Small Bowel Tumors Keith D. Lillemoe M.D. Dept. of Surgery Indiana University School of Medicine

Small Bowel Tumors Epidemiology Exceedingly rare - < 5500 new cases,1200 deaths/year Explanations lack of bacteria rapid transit role of pancreatic and mucosal enzymes secretory Ig A / intramural lymphoid tissue

Predisposing Conditions Small Bowel Tumors Predisposing Conditions Adenocarcinoma HNPCC Familial Adenomatous Polyposis Crohn’s Disease Lymphoma Celiac Disease Crohn’s Disease Immunologic Dysfunction

Small Bowel Tumors Pathology - Benign Adenomas (20 – 30%) simple tubular adenomas villous adenomas Brunner’s gland adenomas Leiomyomas (30 – 40%) Lipomas (15 – 20%) Hemangiomas (<10%) Hamartomas (<5%)

Small Bowel Tumors Pathology - Malignant Distribution (%) Type of Tumor Duodenum Jejunum Ileum % of Total Adenocarcinoma 35-45 30-40 20-25 40-50 Carcinoid Tumor 10-20 30-40 40-50 20-30 Lymphoma 10-15 5-10 75-85 20-25 Gastrointestinal Stromal Tumors 1 40-50 50-60 10-15

Small Bowel Tumors Clinical Presentation Benign Malignant Symptom % Pain 25 Obstruction 20 Bleeding 10-20 Asymptomatic <50 Malignant Symptom % Weight Loss 90-100 Abdominal Pain 80 Obstruction 30 Abdominal Mass 15 Perforation 10 Bleeding 10 Jaundice 2

Small Bowel Tumors Diagnosis Radiology Endoscopy Plain films Upper Contrast Studies CT Laparotomy/Laparoscopy Endoscopy Upper Lower Enteroscopy Capsule endoscopy

Small Bowel Tumors Management – Benign Neoplasms Adenomas: Duodenum : Endoscopic polypectomy Transduodenal excision Duodenectomy Jejunum/Ileum : Local excision

Small Bowel Tumors Management – Benign Tumors Hamartomas – Limited resection of responsible lesion (s) Hemangiomas – Resection Electrocautery

Small Bowel Tumors Management - Adenocarcinoma Duodenum – Pancreaticoduodenectomy 5 year – survival : 50-60% Jejunum-ileum – En bloc resection of bowel/mesentery 5 year survival : overall 15-30% node  15% node Θ 50-70% ? role for adjuvant therapy

Small Bowel Tumors Gastrointestinal Stomal Tumors formerly leiomyoma / leiomyosarcoma arise from mesenchymal tissue interstital (cell of Cajal) grow extrinsically, often to large size present with palpable mass, hemorrhage associated with mutation of C-kit

Small Bowel Tumors Management – Gastrointestinal Stromal Tumors Limited surgical resection Imatnib Mesylate (gleevac) 5 year survival 60-80%

Small Bowel Tumors Lymphomas vague symptoms – fatigue, malaise, weight loss, pain perforation, obstruction – 25% palpable mass – 33%

Small Bowel Tumors Lympoma – Pathology/Staging Non-Hodgkin’s, B-cell Usually intermediate/high grade with large cell features Ann Arbor classification IE – Tumor continued to SI without lymph nodes IIE – Regional lymph node involvement IIIE – Nonresectable lymph nodes IVE – Spread to nonlymphatic organs

Small Bowel Tumors Management - Lymphoma I-E / II-E – Limited resection, ?CTX 5-year survival : 60% III-E / IV-E – Limited resection + CTX / Radiation 5-year survival : rare

Small Bowel Tumors Carcinoid Tumors arise from enterochromatin cells often present late with nodal/hepatic metastasis obstruction due to desmoplastic reaction of mesentery carcinoid syndrome

Small Bowel Tumors Management – Carcinoid Tumors segmental resection with en bloc mesenteric resection aggressive treatment of metastatic disease treatment of carcinoid syndrome : octreotide 5 year survival : localized 100% regional 65% distant 25-35%

Small Bowel Tumors Metastatic Neoplasms direct extension, carcinomatosis Hematogenous metastasis (melanoma, hypernephroma, breast, lung)