Small Intestine Tumors
Robert R. Cima, MD, MA
Associate Professor of SurgeryConsultant, Colon and Rectal Surgery
Vice-Chair, Quality and Safety, Department of Surgery
Rochester, MN
Small Intestine Tumors
Robert R. Cima, MD, MA
Associate Professor of SurgeryConsultant, Colon and Rectal Surgery
Vice-Chair, Quality and Safety, Department of Surgery
Rochester, MN
Disclosure
• No financial or commercial relationships
Epidemiology
• Rare and diverse group of tumors• 3,104,173 cancers in the SEER database (1973-2004)
• 20.4% are gastrointestinal (small intestine and colo-rectal malignancies)• ~ 90% are large intestine adeno-carcinomas
• Small intestine (SI) accounts for 80% of intestinal length and 90% of absorptive area• SI malignancies represent 3.1% of intestinal malignancies• Appendiceal cancers represent 0.7%
Epidemiology
• Malignant SI neoplasms• NETs (neuroendocrinetumors) 36.5%• Adenocarcinomas 30.9%• Lymphomas 18.7%• Sarcomas/GISTs 10.0%• Non-specified 3.9%
• Metastatic disease• Melanoma• Breast • Lung Gustafsson et al. 2008
Epidemiology
• Colon malignancies • NETs 0.6%• Adenocarcinomas 93%• Lymphomas 0.4%• Sarcomas/GISTs 0.1%• Non-specified 5.9%
Gustafsson et al. 2008
Epidemiology
• Incidence of SI tumors has been increasing over the last three decades
Gustafsson et al. 2008
Presentation
• Symptoms are vague and non-specific• Abdominal pain (often chronic)• Nausea / Vomiting• Bloating• Diarrhea / Constipation
• Delay in diagnosis often leads to locally advanced disease
• Results in uniformly poor outcomes
Neuroendocrine Tumors (NETs)
• Cells of the diffuse neuroendocrinecell system (DNES)
• Entrochromaffin, enterochromaffin- like, gastrin, etc
• Present in GI, bronchopulmonary, and urogenital system
• Originally termed “Carcinoid” in 1907• Not a uniform cell type tumor but
rather each tumor is comprised of an individual specific cell type of the DNES• Subtypes each exhibit distinct disparate
clinical and biological behavior
NETs
• SI-NETs represents 24.3% of all NETs
• Represents a 4.4-fold increased incidence since 1973
• Majority of SI-NETs (60%) are diagnosed when the disease is no longer localized or metastatic to the liver
• Diagnosis is delayed on average 5 years
• 33% of SI-NETs are multi-centric • 18% of patients present with
“carcinoid” syndrome
Carcinoid Syndrome
• Protean symptomatology• Episodic skin flushing, diarrhea,
bronchoconstriction, sweating, abdominal cramping
• Symptoms related to over-production of vaso- active compounds• Symptom control with long acting
somatostatin (SST) analogs, no impact on survival
• 50% of individuals present with cardiac valve disease related to tumor by-products
NETs
• Surgical resection is the primary treatment
• Survival has steadily increased over the last three decades
• Overall 5 year survival for local disease is 71.8%
• 5 yrs survival with hepatic disease 40% Gustafsson et al. 2008
SI-Adenocarcinoma
• 30.9% of all SI tumors• Duodenum ~50%• Jejunum ~20%• Ileum ~15%
• Peak incidence in 6th
decade• Male predominance• Abdominal pain (67%),
obstruction (40%), bleeding/anemia (24%)
• 5 yr survival poor (~20%)
SI-AdenocarcinomaCrohn’s Disease
• Increased risk of SI adenocarcinoma(x12)
• Associated with sites of chronic inflammation
• Often “watched” as sites of “stable” CD activity
• Survival no worse than idiopathic SI-CA
SI-Adenocarcinoma
• Mayo Clinic series of 491 patients (1970-2005)
• Median survival 20.1 months
• Adjuvant chemotherapy not associated with increased survival (p=0.44)
Halfdanarson et al. 2010
Lymphoma
• Categorized by cell type; broadly grouped by Hodgkin’s and Non-Hodgkin’s
• Commonly present with abdominal pain, weight loss, bleeding, perforation, palpable mass
• Survival tied to cell type• Surgery reserved for
treatment of complications
Sarcomas/Gastrointestinal stromaltumors (GISTs)
• Derived from mesenchymal cells• GISTs are KIT (CD117) positive cells
thought to originate from interstitial cells of Cajal
• 10% of SI tumors• Usually present with bleeding which
can be significant, then obstruction• In a series of 288 GISTs, 69%
detected due to symptoms, 21% incidental findings at surgery, 10% found at autopsy
• Correct histologic type of GIST essential as tyrosine kinase inhibitor agents improve survival in KIT positive cell types
Other tumors of the SI
• Usually metastatic disease
• Melanoma• Breast• Lung
• Common presentation• Anemia• Abdominal pain• Obstruction• Perforation
• Surgery for complications of the disease
Summary• Small intestine malignancies are rare GI
tumors but incidence is increasing• Associated with higher mortality when
compared to colo-rectal malignancies• Non-specific symptoms, difficult to diagnose,
delayed presentation is associated with advanced disease stage
• Surgery primary treatment for symptoms and potential cure
• Current chemotherapy regimens not very effective (exception is KIT+ GISTs)
References
• Gustafsson et al. Int J Oncol. 2008;33:1121• Halfdanarson et al. Am J Surg. 2010;199:797• Qubaiah et al. Cancer Epidemiol Biomarkers
Prev. 2010;19:1908• Ruffolo et al. J Gastrointest Surg. Published
online 14 July 2010• Han et al. World J Gastroenterol. 2010;16:1527• Trikudanathan et al. Expert Opin Pharmacother.
2010:11:1695