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Small Bowel Lymphoma

Small Bowel Lymphoma. September 15, 2011 UB Department of Surgery Grand Rounds. Craig Collins MD. Outline. Anatomy/Physiology Pathogenesis Background Incidence Risk Factors Diagnosis Management Prognosis Take Home Points Future. Anatomy/Physiology. Anatomy/Physiology.

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Small Bowel Lymphoma

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  1. Small Bowel Lymphoma September 15, 2011 UB Department of Surgery Grand Rounds Craig Collins MD

  2. Outline • Anatomy/Physiology • Pathogenesis • Background • Incidence • Risk Factors • Diagnosis • Management • Prognosis • Take Home Points • Future

  3. Anatomy/Physiology

  4. Anatomy/Physiology • Small Bowel ~3m • Duodenum ~20-30cm • Jejunum ~100-110cm • Ileum ~ 150cm • Blood supply based upon celiac axis and superior mesenteric artery (SMA). Venous return via superior mesenteric vein (SMV).

  5. Anatomy/Physiology • Layers of Small Bowel • Mucosa • Submucosa • Muscularis • Serosa

  6. Anatomy/Physiology • Spiral folds of mucosa and submucosa (plicae circularis) are more prominent proximally. • Jejunum is larger in diameter, is generally thicker, and has more prominent mucosal folds. • Peyer’s Patches (lymphoid tissue) found in submucosal layer and become more prominent distally in the Ileum.

  7. Anatomy/Physiology • Primary functions are digestion, absorption, and motility. • Endocrine function (CCK, secretin, other peptides) • Immune function via secretion of IgA from Peyer's patches.

  8. Anatomy/Physiology • Small bowel accounts for 75% of GI tract length and 90% of mucosal surface. • Accounts for 3-6% of GI tract tumors and 1-3% of all malignant GI tumors. • 2/3 of symptomatic GI tract tumors are malignant. • Majority of benign lesions are asymptomatic and are discovered at autopsy.

  9. Pathogenesis • Several factors thought to account for rarity of small bowel neoplasms. • Rapid transit time • Liquid contents • Neutral pH and high levels of benzopyrene hydroxylase • Bacterial flora/load • Increased lymphoid tissue and IgA- Immunoprotective role.

  10. Background • Hodgkin’s Lymphoma first described in 1832 by Dr. Thomas Hodgkin. • Orderly spread of disease from one lymph node group to another, pathologically characterized by presence of Reed Sternberg cells. • One of the first cancers to be cured by XRT and subsequently by combination chemotherapy. ~93% cure rate.

  11. Background • Classified into 4 pathologic subtypes based upon Reed-Sternberg cell morphology. • Nodular Sclerosing • Mixed Cellularity • Lymphocyte rich • Lymphocyte depleted

  12. Background • Non-Hodgkin’s Lymphoma • B-Cell* • diffuse large cell* • Small cell (Mantle cell and follicular) • mixed small and large cell • MALT Lymphoma • Burkitt’s • EATL (Enteropathy Associated T-Cell Lymphoma) • Immunoproliferative small intestinal disease (IPSID) * Most common (2/3), 70-80% High grade, 20-30% low grade

  13. Background • Lymphomas can affect any lymph node station and nearly every organ. • Primary GI (extra-nodal) lymphomas represent ~30% of all lymphomas. • Gastric- 75% • Small Bowel (including duodenum)- 9% • Ileo-cecal region- 7% • >1 GI site- 6% • Rectum- 2% • Colon- 1%

  14. Background • All sub-types of nodal lymphomas may also arise in GI tract but NHL are most common. • Ulcerating or infiltrating.

  15. Incidence • Colon cancer 50-60x more common than small bowel cancers. • Primary small bowel cancer: • Adenocarcinoma (40%) > NET (30%) > Lymphoma (20%) > Sarcoma/other (~10%) • ~ 50% of small bowel neoplasms are secondary (metastasis)*. • Colon, stomach, pancreas, melanoma, breast, & lung. *Direct extension, intraperitoneal seeding, hematogenous/lymphatic spread

  16. Incidence • 1.6 cases/million/year • Steep rise in the 1980’s in correlation to AIDS • Bimodal age distribution, 20’s-30’s and >50.

  17. Risk factors • Celiac disease • IBD • RA • Chronic infection, poor sanitation • HIV/AIDS with low CD4 count • Post transplant Inflammation Immunosuppression

  18. Data taken as a whole do not support the hypothesis that IBD alone is a risk factor for lymphoma. • Suggests IBD pts. Treated with AZA and 6-MP are at greater risk of lymphoma than general population. • ? Regarding risk of severe or prolonged IBD compared with less severe disease.

  19. Small but real increase in the risk of lymphoma in IBD patients receiving anti-TNF-α therapy, but risk yet to be clearly quantified.

  20. Undoubtedly an increased risk of malignancy in celiac disease with regard to small bowel lymphoma and adenocarcinoma. • Risk of NHL may be increased 3-9 fold, but the overall risk to celiac population is < 1 %. • Risk diminishes over time if compliant with gluten free diet and is equal to general population 15 years after diagnosis.

  21. Prospective cohort study of 637 pts with treated celiac disease in the UK from 1978-2001. Malignancy rates recorded. • Risk to general population was estimated from cancer registries. • Median follow up was 6.6 years (2.2-14.5 yrs). • Cancer diagnosis within 2 years of celiac disease excluded.

  22. No increase in overall malignancy rate in diagnosed celiac disease in the post-diagnosis period when compared with the general population. • 5x greater rate of NHL and 40x greater rate of small bowel lymphoma compared with general population in the post-diagnosis period.

  23. Clinical Presentation • Abdominal pain, weight loss, nausea, emesis, GIB, chronic anemia. • Median duration of symptoms- 6 months. • Normal PE in 24%, abdominal mass in 46%. • Often present with perforation, bleeding, or obstruction necessitating emergent surgery (~25-50%)

  24. Diagnosis • Criteria: • Lack of peripheral or mediastinallymphadenopathy. • Normal WBC count and differential on peripheral smear. • Tumor involvement primarily in GI tract. • No involvement of liver or spleen. • No history of previously treated nodal lymphoma.

  25. Diagnosis- Modalities • SBFT/Enteroclysis • Barium Enema • EGD/Colonoscopy • Push Enteroscopy, Double Balloon Endoscopy • Capsule endoscopy • CT • MRI • PET/ PET CT • Exploratory Laparotomy/Laparoscopy* * Diagnosis in ~50% of patients

  26. Diagnosis • Wide variety of radiologic manifestations- ulceration, stricture, polypoid mass, mechanical obstruction, intussusception, fistulas, aneurysmal bowel dilatation, thick mucosal folds, separation of adjacent loops, mesenteric adenopathy and mesenteric thickening. • Clinical and radiographic challenge due to vague symptoms, rarity of disease, and relative inaccessibility of the small bowel.

  27. Diagnosis/Work up- NCCN • Physical Exam with performance status • CBC with differential, platelets • LDH • Hep B testing if Rituximab contemplated • CT Chest/Abdomen/Pelvis for staging • Pregnancy testing in women of childbearing age if chemo planned. • Select cases- Bone marrow biopsy with aspirate for multifocal disease. PET-CT, MRI, Hep C testing

  28. Diagnosis- CT Enteroclysis

  29. Diagnosis- CT Enteroclysis

  30. Diagnosis- CT

  31. Diagnosis- CT

  32. Diagnosis- CT

  33. Diagnosis- CT

  34. Diagnosis- CT

  35. Diagnosis- MRI

  36. Diagnosis- Double Balloon Endoscopy • Endoscope introduced, balloon inflated, scope advanced. Performed via oral and anal routes. Depth range from 1-8.8m. • Allows for visualization and tissue diagnosis.

  37. Retrospective review of 29 pts with GI lymphoma, further examined by double balloon endoscopy. • Sought to determine prevalence of additional GI lymphomas.

  38. 50% of their pts had additional GI lymphomas. • Recommends complete evaluation of the small bowel in any patient with GI lymphoma.

  39. Double Balloon Endoscopy

  40. Diagnosis- Capsule Endoscopy

  41. Low yield but superior test for small bowel • Diagnostic impact in 57%, exclusive therapeutic decisions in 12% • Overall diagnostic yield for obscure GIB 58-80% • 6% were SB tumors

  42. PET-CT

  43. Staging- Ann Arbor • Stage I- limited to intestine • Stage II- Extension into regional nodes or infiltration of surrounding organ • Stage III- Involvement of lymph nodes on both sides of diaphragm • Stage IV- Involvement of distant organs or extra abdominal lymph nodes

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