Small Bowel Tumors Keith D Lillemoe M D
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 Lipomas Hemangiomas Hamartomas (30 – 40%) (15 – 20%) (<10%) (<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 1 40 -50 50 -60 10 -15 Gastrointestinal Stromal Tumors
Small Bowel Tumors Clinical Presentation Malignant Benign Symptom Pain Obstruction Bleeding Asymptomatic % 25 20 10 -20 <50 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 • Lower • CT • Enteroscopy • Laparotomy/Laparoscopy • Capsule endoscopy
Small Bowel Tumors Management – Benign Neoplasms Adenomas: Duodenum : Jejunum/Ileum : Endoscopic polypectomy Transduodenal excision Duodenectomy 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 regional distant 100% 65% 25 -35%
Small Bowel Tumors Metastatic Neoplasms • direct extension, carcinomatosis • Hematogenous metastasis (melanoma, hypernephroma, breast, lung)
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