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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
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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
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 TumorDuodenumJejunumIleum% 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 Malignant Benign Symptom % Pain 25 Obstruction 20 Bleeding 10-20 Asymptomatic <50 Symptom % Weight Loss 90-100 Abdominal Pain 80 Obstruction 30 Abdominal Mass 15 Perforation 10 Bleeding 10 Jaundice 2
Small Bowel Tumors Diagnosis • Endoscopy • Upper • Lower • Enteroscopy • Capsule endoscopy • Radiology • Plain films • Contrast Studies • CT • Laparotomy/Laparoscopy
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% node15% 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)