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
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 lymphoma1. No palpable superficial lymph nodes2. Normal CXR3. WBC count (TLC and DLC) are normal4. At laparotomy, alimentary tract is involved with lymph
node involvement, if any, confined to drainage of involved gut
5. No involvement of liver/spleen– * advanced stages mimic secondary GI
lymphoma
Dr. Varun
Introduction
•Primary GI lymphoma – MC extranodal manifestation of NHL (20% of all cases)
•Association with HL – extremely rare
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
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
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
Dr. Varun
Dr. Varun
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
•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
•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 ▫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 appearancesEsophagus • 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
Dr. Varun
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%
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
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 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
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
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 cell•Mantle cell – multiple polyps –
lymphomatous polyposis
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
Dr. Varun
Dr. Varun
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
Dr. Varun
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
Dr. Varun
Dr. Varun
Dr. Varun