Post on 25-Dec-2015
Small Bowel TumorsKeith 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 TumorsPredisposing Conditions
Adenocarcinoma HNPCCFamilial Adenomatous PolyposisCrohn’s Disease
Lymphoma
Celiac DiseaseCrohn’s DiseaseImmunologic 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 TumorsPathology - 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
Symptom %
Pain 25 Obstruction 20 Bleeding 10-20 Asymptomatic <50
Malignant
Symptom %
Weight Loss 90-100Abdominal Pain 80Obstruction 30Abdominal Mass 15Perforation 10Bleeding 10Jaundice 2
Small Bowel TumorsDiagnosis
Radiology
• Plain films
• 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 TumorsManagement – Benign Tumors
Hamartomas – Limited resection of responsible lesion (s)
Hemangiomas – Resection Electrocautery
Small Bowel TumorsManagement - Adenocarcinoma
Duodenum – Pancreaticoduodenectomy5 year – survival : 50-60%
Jejunum-ileum – En bloc resection of bowel/mesentery 5 year survival : overall 15-30% nodenode Θ 50-70%
? role for adjuvant therapy
Small Bowel TumorsGastrointestinal 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 TumorsLymphomas
• vague symptoms – fatigue, malaise, weight loss, pain
• perforation, obstruction – 25%
• palpable mass – 33%
Small Bowel TumorsLympoma – 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 TumorsManagement - Lymphoma
I-E / II-E – Limited resection, ?CTX5-year survival : 60%
III-E / IV-E – Limited resection + CTX / Radiation
5-year survival : rare
Small Bowel TumorsCarcinoid Tumors
• arise from enterochromatin cells
• often present late with nodal/hepatic metastasis
• obstruction due to desmoplastic reaction of mesentery
• carcinoid syndrome
Small Bowel TumorsManagement – 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 TumorsMetastatic Neoplasms
• direct extension, carcinomatosis
• Hematogenous metastasis (melanoma, hypernephroma, breast, lung)