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)