dr. Varun

Incidence and pathogenesis:

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 Dr. Varun

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Dr. Varun

Incidence and pathogenesis:

Incidence and pathogenesis HIV related cases have a B cell type lymphoma with unusual morphological features high grade and poor prognosis Dr. Varun

Pathologic features :

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) Dr. Varun

Staging :

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 Dr. Varun

Staging :

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 Dr. Varun

Radiologic appearances:

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 Dr. Varun

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Radiologic appearances:

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% Dr. Varun

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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 Dr. Varun

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

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Dr. Varun

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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. Dr. Varun

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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 Dr. Varun

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Dr. Varun

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Dr. Varun

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Dr. Varun

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Dr. Varun

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Dr. Varun

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Dr. Varun

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Dr. Varun

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Dr. Varun

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Dr. Varun

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Dr. Varun

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

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Dr. Varun

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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 Dr. Varun

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DCBE Polypoid massed near IC valve Circumferential infiltration Cavitary mass excavating into mesentery Endoexoenteric tumors Mucosal nodularity Fold thickening Focal strictures, aneurysmal dilatation Dr. Varun

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Dr. Varun

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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 Dr. Varun

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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 Dr. Varun

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Dr. Varun

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Dr. Varun

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Dr. Varun

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