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