SMALL INTESTINE NEOPLASMS

AND CARCINOID TUMORS

BY- HAPPY MALIK AND KOMAL JAISWAL
 SMALL
INTESTINE
 Anatomy
A. Duodenum:
 Retro-peritoneal
 Supplied by the celiac artery & SMA
B. Jejunum:
 Occupies upper left of the abdomen
 Thicker wall and wider lumen than the
ileum
 Mesentery has less fat and forms only 1-2
arcades
C. Ileum:
 Occupies the lower right; has more fat and
forms more arcades
 Contains Payer ’s patches
 Ileum & jejunum is supplied by the SMA
 TUMORS OF THE SMALL INTESTINE
CLASSIFICATION
• Non-neoplastic polyps
 Hamartomatous polyps (Juvenile & Peutz-Jeghers polyps)
 Inflammatory polyps
 Lymphoid polyps
• Neoplastic ( epithelial) polyps
 Benign polyps (adenoma)
 Malignant lesions
 Adenocarcinoma
 Carcinoid
• Mesenchymal lesions
 GI stromal tumors (benign & malignant)
 Others ( lipoma, neuroma, angioma, Kaposi sarcoma)
• Lymphoma
 Diffuse large cell lymphoma. MALT cell lymphoma.
 Epidemology of SI Tumors
• Sites of the highest risk are the duodenum, for adenocarcinomas, and the ileum, for
carcinoids and lymphomas.
• In industrialized countries, small bowel cancers are predominantly adenocarcinomas;
• In developing countries, lymphomas are much more common.
• The incidence of small bowel cancer rises with age and has generally been higher
among males than among females.
• The risk factors for small bowel cancer
include

 Dietary
Cigarettefactor
smoking,
 Alcohol intake,
 Medical conditions -Crohn's disease, familial adenomatous polyposis,
cholecystectomy, peptic ulcer disease, and cystic fibrosis.
• The protective factors may include rapid cell turnover, a general absence of bacteria,
an alkaline environment, and low levels of activating enzymes of precarcinogens.
SITE
: – Adenocarcinomas - proximal small bowel.
– other malignant lesions - in the distal intestine.
– Adenomas - are found in the duodenum, 30% are found in the jejunum, and 50%
are found in the ileum.
– Lipomas - are most common in the ileum
– Leiomyoma - smooth muscle origin
– Hemangiomas - the jejunum is the most commonly affected small bowel
segment.
– PeutzJeghers syndrome - The entire jejunum and ileum are the most usual
portions of the gastrointestinal tract involved with these hamartomas.

Carcinoid tumors and lymphomas are more frequent than adenocarcinoma and
leiomyo-sarcoma.
 SMALL-BOWEL
• BENIGN NEOPLASMS
– Adenomas are the most common benign neoplasm of the small intestine.
– Other benign tumors include fibromas, lipomas, hemangiomas,
lymphangiomas, and neurofibromas.
• Majority of small bowel neoplasm are benign
• Adenomas, lipomas, hemangiomas, neurogenic tumors
• Frequently asymtomatic & identified incidentally
• Can present with intersusception, small bowel obstruction, bleeding,
anaemia
• Inv: capsule endoscopy & small bowel endoscopy
• Symptomatic lesion can be treated by small bowel resection & anastomosis.

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 Leiomyomas
• Leiomyomas, are the most common symptomatic benign
neoplasms of the small bowel.
• As the origin of these tumors has become clearer,
pathologists have shifted from designations such as
leiomyoma or leiomyosarcoma to the term stromal tumors
(i.e., GISTs). And benign GISTs are three to four times
more common than malignant GISTs.
• Currently, these tumors are thought to arise from the
interstitial cell of Cajal and made up of spindle (70%) and
epithelioid (30%) cells
Leiomyoma
ADENOMAS
Three primary types:
 True adenomas
 Villous adenomas
 Tubulovillous
• Mostly asymptomatic some times present with bleeding and
obstruction with most occurring singly and found incidentally.
• The malignant potential of these lesions is reportedly between
35% and 55%.
• Treatment is determined by location and adenoma type.
 Jejunum and ileum - segmental resection.
 duodenal adenomas - duodenal resection by
pancreaticoduodenectomy
 sporadic adenomas - pancreas-preserving duodenectomy
(endoscopic or open polypectomy can be performed if
technically feasible.)
DIAGNOSIS
• . Diagnosis can usually be accomplished by endoscopy
and biopsy, and
• symptomatic lesions in an accessible region should be
resected by simple excision.
• There is no malignant potential for Brunner gland
adenomas
Lipomas
• Also included in the category of stromal tumors.
• present as single intramural lesions located in the submucosa.
• occur in the sixth and seventh decades of life and are more
frequent in men.
• Less than one third of these tumors are symptomatic
• manifestations are obstruction and bleeding from superficial
ulcerations.
• The treatment of choice for symptomatic lesions is excision.
• have no malignant potential, when found incidentally, should
be removed only if simple resection.
LIPOM
A
Hemangiomas
• Developmental malformations as submucosal proliferation
of blood vessels at any level of the gastrointestinal tract.
 the jejunum is the most commonly affected small bowel
segment.

• Can 3% to 4% of all benign tumors of the small bowel and

are multiple in 60% of patients.
• if inherited disorder known as OslerWeber-Rendu disease.
• Can present in the lung, liver, and mucous membranes.
• The most common symptom of small bowel hemangiomas
is intestinal bleeding.
• Angiography and 99mTc–red blood cell scanning are the
most useful diagnostic studies.
• Intraoperative trans illumination and palpation can be
helpful
• Resection of involved segment to be done.
Peutz-Jeghers Syndrome

• Hamartomas of the small bowel occur as part of the

PeutzJeghers syndrome
• 1- to 2-mm, brown or black spots located in the entire jejunum
and ileum are the most usual portions of the gastrointestinal
tract involved with these hamartomas;
• however, 50% of patients may also have rectal and colonic
lesions, and 25% of patients have gastric lesions.

Symptoms
• recurrent colicky abdominal pain,

Sign
• Intermittent intussusception.
• Lower abdominal pain associated with a palpable mass.
• Hemorrhage as a result of autoamputation of the polyps
Hamartoma
 SMALL-BOWEL
• NEOPLASMS
MALIGNANT
– The small intestine is frequently affected by metastases from or local invasion by
cancers originating at other sites.
– Primary small-bowel cancers are rare
• Adenocarcinomas: comprise 35–50 percent of all cases
• carcinoid tumors comprise 20–40 percent
• lymphomas comprise approximately 10–15 percent.
• Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal
tumors arising in the small intestine and comprise up to 15 percent of
smallbowel malignancies.

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 SMALL-BOWEL
• NEOPLASMS
Clinical Presentation
– Most small-intestinal neoplasms are asymptomatic until they become large.
– Small-bowel obstruction is the most common mode of presentation.
– Hemorrhage is the second most common mode of presentation.
– Physical examination may reveal an abdominal mass or signs of intestinal
obstruction.
– Fecal occult blood test may be positive. Cachexia or ascites may be present with
advanced disease.
– Lesions in the periampullary location can cause obstructive jaundice or
pancreatitis.

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 Malignant
neoplasm:
Histologic types:
Tumor type Cell of origin Frequency Predominant
Site

adenocarcinoma Epithelial cell 35 – 50% Duodenum

carcinoid Enterochromaffin 20 – 40% Ileum

cell

lymphoma lymphocyte 10 – 15% Ileum

GIST (gastrointestinal ? Interstitial cell of 10 – 15% -

stromal tumors) Cajal
 Adenocarcinoma

• Adenocarcinomas constitute about 50% of the malignant

tumors of the small bowel in most reported series.
• The peak incidence is in the seventh decade of life, and
most series show a slight male predominance.
• Tumors of the duodenum tend to present somewhat earlier
than those occurring in the most distal intestine, with
symptoms of jaundice and chronic bleeding.
• Adenocarcinomas of the jejunum and ileum usually produce
symptoms that may be more nonspecific and include vague
abdominal pain and weight loss.
• Most common CA of small bowel
• Most common in duodenum and proximal jejunum half
involve the ampulla of Vater.
• These are most commonly found in the duodenum, except in
patients with Crohn's disease, in whom most are found in the
ileum.
• Lesions in the periampullary location can cause obstructive
jaundice or pancreatitis.
• Adenocarcinomas located in the duodenum tend to be
diagnosed earlier in their progression than those located in the
jejunum or ileum, which are rarely diagnosed prior to the
onset of locally advanced or metastatic disease.
• Clinical features: anaemia, GI bleeding, intususception,
obstruction
• As with adenocarcinomas in other organs, survival of patients
with small bowel adenocarcinomas is related to the stage of
disease at the time of diagnosis
Pathophysiology
• (1) dilution of environmental carcinogens in the liquid chyme present in the small-
intestinal lumen
• (2) rapid transit of chyme, limiting the contact time between carcinogens and the
intestinal mucosa.
• (3) a relatively low concentration of bacteria in small-intestinal chyme and,
therefore, a relatively low concentration of carcinogenic products of bacterial
metabolism.
• (4) mucosal protection by secretory IgA and hydrolases such as benzpyrene
hydroxylase that may render carcinogens less active.
• (5) efficient epithelial cellular apoptotic mechanisms that serve to eliminate clones
harboring genetic mutations.
Large circumferential adenocarcinoma of
the
jejunum
– Risk factors
• Consumption of red meat
• Ingestion of smoked or cured foods
• Crohn disease
• Celiac sprue
• Peutz-Jeghers syndrome.
• Familial adenomatous polyposis (FAP)
– nearly 100 % cumulative lifetime risk of developing duodenal adenomas
that have the potential to undergo malignant transformation.
– The risk of duodenal cancer in these patients is more than 100-fold than in
the general population
 Diagnosis
•Due to absence or nonspecific symptoms these lesions are rarely
diagnosed preoperatively.

•Laboratory tests are nonspecific EXCEPT

I. Elevated serum 5-hydroxyindole acetic acid (5-HIAA) levels
in carcinoid syndrome.
II. Elevated carcinoembryonic antigen (CEA) levels in
adenocarcinomas, but only in the presence of liver metastases.

• Contrast radiography of the small intestine may demonstrate

benign and malignant lesions
• Enteroclysis -sensitivity of 90% in the detection of small-
bowel tumors and is the test of choice, particularly for tumors
located in the distal small bowel.

• Upper GI with small-bowel follow-through examinations

sensitivities ranging from only 30 to 44%.

• CT scanning has low sensitivity for detecting mucosal or

intramural lesions, can demonstrate large tumors and is useful
in the staging of intestinal malignancies.

• Angiography or radioisotope-tagged red blood cell (RBC)

scans-Tumors associated with significant bleeding

• Flexible endoscopy in diagnosing duodenal lesions, and

colonoscope in the terminal ileum for visualization and biopsy
of ileal neoplasms.
Contrast radiograph demonstrating a lesion in the proximal jejunum determined
to be an adenocarcinoma of the small intestine at the time of surgery
CT scan demonstrating a small-bowel loop in the left upper quadrant with an irregularly thickened wall
and areas of ulceration (arrow). This lesion proved to be a jejunal adenocarcinoma
 Lymphoma
1. Primary lymphoma
2. Secondary lymphoma [ Metastatic ]

Primary lymphomas arise with in the lymphoid tissue of

GI tract.
• 1- 5 % of all GI malignancies are lymphomas.
• Secondary lymphomas are brought to GIT from
lymphomas primarily arising some where else in the
body.
• Lymphoma may involve the small intestine primarily or
as a manifestation of disseminated systemic disease.
• Primary small-intestinal lymphomas are most commonly
located in the ileum, which contains the highest
concentration of lymphoid tissue in the intestine

• Lymphomas constitute 7% to 25% of small bowel malignant

tumors in the adultin children younger than age 10 years,
they are the most common intestinal neoplasm.

• Risk of devloping in patient withceliac disease and

immunodeficiency states (e.g., AIDS).

• small intestine lymphomas are usually large, with most

larger than 5 cm; they may extend beneath the mucosa

• Microscopically, there is often diffuse infiltration of the

intestinal wall.
• Symptoms of small bowel lymphoma include pain,
weight loss, nausea, vomiting, and change in bowel
habits. Perforation may occur in up to 25% of the
patient.

• Primary lymphomas commonly arise in the stomach

and small intestine and carry better prognosis.
primary lymphoma of the ileum with tumor.
 TREATMENT

The treatment of adenocarcinomas and lymphomas of the small

bowel is

• wide resection including regional lymph nodes .This may

require pancreaticoduodenectomy (Whipple operation) for
duodenal lesions.

• surgical resection for cure is not possible. Therefore, palliative

resection should be performed to prevent further complications of
bleeding, obstruction, and perforation.

•If this is not possible, bypass of the involved segment may

provide relief of symptoms
A, Malignant tumors should be resected with a wide margin of normal bowel and a
wedge of mesentery to remove the immediate draining lymph nodes.

B, End-to-end anastomosis of the small bowel and repair of the mesentery

 METASTATIC NEOPLASM
• Metastatic tumors involving the small bowel are much more
common than primary neoplasms.
• The most common metastases to the small intestine are those
arising from other intra-abdominal organs, including the
uterine cervix, ovaries, kidneys, stomach, colon, and pancreas.
• Small intestinal involvement is by either direct extension or
implantation of tumor cells. Metastases from extra-abdominal
tumors are rare but may be found in patients with
adenocarcinoma of the breast and carcinoma of the lung.
• Cutaneous melanoma is the most common extra-abdominal
source to involve the small intestine, with involvement of the
small intestine noted in more than half of patients dying from
malignant melanoma.
Symptoms
• Anorexia
• weight loss
• anemia,
• bleeding
• partial bowel obstruction.
Treatment- is palliative resection to relieve symptoms or,
occasionally, bypass if the metastatic tumor is extensive and
not amenable to resection
A, Barium radiograph shows
“target
lesions” consistent with metastatic
melanoma of the small bowel
(arrow).

B, Specimen demonstrating
metastatic melanoma to the small
bowel.
 CARCINOID TUMOR.
Literally, carcinoid means carcinoma like.

•These are named carcinoid because these resemble in

biological behavior with carcinoma i.e., they have the ability to
metastasis though very slowly but the neoplastic cells forming
the tumors, microscopically do not appear typically malignant.

•These tumors arise from the diffuse components of endocrine

system that are present in different parts of the body.

•These tumors are now called “well-differentiated

neuroendocrine tumors.
•Carcinoids of the small bowel arise from enterochromaffin
cells found in the crypts of Lieberkühn also known as
argentaffin cells because of their staining by silver compounds

•Carcinoid tumors may be derived from the foregut (respiratory

tract, thymus), midgut (jejunum, ileum and right colon, stomach,
and proximal duodenum), and hindgut (distal colon and rectum).
Carcinoid syndrome.
25-50% carcinoid tumor develops carcinoid syndrome.

1.Diarrhea
2.Flushing
3.Hypotension
4.tachycardia
5.fibrosis of endocardium and valves of the right
heart.
• GI carcinoid produce carcinoid syndrome only when these
metastasize to liver but it is not necessary for extra intestinal
carcinoid
 Distribution of Gastrointestinal Carcinoids: Incidence of
Metastases and of Carcinoid Syndrome

AVERAGE METASTASIS CASES OF

SITE CASES (%)
CARCINOID SYNDROM
Esophagus 1 0E
Stomach 93 (2%) 23 8
Duodenum 135 (4%) 20 4
Jejunoileum 1032 (28%) 34 91
Meckel's diverticulum 42 (1%) 19 3

Appendix 1686 (45%) 2 6

Colon 91 (2%) 60 5
Rectum 592 (16%) 18 1
Ovary 34 6 17
Biliary tract 10 30 0
Pancreas 2 1
Total 3718 136
Diagnosis

• Carcinoid tumors of the small intestine are rarely diagnosed preoperatively.

• Barium radiographic studies of the small bowel may exhibit multiple filling
defects as a result of kinking and fibrosis of the bowel.

• Angiography and high-resolution ultrasonography can provide information on

mesenteric involvement as well as hepatic involvement.

• Carcinoid tumors produce serotonin which metabolized in the liver and the lung
to the pharmacologically inactive 5-hydroxyindoleacetic acid (5-HIAA).
Elevated urinary levels of 5-HIAA measured over 24 hours with high-
performance liquid chromatography are highly specific
• CT is useful in detecting hepatic and lymph node metastases and the extent of bowel
wall
and mesenteric involvement.
•Advantage of the fact that many of these tumors possess somatostatin receptors is
somatostatin receptor scintigraphy using 111In-labeled pentetreotide

• The plasma concentration of chromogranin A, a protein made in the secretory granules,

which is elevated in more than 80% of patients with carcinoid tumors is the test of choice
, the lack of specificity limits its usefulness as a sole marker.

• Positron emission tomography (PET) scan

• Capsule endoscopy
• Double ballon enteroscopy
Barium radiograph of a carcinoid
tumor of the terminal ileum

demonstrates fibrosis with multiple

filling defects and high-grade
partial obstruction (arrows).
A-Carcinoid tumor of the distal ileum demonstrates the intense
desmoplastic reaction and fibrosis of the bowel wall.

B -Mesenteric metastases from a carcinoid tumor of the small

bowel.
 TREATMENT
The treatment of carcinoid tumors requires a multidisciplinary
approach, and combined modalities may be the best option

• Surgical debulking,
• Hepatic artery embolization or chemoembolization,
• Medical therapy

•The treatment of patients with small bowel carcinoid tumors is

based on tumor size and site and presence or absence of
metastatic disease

•Primary tumors of less than 1 cm in diameter without evidence

of regional lymph node metastasis, a segmental intestinal
resection is adequate.
•Patients with lesions larger than 1 cm, with multiple tumors
or with regional lymph node metastasis, regardless of the size
of the primary tumor, wide excision of bowel and mesentery
is required.

•Lesions of the terminal ileum are best treated by right

hemicolectomy.
•Small duodenal tumors can be excised locally.
•Extensive lesions may require pancreaticoduodenectomy.
• In addition to treatment of the primary tumor, it is important
that the abdomen be thoroughly explored for multicentric
lesion.
 Chemotherapy: The role of chemotherapy is confined
predominantly to patients with metastatic disease who are
symptomatic and unresponsive to other therapies
30 -50% response
»Cyclophosphamide
»5-fluorouracil
»Streptozocin
Prognosis

• Carcinoid tumors have the best prognosis of all small bowel

tumors,

– Resection of a carcinoid tumor localized - 100% survival

rate.
– Five-year survival rates are about 65% among patients with
regional disease and
– 25% to 35% among those with distant metastasis
– Elevated level of chromogranin A, which was found to be
an independent predictor of an adverse prognosis.