r Gastrointestinal and Hepatobiliary Malignancies 0039-6109/86 $0.00 + .

20

Malignant Tumors of
the Small Intestine

Richard G. Martin, M.D. *

The small intestine comprises the duodenum, the jejunum, and the
ileum. Although its total length is much greater than that of the remaining
gastrointestinal tract, this area develops fewer tumors. In our series of
11,438 cases of gastrointestinal tumors seen between 1944 and 1982 at The
University of Texas M.D. Anderson Hospital and Tumor Institute at
Houston, there were only 280 cases of tumors located in the small intestine
(Table 1). The reason for this is not known; however, there are several
theories. One is that because the transit time is rapid the intestinal contents
do not stay in this portion of the gastrointestinal tract for any substantial
length of time. However, the transit time through the esophagus is also
rapid, and it produces more tumors than does the small intestine. Another
theory is that because the contents are rapidly absorbed, going to the liver
for assimilation, the by-products do not stay in this area. Also, the small
intestine is relatively free of bacteria that may take part in the breakdown
of certain substances within the bowel content, causing carcinogenic agents
to be formed.
Included in the aforementioned series of 280 small-bowel tumors are
63 ampulla of Vater lesions. The reason these were included is that they
were categorized with the small-bowel tumors in our registry. It is very
difficult at times to decide whether such lesions develop from the bile ducts
or from the lining of the duodenum in the area of the ampulla of Vater.
However, these 63 patients will not be considered in detail. Therefore,
this review is based on a series of 217 small bowel malignancies.
All the tumors in this series were classified by our pathologist. Table
2 lists the number of cases in each location, with Table 3 showing the
distribution of the various histologic types within the small intestine. All
the sarcoma cases were classified as leiomyosarcoma except for two cases
ofliposarcoma arising in the ileum. It is interesting to note that the majority
of the adenocarcinoma lesions occur in the duodenum and the jejunum and

*Professor of Surgery, Department of General Surgery, The University of Texas M. D.

Anderson Hospital and Tumor Institute, Houston, Texas

Surgical Clinics of North America-Vol. 66, No.4, August 1986 779

780 RICHARD C. MARTIN

Table 1. Percentage of Gastrointestinal Tumors by Specific Site

LOCATION PER CENT

Esophagus 10.8
Stomach 16.4
Small Intestine 2.4
Large Intestine 48.7
Rectum 21.6
Total 100.0

that the sarcoma, carcinoid, and lymphoma lesions are located mostly in
the ileum.
The male:female ratio is fairly even except that the male patients
predominate in the adenocarcinoma category, and although the numbers
are small, all the lymphoma patients are male (Table 4). Also of interest is
the predominance in this series of the number of white patients over the
number (17) of black patients. All of these tumors appeared in the second
decade of life or later, except for two lymphomas occurring in pediatric
patients. Small bowel tumors occur most frequently in the fourth, fifth,
and sixth decades of life (Table 5).
Associated conditions that might be risk factors are Crohn's disease or
ileitis and multiple polyposis. We had in this series two cases that had
associated Crohn's disease. It is still difficult to determine whether long-

Table 2. Number of Small-Bowel Tumors by Specific Site

LOCATION NUMBER OF CASES

Duodenum 45
Jejunum 42
Ileum 87
Not specified ~
Total 217

Table 3. Histologic Types of Tumors by Specific Sites in the Small Intestine

CASES IN CASES IN CASES IN CASES IN
HISTOLOGY DUODENUM JEJUNUM ILEUM UNSPECIFIED SITE

Adenocarcinoma 35 25 17 10
Sarcoma 3 9 15 6
Carcinoid 7 6 51 25
Lymphoma ~ ~ ...1 ~
Totals 45 42 87 43

Table 4. Sex Distribution of Small-Bowel Tumors

HISTOLOGY MALE FEMALE

Adenocarcinoma 57 30
Sarcoma 18 15
Carcinoid 48 41
Lymphoma ~ ~
Totals 131 86
MALIGNANT TUMORS OF THE SMALL INTESTINE 781
Table 5. Small Bowel Tumors by Specific Type and Patient Age
ADENO·
AGE IN YEARS CARCINOMA SARCOMA CARCINOID LYMPHOMA

<20 0 0 0 2
20--29 3 1 0 0
30--39 6 1 7 0
40-49 23 6 24 1
50--59 25 9 20 2
60--69 22 7 29 2
70-79 7 8 9 1
;=: 80 ~ J ~ Q
Totals 86 33 89 8

standing Crohn's disease is a precursor for malignancy of the small intes-

tine. 12 Some electron microscopy studies have been done to see if this
could be proven. 3 Multiple polyposis, of course, is a risk factor in the
development of carcinoma of the colon; however, cases have been re-
ported,5. 11 including one in our series, in which polyps developed in other
areas of the gastrointestinal tract in patients having polyposis coli, and
these may be associated with malignant growths in the small bowel. A black
woman lived for over 20 years with polyposis of the colon, having had a
total colectomy. Later she developed polyps in the stomach and duodenum,
which were removed and showed dysplasia but no malignant changes. She
then developed polypoid lesions in the ileum, which developed into
adenocarcinoma of the ileum before her death.

SIGNS AND SYMPTOMS OF SMALL-BOWEL MALIGNANCY

The signs and symptoms of small-bowel malignancy are very vague

and often are present for a number of months or even 1 to 2 years before
the diagnosis is made. These may be divided into chronic and acute signs
and symptoms (Table 6). The chronic signs and symptoms may include
melena, anemia, malaise, and fatigue. These are frequently present. Often
the stomach and the colon are studied thoroughly for the cause of anemia
but the small bowel is overlooked. Whenever anemia is present without
known cause, and there is blood in the stool, a small-bowel barium follow-
through study must be done to determine the possibility of malignancy in

Table 6. Chronic and Acute Signs and Symptoms of Small-Bowel Malignancies

CHRONIC SIGNS AND SYMPTOMS ACUTE SIGNS AND SYMPTOMS

Melena, anemia Hemorrhage

Vague pain Pain
Obstruction Obstruction
Malaise Perforation
Weight loss
Diarrhea
Jaundice
Mass
782 RICHARD G. MARTIN

this area. Pain may be very vague or it may be acute. Obstruction is quite
frequently chronic, but this also may become acute. Rarely, a mass is felt
in the abdomen. Weight loss is another symptom that is quite frequently
present, along with malaise. Epigastric or abdominal distress is quite
frequent, and diarrhea may be present, as may alternating diarrhea and
constipation. Jaundice can be present in malignancies of the duodenum and
ampulla of Vater; it is most likely present in ampulla of Vater lesions and
may wane in intensity because of the intermittent obstruction. Acute
symptoms frequently bring the patient to surgery, where the diagnosis is
made. Hemorrhage or severe bleeding from the gastrointestinal tract may
occur, as may acute obstruction and perforation. Perforations were noted
in our series in two adenocarcinomas of the small bowel and also in a
lymphoma of the ileum. Barium follow-through studies are the only certain
method of diagnosing small-bowel tumors. Endoscopy may be of value in
diagnosing duodenal lesions. Computed tomography (CT) has not proven
to be of much value unless the mass becomes quite large, extending beyond
the bowel wall. In cases of carcinoid tumors, arteriograms can be of value.
A selective arteriogram, in which the dye is injected directly into the
superior mesenteric artery, frequently shows carcinoid tumors well. It is
also helpful in determining whether the carcinoid lesions are solitary or
multiple. Metastatic lesions may be the first evidence to suggest a primary
small-bowel tumor. This is especially true with carcinoids; often biopsies of
liver metastases or, in one case, bone metastases, gave the diagnosis of a
carcinoid tumor. The primary lesion of the small intestine was found with
follow-through barium studies.

METASTATIC LESIONS

Metastatic lesions should be mentioned as malignant lesions of the

small bowel. They are not included in this series because they are not
primary lesions, but it behooves one to be aware that metastases may occur
in the area of the small bowel, causing the symptoms noted above. Such
metastatic lesions have been known to occur in cases of melanoma,
carcinoma of the cervix, and lung, breast, and soft-tissue tumors. Often
these metastases to the small bowel cause bleeding and obstruction. In one
case they caused an intussusception and in another an obstruction due to
blockage of the lumen by the tumor itself. In a patient with a known history
of malignancy, obstructive symptoms, or bleeding from the gastrointestinal
tract, a metastatic lesion must be considered. Although the signs and
symptoms tabulated above may be present in anyone of the categories of
small-bowel malignancies, there are certain ones that stand out or are more
common with specific histologic types. In the case of lymphoma, the main
symptoms that occur are obstruction, chronic obstruction, malaise, and
possibly bleeding. In sarcomas, it was very interesting that bleeding was
the main symptom, along with pain. 1, 6, 13 Obstruction was not common in
this category, mainly because the tumor more often grows out from the
lumen of the bowel instead of into it as a polypoid lesion. The tumor
frequently necroses in the center, causing the bleeding. Carcinoid tumors
MALIGNANT TUMORS OF THE SMALL INTESTINE 783
may often cause vague abdominal distress with obstructive symptoms.
Frequently, diarrhea is a common symptom with these tUmors along with
possible flushing and other characteristics of the carcinoid syndrome. When
a carcinoid lesion is suspected, urinary 5-HIAA determination should be
made. Adenocarcinomas frequently cause pain and obstruction, which may
be intermittent. The obstruction in these cases is usually due to polypoid
lesions that grow into the lumen or napkin-ring lesions that cut off the
lumen. Chronic anemia is also quite often present.

TREATMENT AND PROGNOSIS

The treatment of small bowel tumors is primarily surgical. The pre-

operative preparation of these patients depends a great deal on the presence
of severe bleeding or obstruction. If obstruction is present, one must be
very careful about any gastrointestinal preparation such as cathartics. In
the presence of either pain or obstruction, the operation may become an
emergency. Cleansing of the bowel is not as important for these lesions as
for colon lesions; however, it is good to have the colon well prepared if at
all possible. The treatment of choice is segmental resection of the small
bowel. As much of the ileum as possible should be preserved at the time
of resection to prevent postoperative diarrhea and the loss of vitamin B12
absorption. Ileal lesions may require a right hemicolectomy. All regional
lymph nodes are resected with the primary lesion. If the lesion is in the
third or fourth portion of the duodenum, a segmental resection is frequently
possible. However, if the lesion is of significant size and has extended into
the pancreas, a pancreatoduodenectomy or Whipple procedure may be
indicated. Tumors arising in the third and fourth portions of the duodenum
may ulcerate into the superior mesenteric vessels, causing hemorrhage.
The vessels also may cause difficulty when proximal jejunal lesions near the
ligament of Treitz are rejected. The prognosis for small-bowel tumors, as
in other areas of the gastrointestinal tract, depends on the extension of
tumors through the bowel wall and the number of lymph nodes positive
for metastasis. Perforation of a lesion usually causes peritoneal seeding.
In cases of nonresectable lesions, a sidetracking procedure should be
performed to establish continuity of the lumen of the bowel to permit oral
nutrition. For lymphomatous lesions, chemotherapy frequently may be
used as an adjunct to surgery or, at certain times, in place of surgery.
Radiation therapy may be indicated also for these lesions. Adjunctive
therapy for adenocarcinoma and sarcoma lesions is of little value at this
time. Liver embolization or infusion may be useful in controlling carcinoid
syndrome symptoms due to liver metastases. Aggressive surgical attack
upon solitary metastatic lesions of the liver, lung, or other locations should
be carried out.
Pre- and postoperative intubation for decompression usually can be
accomplished by using a sump-type nasogastric tube. Rarely is it necessary
to use a long intestinal tube, which is very difficult to place properly.
The 5-year survival rates for the various categories of small-bowel
lesions in this series do not differ greatly from those that have been reported
784 RICHARD G. MARTIN

Table 7. Survival Rates for Small-Bowel Turrwrs by Specific Types

MORTALITIES MORTALITIES MORTALITIES MORTALITIES 5-YEAR
AFTER 3 AFTER 5 AFTER 10 AFTER 20 SURVIVAL
HISTOLOGY YEARS YEARS YEARS YEARS RATE

Lymphoma <3 1 1 3 62.5%

Sarcoma 27 4 1 1 18.2%
Carcinoid 44 32 12 1 50.5%
Adenocarcinoma 69 10 8 0 20.6%

in the literature. 2 , 4, 7, 8, 9, 10 Table 7 tabulates the 5-year survival rates for

the various categories of tumors: lymphomas, approximately 62.5 per cent;
sarcomas, 18.2 per cent; and carcinoid tumors, 50.5 per cent, with patients
in the last group quite frequently surviving for long periods of time with a
considerable volume of disease, often in the liver and other areas of the
body, including the bone. For adenocarcinoma tumors, our 5-year survival
rate was 20.6 per cent. A large number of our patients in all of these
categories, when they succumbed to their disease, did so in the first 3
years after diagnosis. The low survival rate for small-intestinal malignant
tumors is most likely influenced by the long prediagnosis period, allowing
for the increased stage of disease when diagnosed.

SUMMARY
The four main histologic categories of malignant small intestine tumors
are (1) adenocarcinoma, (2) sarcoma, (3) carcinoid, and (4) lymphoma. Signs
and symptoms may be chronic or acute and include bleeding, obstruction,
pain, and weight loss. When chronic, the symptoms are so vague that early
diagnosis is difficult. Often the diagnosis is made only at the time of surgery.
Treatment is segmental resection of intestine with adequate margin of the
tumor and resection of the regional lymph nodes. Prognosis is evaluated
by tumor extension through the bowel wall and lymph node metastasis.
The 5-year survival rates are low except for carcinoid and lymphomatous
lesions.

REFERENCES
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large bowel. Cancer, 42:1375-1384, 1978.
2. Arthaud, J. B., and Guinee, V. F.: Jejunal and ileal adenocarcinoma. Am. J. Gastroen-
terol., 72:638-646, 1979.
3. Balazs, M.: Electron microscopic study of adenocarcinoma of the small bowel associated
with Crohn's disease. Exp. Pathol., 23:53-62, 1983.
4. Barclay, T. H. c., and Schapira, D. V.: Malignant tumors of the small intestine, Cancer,
51:878-881, 1983.
5. Bussey, H. J. R., Veale, A. M. 0., and Morson, B. C,: Genetics of gastrointestinal
polyposis. Gastroenterology, 74:1325-1330, 1978.
6. Chiotasso, P. J. P., and Fazio, V. W.: Prognostic factors of 28 leiomyosarcomas of the
small intestine. Surg. Gynecol. Obstet., 155:197-202, 1982.
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7. Colcock, B. P., and Braasch, J. W.: Surgery of the Small Intestine in the Adult.
Philadelphia, W. B. Saunders Company, 1968, pp. 66--88.
8. Coutsoftides, T., and Shibata, H. R.: Primary malignant tumors of the small intestine.
Dis. Colon Rectum, 22:24-26, 1979.
9. Garvin, P. J., Herrmann, V., Kaminski, D. L., et al.: Benign and malignant tumors of
the small intestine. Curro Prabl. Cancer, 3:1-46, 1979.
10. Goel, I. P., Didolkar, M. S., and Elias, E. G.: Primary malignant tumors of the small
intestine. Surg. Gynecol. Obstet., 143:717-719, 1976.
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13. Wood, David, A.: Tumors of the Intestines: Atlas of Tumor Pathology, Section
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Department of Surgery
M.D. Anderson Hospital and Tumor Institute
Texas Medical Center
6723 Bertner Avenue
Houston, Texas 77030