SMALL BOWEL

NEOPLASM
JAVAIRIA A. SABDULLAH
POST GRADUATE INTERN
NMMC
Objectives:

Review about the Anatomy and Physiology of the small intestine

Describe the general features of small bowel neoplasms, clinica
presentation, and diagnostic tests followed by a description of the
more common tumor types and their management.
Anatomy and Physiology
Gross Anatomy

tubular structure that extends from the

pylorus to the ileocecal junction
measuring about 4-6 meters in adult

compared with large intestines, have

plicae circulares or valvulae conniventes
or valves of Kerkring which are internal
mucosal folds visible grossly and on
radiography

composed of 3 segments: Duodenum,

Jejunum and Ileum
SEGMENT LOCATION EMBRYOLOGY REMARKS

• 1st portion: • 25cm in lengt

intraperitoneal foregut • wraps around
DUODENUM • 2nd, 3rd & 4th pancreatic he
portion: • extends from
retroperitoneal pylorus and e
at the ligame
Treitz
 • proximal 2/5 of jejunoileal seg
>Compared to Ileum:
• larger diameter
• thicker wall
JEJUNUM Intraperitoneal midgut • less fatty mesentery
• more prominent plicae circula
• longer vasa recta
• fewer vascular arcades & Peye
patches

• distal 3/5 of jejunoileal segme

ILEUM Intraperitoneal midgut • demarcated from the cecum
by the ileocecal valve
Blood Supply and Lymphatic Drainage

SEGMENT ARTERIAL SUPPLY VENOUS DRAINAGE LYMPH DRAINAGE

Superior and Inferior Pancreaticoduodenal

Duodenum pancreaticoduodenal artery nodes to gastroduodena
nodes, celiac nodes, an
superior mesenteric
nodes

Jejunum and Superior mesenteric artery Superior mesenteric

Ileum nodes
Histology

mucous membrane contains

Peyer patches which are clusters
of lymphoid follicles that are part
of gut-associated lymphoid tissue
that plays a role in mucosal
defense mechanism

mucosa contains crypts of

Lieberkuhn and villi which are not
seen in large intestines
Physiology
About 8-9L of fluid enter the small intestines, of
which 80% are absorbed and about 1.5L continue
to the colon

Major site of carbohydrate, protein and fat

digestion and absorption

Contraction of the outer longitudinal muscle layer

results in bowel shortening, while that of the inner
circular layer, in luminal narrowing

Important hormones produced by the small

intestines include somatostatin, secretin,
cholecystokinin and motilin
 0.2% to 0.3% prevalence rate
identified at autopsy w/c is
significantly higher than the rate
of operation; suggests that the
majority of small bowel tumors
are asymptomatic
Small bowel
tumors
these lesions are most frequently
encountered in the duodenum
as incidental finding during
esophagogastroduodenoscopic
(EGD) examinations
 Benign neoplasms
(30%-50% )

Adenomas Lipomas Hamartomas Hemangiomas

Fibromas Lymphangioma Neurofibromas

 Adenomas • Occur predominantly in duodenum (periampullary region)
• Associated with potential risk for malignant transformation

True fatty neoplasms of small bowel that are typically asymptomatic

Lipomas because these tumors are polypoid, compressible intraluminal lesions,
they are predisposed to induced intussusception
• Arise predominantly in jejunum & ileum; often represents as
Hamartomas intussusception
• Associated with Peutz-Jeghers syndrome
• Rare lesions of the small bowel, developing predominantly in
Hemangiomas jejunum & ileum
• Usually solitary lesions
• Malignant degeneration is rare
 Presents as polypoid pedunculated masses on a stalk, sessile or mural
based nodule within mucosa
Adenomas

Histologically classified as tubular, villous, and tubulovillous ; tubular

adenomas have the least aggresive features; villous adenomas have the
most aggresive features and noted to be large, sessile and located in
the second portion of the doudenum

Malignant degeneration has been reported to be present in up to 45%

of villous adenocarcinoma by the time of diagnosis; patient with FAP
(Familial Adenomatous Polyposis) have nearly 100% cumulative lifetime
risk of developing duodenal adenomas that have potential to undergo
malignant transformation

Indeed, doudenal cancer is the leading cause of cancer related death

among patients with FAP who have undergone colectomy
Primary small bowel cancers are rare.

 Adenocarcinoma comprise 35-50%; duodenum

 Carcinoid tumors comprise 20-40%; ileum
 Lymphomas comprise approx 10-15%; ileum
 GI stromal tumors (GISTs) are the most common mesenchymal tumor
arising in the small intestine and comprise the vast majority of tumor
that were formerly classified as leiomyomas, leiomyosarcoma and
smooth muscle tumors of the intestine
Epidemiology

Small bowel tumors are uncommon

<5% of primary GI malignancies arise from the small intestine
Approximately 1/3 of neoplasms are benign and 2/3 are malignant
Small bowel neoplasm are less common in women than in men
• Most patients with cancers are in their 5th or 6th decade of life
• Malignant small bowel tumors are more common in the distal small bowel
• Location of tumor:
-20% arise in duodenum
-30% arise in jejunum
-50% arise in ileum
Reported risk factors includes:
consumption of red meat
ingestion of smoked or cured foods
Crohn's disease, celiac sprue, hereditary nonpolyposis colorectal cancer (HNPCC)
familial adenomatous polyposis (FAP) and Peutz-Jeghers syndrome, gardne
disease, Immunosuppression, Infection
Clinical Presentation

partial small bowel

obstruction with assoc. hemorrhage,
symptoms of crampy usually indolent,
asymptomatic
abdominal pain and is the second
until they become
distention, nausea and most common
large
vomiting is the most mode of
common mode of presentation
presentation
Physical exmanination

jaundice secondary cachexia,

to biliary obstruction hepatomegaly and
palpable may be present or ascites may be
abdominal mass hepatic metastasis present w/
may be present advanced disease
Carcinoid tumors of small intestine

-usually diagnosed after development of metastatic disease

-associated with a more aggresive behavior
-carcinoid tumor derived liver metastases will develop manifestation of
carcinoid syndrome (diarrhea, flushing, hypotension, tachycardia, and
fibrosis of endocardium and valves of right heart)
Pathophysiology
dilution of environmental carcinogens in the liquid
chyme present in the small intestinal lumen

rapid transit of chyme, limiting the contact time

between carcinogen and intestinal mucosa

relatively low concentration of bacteria in small

intestinal chyme and therefore a relatively low
concentration of carcinogenic products of bacterial
metabolism
mucosal protection by secretory IgA and hydrolase
such as benzpyrene hydroxylase that may render
carcinogens less active
efficient epithelial cellular apoptotic mechanisms
that serve to eliminate clones of harboring genetic
mutations
Small intestinal adenocarcinomas are believed to arise from preexisting
adenomas through a sequential accumulation of genetic abnormalities
like in colorectal cancer.

A defining feature of GISTs is their gain-of-function mutation of proto-

oncogene KIT, a receptor kinase and believed to be the central event in
GIST pathogenesis.
Diagnostics
Due to nonspecific symptoms, lesions are rarely diagnosed preoperatively.

Laboratory tests are nonspecific w/ the exception of:

elevated serum 5-hydroxyindole acetic acid (5-HIAA) level in patient w/ carcinoid

yndrome

elevated carcinoembryonic antigen (CEA) levels are associated with small intestina
adnocarcinomas but only in the presence of liver metastases
Contrast radiography of small intestine
may demonstrate benign and malignant lesions

Enterocolysis
sensitivity of 90% in detetion of small bowel tumors and is the test of choice
particularly for tumors located in the distal small bowel

CT scan
can detect abnormalities in 70%-80% of cases w/ small bowel tumor and assess
or metastatic spread
can demonstrate large tumors and useful in staging of intestinal malignancies
Esophagogastricdoudenoscopy
visualize tumor located in the doudenum

Colonoscopy
visualize distal ileum

Capsule endoscopy, double-baloon endoscopy

used to evaluate distal small bowel lesions
Intraoperative enteroscopy
visualize small intestinal tumors beyond the reach of standard endoscopic
echniques

CT and MR enterography

noninvasice tests to look for small bowel masses

PET scan
help assess metabolic activity of lesions and risk of malignancy
Management
 Lesion Management
Duodenal adenoma • If <2cm : endoscopic polypectomy
• If >2cm: transduodenal polypecctomy or segmental
duodenal resection or pancreaticoduodenectomy
Duodenal adenomas in • Endoscopic polypectomy
patients w/ FAP • Surveillance endoscopy at 6 months interval then
annually
Duodenal adenocarcinoma • Pancreaticoduodenectomy for proximal lesions
• Segmental resection for distal duodenal lesions
Jejunal or Ileal tumors Segmental resection with 5cm of tumor-free proximal &
distal margins
Localized small bowel Wide en bloc resection (includes adjacent mesentery &
carcinoid lymph nodes)
Metastatic carcinoids Dubulking surgery

Carcinoid syndrome • Preoperative somatostatin or octreotide

• Debulking including resection of hepatic metastases

Localized or resectable GIST Wide local excision of the primary tumor with in
continuity resection of adherent organs

Unresectable or metastatic Imatinib (Gleevec)

GIST

Advanced disease Palliative resection or bypass (bowel diversion)

Small bowel lymphoma • Primary chemotherapy (CHOP regimen)

• Segmental resection for tumor complications
Prognosis

Complete resection of duodenal adenocarcinomas is associated with

postoperative 5 year survival rates ranging from 50% to 60%.

Complete resection of adenocarcinomas located in the jejunum or ileum is

associated with 5 year survival rates of 20%-30%.

5 year survival rates of 75-95% following resection of localized small

intestinal carcinoid tumors have been reported.
• Overall 5 year survival rate for patients diagnosed with intestinal
lymphoma ranges 20-40%; localized lymphoma amenable to surgical
resection has 5year survival rate of 60%

• Recurrence rate following resection of GISTs averages 35%; 5year survival

rate following surgical resection ranges 35-60%

• Both tumor size and mitotic index are independently correlated with
prognosis; low grade tumors (mitotic index <10 per high power field)
measuring <5cm in diameter are associated w/ excellent prognosis
Sources:
Schwartz's Principles of Surgery, 11th edition
Surgery Platinum, 2018 edition
Thank you!