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Tumors of the small bowel
Tumors
of the Small Bowel*
KARL J. SCHMUTZER, M.D., WALTER M. HOLLERAN,M.D. AND JAMESF. REGAN, M.D.,
Los Angeles, California From tbe Department of Surgery, Queen of Angels Hospital, Los Angeles, California, and tbe Ross-Loos Medical Group, Los Angeles, California.
to review the pathoIogy and hospita1 records of the Queen of AngeIs Hospital, which is a 500 bed private hospital, covering the years 1944 through 1963. During this twenty year period, a tota number of IOO patients with benign or maIignant primary tumors and fourteen metastatic tumors to the smaI1 bowel were recorded. It is the purpose of this study to evaIuate the various types of neopIasms encountered and to discuss certain features of symptomatoIogy, diagnosis and treatment.
this uItramodern and sophisticated era of medicine, the earIy diagnosis of tumors of the smaI1 bowe1 stiI1 remains a cIinica1 probIem. Whereas neoplasms of the upper part of the arimentary tract and the coIon are not onIy more common in occurrence but aIso signa their presence earIier, tumors of the smaI1 bowe1 are often not considered in the differentia1 diagnosis of intestina1 diseases as a resuIt of their reIativeIy Iow incidence and the usua1 Iack of earIy distinctive cIinica1 manifestations. After we had encountered a number of interesting and unusua1 cIinica1 situations pertaining to tumors of the smaI1 intestine, we decided
I
N
TABLE HISTOLOGIC
TYPE
TYPES
Of the IOO cases of primary tumors of the smaI1 bowe1, fifty-nine were benign and fortyone were maIignant. In contrast to other reported series on this subject, there is a preponderance of benign tumors in this unseIected group of patients. However, if the twenty-two cases of ectopic pancreas are excIuded, an approximateIy equa1 number of benign and maIignant tumors is in evidence.
I AND
LOCATION
Location
-
- -
TYPO
J+J-
DUO-
Total.. . Malignant: Carcinoma. Lymphoma..
3 I
5 17 5 3 3
0 2
4 21
17
59
3 3 0 1
13 7 5 16
7 24
41 100
I
.. .. . .. . . 1
8
..
. .. . . .
rota1
9 I
Leiomyosarcoma.. Cm&mid. .
Totd. Sum TotaI..
TYPES
UIlpecified
denum
Benign: Adenoma (polyp) Leiomyoma.. . . . . Lipoms. . . . . . . . Hemangioma. Lymphangioma.. Brunner’s gland adenoma.. . . Ectopic pancreas..
40
OF TUMORS
SITES
OF BENIGN
TUMORS
The most commonIy encountered benign Iesion of the smaI1 intestine was ectopic pancreas with twenty-two cases. (TabIe I.) Next in frequency were Ieiomyomas with seventeen cases, foIIowed by adenomas (five), Iipomas (five) (Fig. I), Brunner’s gIand adenomas (four), hemangiomas (three) and Iymphangiomas (three). The duodenum was the most common site of benign tumors (thirty-two of fifty-nine) because of the reIativeIy Iarge number of ectopic pancreata. The jejunum was next in frequency with seventeen tumors, incIuding two cases of ectopic pancreas. OnIy eight tumors were Iocated in the iIeum. The specific
-
-
AND
* Presented at the annual meeting of the Pacific Coast Surgical Association, San Francisco, CaIifornia, February g-12, 1964. American
Journd
of Surgery.
Volume IO& August
rg64
270
Tumors
of Small BoweI TABLE
SYMPTOMS
-
II
AND
FINDIKGS
Symptoms Tumor Bkeding
Pain
_.--
and Findings
1
4nemia
Obstruction
Has
.._______
I ntussusception
Weight Loss
T 1
I ncidenta1 Finding
Y-ray FiIm
.I
Benign: Adenoma (poIyp) Leiomyoma. . Lipoma................... Hemangioma Lymphangioma. . . Brunner’s gIand adenoma. Ectopic pancreas.. TotaI. Malignant : c arcmoma. * Lymphoma. . Leiomyosarcoma. Carcinoid TotaI.
.
I I
0
I
0
2
2
0
I
I
I 2 I
0
0
0
I
4 4
0
0
0
0
0
0
0
0
0
0
0
0
0
0
0
I
0
0
0
0
0
3 I3 4 3 3 3
2
0
0
0
0
0
2
20
5
2
2
2
I
2
12
49
4
3
4
4
I
3 3 I
0
I
0
3 3
I
I
.
. .
0
3
II
15
-
II I
-
site could not be determined
AND
SYMPTOMS
OF BENIGN
9 4
I
0
2
0
2
2
3
4
9
8
5
II
19
-
-
-
I
IO
-
A Iarge Ieiomyoma was overIooked in two instances in the preoperative examination. Each of these masses wouId have been easiIy paIpabIe if a bimanua1 rectoabdomina1 examination had been performed. Roentgenologic manifestations were present 20 per cent, of in tweIve, or approximateIy these cases. Forty-nine of the benign tumors were found either incidentally at operation or at necropsy.
from the records in two instances. Nine of seventeen Ieiomyomas were Iocated in the jejunum. It is evident from these figures that there is a dechne in the incidence of benign tumors from the pylorus to the iIeoceca1 vaIve. SIGNS
I
4 4
0
I I
TUMORS
The most common symptom and sign associated with benign tumors were pain, usuaIIy after eating, and bleeding. (TabIe II.) These were present in ten cases. A mass was paIpated in two cases. Obstruction of the smaII bowe1 was present in two instances. In contrast to other reported series, intussusception was encountered in only one patient. However, the finding of a hypertrophied intestinal waI1 proxima1 to the tumor site at operation suggests that intermittent intussusception had occurred more often and may have been the cause of abdomina1 pain and other manifestations of transient incompIete intestina1 obstruction. One of four duodena1 adenomas and one of four Brunner’s gIand adenomas were identified as the source of bIeeding in this series. The particuIar Iesion in the Iatter group was soIitary and measured 3 by 1.3 by 1.5 cm. (Fig. 2.)
FIG. I. Brunner’s gIand adenoma of duodenum.
271
Schmutzer,
HoIIeran
and
Regan
revealed attack of acute abdomina1 distress, typical intussusception caused by a polypoid lesion. (Fig. 4.) A review of the famiIy history revealed two sisters with tumors of the smaI1 bowel and a brother with muItipIe poIyps of the coIon. One aunt died of carcinoma of the intestine. Neither the patient nor his brother and sisters had any evidence of meIanin spots on the mucous membranes. This patient may we11 represent an atypica1 case of Peutz-Jeghers syndrome without melanin spots.
FIG.
typica
2.
Lipoma of termina1 roentgenogram.
CASE II. Jejunal Leiomyoma. The patient was a forty-two year old man in whom melena deveIoped shortIy after a raiIroad accident. The bIeeding continued sIowIy over a two week period. RoentgenoIogic examination of the upper and Iower intestina1 tract reveaIed no abnormaIities. Upon expIoratory Iaparotomy a Iarge ieiomyoma was found. This tumor was Iocated in the pelvis. It had been overIooked on physical examination. If a bimanua1 rectoabdomina1 examination had been performed, this tumor would have been easiIy detected. (Fig. 5.)
iIeum;
In six cases there was an association with maIignant neopIasms of other organ systems. The complex probIems with respect to symptomatoIogy and diagnosis of benign tumors are Gstrated by the foIIowing case reports.
CASE III. Duodenal Leiomyoma. The patient, a fifty-two year oId woman, had severe gastrointestina1 bleeding. She was vomiting bright red bIood and passing tarry stooIs. The history was compatibIe with that of a peptic uIcer. An upper gastrointestina1 series reveaIed evidence suggestive of a duodena1 uIcer. After several bIood transfusions, expIoratory Iaparotomy was performed. A gastroduodenotomy was made. There was no evidence of an ulcer in either the stomach or the first portion of the duodenum. BIood was noted weIIing up from the second portion of the duodenum; ConsequentIy, the duodenotomy was enIarged, and upon intraIumina1 paIpation a tumor was feIt in the third portion of the duodenum.
CASE I. Jejunal Adenoma with Chronic Intermittent Intussusception. The patient, a forty-six year old man, had a five year history of intermittent severe upper abdomina1 cramps which usuaIIy occurred after a Iarge meaI. The pain ceased suddenly after the patient vomited. Three upper gastrointestina1 studies were made. In the first two instances, no Iesion was discovered by roentgenogram taken when the patient was free of pain. The third (Fig. 3), performed during an
4
3 FIG. 3. Roentgenogram FIG. 4. Photograph
of intussuscepting
adenoma of jejunum.
taken at surgery showing intussusception.
272
Tumors
FIG. 1’. Bleeding Upon rcmo\al Iciomyoma. TYPES
l&myoma
it \vas found
AND
SITES
of SmaIl
of jcjunum.
to be an ulcerating
OF MALIGNANT
FIG. 6. Adenocarcinoma tion of dur,dcnum.
TUMORS
AND
SYMPTOMS
OF MALIGNANT
of third
par-
Acute intestinal obstruction or intussusception was present in thirteen of forty-one cases. Of Iess diagnostic vaIue was an associated weight Ioss (more than 5 kg.) over a period of
Of the forty-one maIignant tumors in this series, the carcinoid was the most common type (sixteen cases), foIIowed in thirteen instances by carcinoma with varying degrees of I.) Lymphomas and differentiation. (TabIe Ieiomyosarcomas were found in seven and five cases, respectiveIy. The site of prediIection of malignant neopIasms was the iIeum with twenty-three cases. The duodenum and jejunum were Iess frequentIy invoIved. The number of tumors found at these two sites was aImost equal (duodenum eight cases, jejunum seven cases). There were five primary carcinomas of the duodenum in this series. AI1 were Iocated dista1 to the ampuIIa of Vater. In agreement with other reported series, carcinoid tumors were found most often in the iIeum. In seven cases an association with unreIated malignant Iesions in other organ systems was present. SIGNS
BoweI
one to two years
in eIeven
patients.
In the group of maIignant neoplasms, x-ray examinations of the intestina1 tract or scout fiIms of the abdomen in emergency situations were of diagnostic value in nineteen patients, representing sIightIy Iess than 50 per cent. Definitive roentgenoIogic manifestations were present in nine of thirteen cases of primary carcinoma of the smaI1 bowel. Nine of the sixteen carcinoid tumors were asymptomatic and found incidentaIIy at operation or at necropsy. Two of these patients exhibited the typcal carcinoid syndrome, but this was overIooked cIinicaIIy and onIy became evident in retrospect after postoperative re-evaIuation. CASE IV. Lymphosarcoma OJ Jejunum. This patient was admitted because of acute abdomina1 pain which Iocalized in the right Iower quadrant. Appendectomy was performed and a norma appendix removed. The abdomina1 cavity was not compIeteIy expIored at this time. The pain persisted. Two weeks Iater a partiaIIy obstructing, ulcerating Iymphosarcoma of the jejunum was discovered and removed.
TUMORS
As might be expected, malignant tumors of the smaI1 intestines give rise to more definitive and earIier signs and symptoms than the benign Iesions. The main symptom found was usualIy vague in nature, abdominal pain, which was present in fifteen cases. The most frequent causes for a more detaiIed investigation and examination of the patient were the presence of an abdominal mass and gastrointestina1 bleeding.
CASE v. Adenocarcinoma of tbe Third Portion of Duodenum. The patient, a forty-two year oId man, was admitted with intermittent epigastric pain unreIieved by food. He had passed tarry
273
Schmutzer,
FIG. 7.
PoIypoid adenocarcinoma
HoIIeran
of ileum.
stooIs two years prior to this admission. The hemoglobin on admission was 9.8 gm. per cent. The roentgenoIogic findings were a fiIling defect and loss of normal mucosal pattern. A WhippIe-type pancreatoduodenectomy was performed. (Fig. 6.) Adenocarcinoma of Ileum. CASE VI. Papillary The patient, a fifty-eight year oId man, had vague intermittent abdomina1 cramps. Repeated examinations of the stoo1 were positive for occult bIood. No abnorma1 findings were noted on x-ray examination. Pain became very severe. He was operated upon, and a papiIlary adenocarcinoma of the iIeum was found and removed. (Fig. 7.) COMMENTS
There is unanimous agreement by a11 authors who have written on the subject of tumors of the small bowel that neoplasms of this region are notorious for their vague symptomatoIogy and deIayed cIinica1 detection. By the same token, this type of tumor is rareIy incIuded in the differentia1 diagnosis in patients who present themseIves with symptoms of chronic, intermittent or acute abdominaI pain with or without melena or weight 10s~. ResponsibIe for this omission in the case of benign neopIasms seems to be the fact that these Iesions are reIativeIy uncommon [ 1,2] and that the majority of them usuaIIy do not cause any symptoms [3]. AIthough this appears to be true and is confirmed by our findings, it shouId be pointed out that after subtracting the twenty-two cases with ectopic pancreas 274
and
Regan
from the encountered benign tumors, fifteen of the remaining thirty-seven patients had A simiIar incidence of definite symptoms. significant cIinica1 symptoms and signs has been stated by Good in a recent pubIication [4]. RoentgenoIogic manifestations were present in tweIve cases. It is apparent from these figures, therefore, that a substantia1 number of benign Iesions of the smaI1 bowe1 do cause suflicient symptoms to warrant their clinica consideration in patients with iI defined abdominaI symptoms. With regard to the diagnostic vaIue of roentgenoIogic examination, it is encouraging to find that in tweIve of fifteen symptomatic patients positive resuIts were obtained. There shouId be IittIe doubt that this incidence of diagnostic finding couId be improved if cIinicians and radiologists were more “smaI1 [5]. Moreover, the clinician bowe1 conscious” shouId insist on a compIete and thorough examination of the smaI1 bowe1 by the radioIogist even if a lesion of the stomach or duodenum has been found. This may not be soIdy responsibIe for the patient’s symptomatoIogy. The infrequent occurrence of maIignant growths in the smaI1 intestinal tract has been [r,2,6-81 and the condocumented repeatedIy trast to the frequent IocaIization of maIignant tumors in the upper gastrointestinal tract and the Iarge bowe1 has led Harkins et al. [7] to epitomize the smaI1 bowe1 as a “rose between two thorns.” This euphemistic appraisa1 becomes reversed if one considers the obvious paucity of earIy signs and symptoms of such Iesions. It is noteworthy that five cases of estabIished intussusception were found in the group of maIignant tumors in contrast to 0nIy one case in the benign group and that there was no instance of perforation encountered in the whoIe series. In this connection Rochlin and Longmire’s [8] evaIuation of the duration of symptoms prior to surgica1 intervention in their series of patients deserves emphasis. Of the twenty-seven patients so evaIuated, fourteen had initia1 symptoms for six to tweIve months before diagnosis was established. With regard to the types and incidence of maIignant tumors in the smaI1 bowe1, carcinomas are found to be most frequent in many earIy reports. Our findings differ sIightIy in that carcinoid tumors (sixteen cases) were encountered more frequentIy than carcinomas (thirteen cases). Seven carcinoids were sympto-
Tumors
of SmaII
Bowel
bowe1 mesentery, obstructing a major tributary vein and encroaching upon one of the large intestinal arteries. There was complete excision of this mass. After removal of the tumor, the divided intestinal artcry and vein were reunited to prevent necrosis of any smaI1 bowel. The patient is still aIive and comfortabIe one year Iater. For years to come, tumors of the smaI1 intestina tract wiII remain a diagnostic chalIenge. This chaIIenge can onIy be met by a constant awareness of the physician towards the roIe which tumors of the smaI1 bowe1 may pIay in the various manifestations of abdomina1 disorders.
matic. The typica carcinoid syndrome was present in only two patients. None of these patients had been correctly diagnosed preoperatively. The finding of an unsuspected asymptomatic pheochromocytoma in one of the patients with symptoms reIated to a carcinoid tumor was a most unusua1 circumstance. In contradistinction to a11 other types of smaI1 bowel tumors in this study, with the exception c>fIeiomyosarcomas, none of the carcinomas was found unsuspectedIy at autopsy. AI1 thirteen carcinomas had caused definite symptoms or findings which Ied to operative intervention. In two cases, however, an emergency operation had been carried out under the presumptive diagnosis of acute appendicitis. In both instances a norma appendix was removed without further carefuI examination of the abdomina1 cavity. OnIy at subsequent re-expIoration one and two weeks Iater, respectiveIy, was the true nature of the iIIness in these two patients discovered and the patient treated accordingIy. These observations emphasize again the importance of a thorough, carefu1 expIoration and paIpatlon of the whoIe smaI1 bowe1 and other abdomina1 organs at every celiotomy, especiaIIy when an erroneous diagnosis has prompted the operation. A positive x-ray diagnosis was made in onIy nine of the thirteen symptomatic carcinomas. AIthough this appears to be a rather favorabIe incidence, it contrasts somewhat with the fact that of the fifteen symptomatic benign neopIasmas a positive x-ray diagnosis was made in tweIve instances. In view of the more pronounced symptomatoIogy of the carcinomas in this series, we had expected a higher incidence of diagnostic findings. Whether the absence of intraIumina1 projection in some of these tumors may account for this discrepancy cannot be decided from the data avaiIabIe to us. The operative treatment of these primary neopIasms is we11 estabIished and has been summarized in severa recent pubhcations [6,8]. AIthough metastatic tumors to the smaI1 bowe1 have not been incIuded in this study, a recent situation encountered at operation by one of us (J. F. R.) justifies mentioning, since it emphasizes the fact that certain metastatic Iesions to the smaI1 bowe1, seemingIy inoperabIe at first sight, may be amenabIe to Iong-term paIIiative treatment. In this particuIar instance a recurrent carcinoma of the iIeum had metastasized to the root of the smaI1
SUMMARY
A series of IOO cases of primary neopIasms of the smaI1 intestine encountered over a twenty year period (1944-1963) is reviewed. were benign (in&ding Fifty-nine tumors twenty-two cases of ectopic pancreas) and forty-one were mahgnant. As a resuIt of the inclusion of ectopic pancreata, the most common site of benign tumors was the duodenum, and the incidence of tumors diminished towards the ileoceca1 vaIve. Fifteen of the thirty-seven benign Iesions were symptomatic. In the group of forty-one maIignant tumors, the iIeum was the most frequent site of Iocaiization. Because of the Iarge number of carcinoids found incidentaIIy, onIy thirty-one neopIasms were symptomatic. Several brief case reports are incIuded to iIIustrate some of the compIexities which may confront the physician in the diagnosis and treatment of tumors of the smaI1 boweI. An improvement in the earIy recognition of tumors of the smaI1 bowe1 can only be achieved if the potentia1 presence of such neopIasms is given due consideration in the differentia1 diagnosis of abdomina1 disorders. REFERENCES I. RAIFORD, T.
S. Tumors of the smaI1 intestine. Arch.
Surg., 25: 122, 1932. 2. DARLING, R. C. and WELCH, C. E. Tumors of the smaI1 intestine. New England J. Med., 260: 397, 1959. 3. RIVER, L., SILVERSTEIN,J. and TOPE, J. W. Benign neoplasms of the smaI1 intestine. A critica comprehensive review with reports of 20 new cases. Internat. Abstr. Surg., 102: 1, 1956. 4. GOOD, C. A. Tumors of the smaII intestine. Am. J. Roentgenol., 89: 685, 1963.
275
Schmutzer,
HoIIeran
BOCKUS, H. L. GastroenteroIogy, voI. 2, p. 124. PhiIadeIuhia. 1044. W. B. Saunders Co. 6. LONGMIRE;W.‘P.; jk. and ADAMS,R. M. Malignant Iesions of the smaI1 intestine. J. Kansas M. Sec., (Cancer Supplement), 5 I : 32a, 1950. 7. HARKINS, H. N., MOYER, C. A., RHOADS, I. E. and ALLEN, I. G. Surgery, PrincipIes and Practice, 2nd ed., p. 959. PhiIadeIphia, 1961. J. B. Lippincott co. 8. ROCHLIN, D. B. and LONGMIRE,W. P., JR. Primary tumors of the smal1 intestine. Surgery, 50: 586, 1961.
and Regan
which treatment is most urgentIy needed, a mass is often paIpabIe. Two cases have been cited in which a mass shouId have been felt but was overIooked, obviously because it was not carefully sought. X-ray studies are frequentIy of great heIp, particuIarIy if, as pointed out by the authors, the cIinician asks for a speciaI smaI1 bowe1 study. Even so, some wiI1 not be demonstrated. Therefore, I think that when our cIinica1 judgment indicates it, abdomina1 expIoration, without a definite preoperative diagnosis, is stiI1 a worthwhiIe operation. MILLARD S. ROSENBLATT (PortIand, Ore.): The smaIIest tumor of the smal1 bowe1 I have ever removed was present in a twenty-two year old woman who had no symptoms until the tumor started to bIeed. She was eight and a haIf months pregnant, Iost the fetus as a resuIt of exsanguinating hemorrhage and aImost Iost her own life from this tumor, which was Iess than I cm. in its Iargest diameter. FREDERICK P. SHIDLER (Men10 Park, Calif.): We recentIy had one such patient who had symptoms of intermittent obstruction. As Dr. Sprong pointed out, it is wise to perform the smaI1 bowe1 x-ray examination during the periods of pain. We observed her many times without noting any evidence of obstruction unti1 pain recurred. FiIms at this time showed a so-caIIed coiled-spring appearance of the smaI1 intestine, which is not unlike that of intussusception, aIthough these fiIms did not actuaIIy show intussusception. To confirm our finding, since the x-ray findings were not altogether conclusive, roentgenograms were taken during an acute attack and the same findings were demonstrated again. A peduncuIated benign adenoma measuring 2 cm. in diameter was found which produced a very demonstrabIe intussusception at surgery. JAMES F. REGAN (cIosing): As mentioned before, we were trying to stress symtomatoIogy, not treatment. We have not mentioned the age, sex, incidence or duration of symptoms. These have occurred in the same ratio as that reported by others. Our foIIow-up data over this twenty year period is incompIete so we cannot teI1 you exactIy the outcome of the operation in a11 patients. However, I wouId Iike to re-emphasize the possibiIity of a smaI1 bowel tumor in a patient presenting repeated rather vague obstructive symptoms and bIeeding and the importance of an examination during the acute phase.
5.
DISCUSSION DAVID H. SPRONG, JR. (Santa Monica, CaIif.): One of the interesting features of tumors of the smaIl bowe1 is their infrequent occurrence. The smaII intestine makes up about 75 per cent of the Iength and over go per cent of the interna surface of the aIimentary tract, yet Iess than 5 per cent of the tumors are found there. The reason for this reIative immunity of the smaI1 intestine to the deveIopment of tumors is not known, aIthough several theories have been suggested to expIain it. The symptoms of smaI1 bowe1 tumors are reIated mainly to: (I) obstruction and (2) bIeeding. The obstruction is usuaIIy intermittent and often foIIowed by diarrhea. Bleeding can be massive, but is more commonIy insidious, the main symptom being weakness resuIting from secondary anemia. Since maIignant tumors grow more rapidIy and show symptoms earher, it is not surprising that they predominate in most surgica1 series reported. On the other hand, autopsy series show a preponderance of benign tumors. Differences in cIassification account for some of the variation in relative incidence reported in different series. One might question the incIusion of accessory pancreatic rests among the tumors aIthough the cIinica1 probIem is the same. In patients in whom smaI1 bowe1 tumors produce symptoms, a mass is paIpabIe in nearIy haIf of the malignancies and in about one fifth of the benign tumors. Evidence of bIeeding is frequent among maIignant tumors, but is much Iess common in the benign group. The treatment of smaI1 bowe1 tumors is surgica1 remova1; anything Iess is paIIiative. I beIieve the emphasis of this paper on diagnosis rather than treatment is we11 pIaced. The oId medica cIichC, “to make the diagnosis, you must first think of it appIies. In maIignant tumors, in as a possibiIity,”
276
of the Small Bowel*
KARL J. SCHMUTZER, M.D., WALTER M. HOLLERAN,M.D. AND JAMESF. REGAN, M.D.,
Los Angeles, California From tbe Department of Surgery, Queen of Angels Hospital, Los Angeles, California, and tbe Ross-Loos Medical Group, Los Angeles, California.
to review the pathoIogy and hospita1 records of the Queen of AngeIs Hospital, which is a 500 bed private hospital, covering the years 1944 through 1963. During this twenty year period, a tota number of IOO patients with benign or maIignant primary tumors and fourteen metastatic tumors to the smaI1 bowel were recorded. It is the purpose of this study to evaIuate the various types of neopIasms encountered and to discuss certain features of symptomatoIogy, diagnosis and treatment.
this uItramodern and sophisticated era of medicine, the earIy diagnosis of tumors of the smaI1 bowe1 stiI1 remains a cIinica1 probIem. Whereas neoplasms of the upper part of the arimentary tract and the coIon are not onIy more common in occurrence but aIso signa their presence earIier, tumors of the smaI1 bowe1 are often not considered in the differentia1 diagnosis of intestina1 diseases as a resuIt of their reIativeIy Iow incidence and the usua1 Iack of earIy distinctive cIinica1 manifestations. After we had encountered a number of interesting and unusua1 cIinica1 situations pertaining to tumors of the smaI1 intestine, we decided
I
N
TABLE HISTOLOGIC
TYPE
TYPES
Of the IOO cases of primary tumors of the smaI1 bowe1, fifty-nine were benign and fortyone were maIignant. In contrast to other reported series on this subject, there is a preponderance of benign tumors in this unseIected group of patients. However, if the twenty-two cases of ectopic pancreas are excIuded, an approximateIy equa1 number of benign and maIignant tumors is in evidence.
I AND
LOCATION
Location
-
- -
TYPO
J+J-
DUO-
Total.. . Malignant: Carcinoma. Lymphoma..
3 I
5 17 5 3 3
0 2
4 21
17
59
3 3 0 1
13 7 5 16
7 24
41 100
I
.. .. . .. . . 1
8
..
. .. . . .
rota1
9 I
Leiomyosarcoma.. Cm&mid. .
Totd. Sum TotaI..
TYPES
UIlpecified
denum
Benign: Adenoma (polyp) Leiomyoma.. . . . . Lipoms. . . . . . . . Hemangioma. Lymphangioma.. Brunner’s gland adenoma.. . . Ectopic pancreas..
40
OF TUMORS
SITES
OF BENIGN
TUMORS
The most commonIy encountered benign Iesion of the smaI1 intestine was ectopic pancreas with twenty-two cases. (TabIe I.) Next in frequency were Ieiomyomas with seventeen cases, foIIowed by adenomas (five), Iipomas (five) (Fig. I), Brunner’s gIand adenomas (four), hemangiomas (three) and Iymphangiomas (three). The duodenum was the most common site of benign tumors (thirty-two of fifty-nine) because of the reIativeIy Iarge number of ectopic pancreata. The jejunum was next in frequency with seventeen tumors, incIuding two cases of ectopic pancreas. OnIy eight tumors were Iocated in the iIeum. The specific
-
-
AND
* Presented at the annual meeting of the Pacific Coast Surgical Association, San Francisco, CaIifornia, February g-12, 1964. American
Journd
of Surgery.
Volume IO& August
rg64
270
Tumors
of Small BoweI TABLE
SYMPTOMS
-
II
AND
FINDIKGS
Symptoms Tumor Bkeding
Pain
_.--
and Findings
1
4nemia
Obstruction
Has
.._______
I ntussusception
Weight Loss
T 1
I ncidenta1 Finding
Y-ray FiIm
.I
Benign: Adenoma (poIyp) Leiomyoma. . Lipoma................... Hemangioma Lymphangioma. . . Brunner’s gIand adenoma. Ectopic pancreas.. TotaI. Malignant : c arcmoma. * Lymphoma. . Leiomyosarcoma. Carcinoid TotaI.
.
I I
0
I
0
2
2
0
I
I
I 2 I
0
0
0
I
4 4
0
0
0
0
0
0
0
0
0
0
0
0
0
0
0
I
0
0
0
0
0
3 I3 4 3 3 3
2
0
0
0
0
0
2
20
5
2
2
2
I
2
12
49
4
3
4
4
I
3 3 I
0
I
0
3 3
I
I
.
. .
0
3
II
15
-
II I
-
site could not be determined
AND
SYMPTOMS
OF BENIGN
9 4
I
0
2
0
2
2
3
4
9
8
5
II
19
-
-
-
I
IO
-
A Iarge Ieiomyoma was overIooked in two instances in the preoperative examination. Each of these masses wouId have been easiIy paIpabIe if a bimanua1 rectoabdomina1 examination had been performed. Roentgenologic manifestations were present 20 per cent, of in tweIve, or approximateIy these cases. Forty-nine of the benign tumors were found either incidentally at operation or at necropsy.
from the records in two instances. Nine of seventeen Ieiomyomas were Iocated in the jejunum. It is evident from these figures that there is a dechne in the incidence of benign tumors from the pylorus to the iIeoceca1 vaIve. SIGNS
I
4 4
0
I I
TUMORS
The most common symptom and sign associated with benign tumors were pain, usuaIIy after eating, and bleeding. (TabIe II.) These were present in ten cases. A mass was paIpated in two cases. Obstruction of the smaII bowe1 was present in two instances. In contrast to other reported series, intussusception was encountered in only one patient. However, the finding of a hypertrophied intestinal waI1 proxima1 to the tumor site at operation suggests that intermittent intussusception had occurred more often and may have been the cause of abdomina1 pain and other manifestations of transient incompIete intestina1 obstruction. One of four duodena1 adenomas and one of four Brunner’s gIand adenomas were identified as the source of bIeeding in this series. The particuIar Iesion in the Iatter group was soIitary and measured 3 by 1.3 by 1.5 cm. (Fig. 2.)
FIG. I. Brunner’s gIand adenoma of duodenum.
271
Schmutzer,
HoIIeran
and
Regan
revealed attack of acute abdomina1 distress, typical intussusception caused by a polypoid lesion. (Fig. 4.) A review of the famiIy history revealed two sisters with tumors of the smaI1 bowel and a brother with muItipIe poIyps of the coIon. One aunt died of carcinoma of the intestine. Neither the patient nor his brother and sisters had any evidence of meIanin spots on the mucous membranes. This patient may we11 represent an atypica1 case of Peutz-Jeghers syndrome without melanin spots.
FIG.
typica
2.
Lipoma of termina1 roentgenogram.
CASE II. Jejunal Leiomyoma. The patient was a forty-two year old man in whom melena deveIoped shortIy after a raiIroad accident. The bIeeding continued sIowIy over a two week period. RoentgenoIogic examination of the upper and Iower intestina1 tract reveaIed no abnormaIities. Upon expIoratory Iaparotomy a Iarge ieiomyoma was found. This tumor was Iocated in the pelvis. It had been overIooked on physical examination. If a bimanua1 rectoabdomina1 examination had been performed, this tumor would have been easiIy detected. (Fig. 5.)
iIeum;
In six cases there was an association with maIignant neopIasms of other organ systems. The complex probIems with respect to symptomatoIogy and diagnosis of benign tumors are Gstrated by the foIIowing case reports.
CASE III. Duodenal Leiomyoma. The patient, a fifty-two year oId woman, had severe gastrointestina1 bleeding. She was vomiting bright red bIood and passing tarry stooIs. The history was compatibIe with that of a peptic uIcer. An upper gastrointestina1 series reveaIed evidence suggestive of a duodena1 uIcer. After several bIood transfusions, expIoratory Iaparotomy was performed. A gastroduodenotomy was made. There was no evidence of an ulcer in either the stomach or the first portion of the duodenum. BIood was noted weIIing up from the second portion of the duodenum; ConsequentIy, the duodenotomy was enIarged, and upon intraIumina1 paIpation a tumor was feIt in the third portion of the duodenum.
CASE I. Jejunal Adenoma with Chronic Intermittent Intussusception. The patient, a forty-six year old man, had a five year history of intermittent severe upper abdomina1 cramps which usuaIIy occurred after a Iarge meaI. The pain ceased suddenly after the patient vomited. Three upper gastrointestina1 studies were made. In the first two instances, no Iesion was discovered by roentgenogram taken when the patient was free of pain. The third (Fig. 3), performed during an
4
3 FIG. 3. Roentgenogram FIG. 4. Photograph
of intussuscepting
adenoma of jejunum.
taken at surgery showing intussusception.
272
Tumors
FIG. 1’. Bleeding Upon rcmo\al Iciomyoma. TYPES
l&myoma
it \vas found
AND
SITES
of SmaIl
of jcjunum.
to be an ulcerating
OF MALIGNANT
FIG. 6. Adenocarcinoma tion of dur,dcnum.
TUMORS
AND
SYMPTOMS
OF MALIGNANT
of third
par-
Acute intestinal obstruction or intussusception was present in thirteen of forty-one cases. Of Iess diagnostic vaIue was an associated weight Ioss (more than 5 kg.) over a period of
Of the forty-one maIignant tumors in this series, the carcinoid was the most common type (sixteen cases), foIIowed in thirteen instances by carcinoma with varying degrees of I.) Lymphomas and differentiation. (TabIe Ieiomyosarcomas were found in seven and five cases, respectiveIy. The site of prediIection of malignant neopIasms was the iIeum with twenty-three cases. The duodenum and jejunum were Iess frequentIy invoIved. The number of tumors found at these two sites was aImost equal (duodenum eight cases, jejunum seven cases). There were five primary carcinomas of the duodenum in this series. AI1 were Iocated dista1 to the ampuIIa of Vater. In agreement with other reported series, carcinoid tumors were found most often in the iIeum. In seven cases an association with unreIated malignant Iesions in other organ systems was present. SIGNS
BoweI
one to two years
in eIeven
patients.
In the group of maIignant neoplasms, x-ray examinations of the intestina1 tract or scout fiIms of the abdomen in emergency situations were of diagnostic value in nineteen patients, representing sIightIy Iess than 50 per cent. Definitive roentgenoIogic manifestations were present in nine of thirteen cases of primary carcinoma of the smaI1 bowel. Nine of the sixteen carcinoid tumors were asymptomatic and found incidentaIIy at operation or at necropsy. Two of these patients exhibited the typcal carcinoid syndrome, but this was overIooked cIinicaIIy and onIy became evident in retrospect after postoperative re-evaIuation. CASE IV. Lymphosarcoma OJ Jejunum. This patient was admitted because of acute abdomina1 pain which Iocalized in the right Iower quadrant. Appendectomy was performed and a norma appendix removed. The abdomina1 cavity was not compIeteIy expIored at this time. The pain persisted. Two weeks Iater a partiaIIy obstructing, ulcerating Iymphosarcoma of the jejunum was discovered and removed.
TUMORS
As might be expected, malignant tumors of the smaI1 intestines give rise to more definitive and earIier signs and symptoms than the benign Iesions. The main symptom found was usualIy vague in nature, abdominal pain, which was present in fifteen cases. The most frequent causes for a more detaiIed investigation and examination of the patient were the presence of an abdominal mass and gastrointestina1 bleeding.
CASE v. Adenocarcinoma of tbe Third Portion of Duodenum. The patient, a forty-two year oId man, was admitted with intermittent epigastric pain unreIieved by food. He had passed tarry
273
Schmutzer,
FIG. 7.
PoIypoid adenocarcinoma
HoIIeran
of ileum.
stooIs two years prior to this admission. The hemoglobin on admission was 9.8 gm. per cent. The roentgenoIogic findings were a fiIling defect and loss of normal mucosal pattern. A WhippIe-type pancreatoduodenectomy was performed. (Fig. 6.) Adenocarcinoma of Ileum. CASE VI. Papillary The patient, a fifty-eight year oId man, had vague intermittent abdomina1 cramps. Repeated examinations of the stoo1 were positive for occult bIood. No abnorma1 findings were noted on x-ray examination. Pain became very severe. He was operated upon, and a papiIlary adenocarcinoma of the iIeum was found and removed. (Fig. 7.) COMMENTS
There is unanimous agreement by a11 authors who have written on the subject of tumors of the small bowel that neoplasms of this region are notorious for their vague symptomatoIogy and deIayed cIinica1 detection. By the same token, this type of tumor is rareIy incIuded in the differentia1 diagnosis in patients who present themseIves with symptoms of chronic, intermittent or acute abdominaI pain with or without melena or weight 10s~. ResponsibIe for this omission in the case of benign neopIasms seems to be the fact that these Iesions are reIativeIy uncommon [ 1,2] and that the majority of them usuaIIy do not cause any symptoms [3]. AIthough this appears to be true and is confirmed by our findings, it shouId be pointed out that after subtracting the twenty-two cases with ectopic pancreas 274
and
Regan
from the encountered benign tumors, fifteen of the remaining thirty-seven patients had A simiIar incidence of definite symptoms. significant cIinica1 symptoms and signs has been stated by Good in a recent pubIication [4]. RoentgenoIogic manifestations were present in tweIve cases. It is apparent from these figures, therefore, that a substantia1 number of benign Iesions of the smaI1 bowe1 do cause suflicient symptoms to warrant their clinica consideration in patients with iI defined abdominaI symptoms. With regard to the diagnostic vaIue of roentgenoIogic examination, it is encouraging to find that in tweIve of fifteen symptomatic patients positive resuIts were obtained. There shouId be IittIe doubt that this incidence of diagnostic finding couId be improved if cIinicians and radiologists were more “smaI1 [5]. Moreover, the clinician bowe1 conscious” shouId insist on a compIete and thorough examination of the smaI1 bowe1 by the radioIogist even if a lesion of the stomach or duodenum has been found. This may not be soIdy responsibIe for the patient’s symptomatoIogy. The infrequent occurrence of maIignant growths in the smaI1 intestinal tract has been [r,2,6-81 and the condocumented repeatedIy trast to the frequent IocaIization of maIignant tumors in the upper gastrointestinal tract and the Iarge bowe1 has led Harkins et al. [7] to epitomize the smaI1 bowe1 as a “rose between two thorns.” This euphemistic appraisa1 becomes reversed if one considers the obvious paucity of earIy signs and symptoms of such Iesions. It is noteworthy that five cases of estabIished intussusception were found in the group of maIignant tumors in contrast to 0nIy one case in the benign group and that there was no instance of perforation encountered in the whoIe series. In this connection Rochlin and Longmire’s [8] evaIuation of the duration of symptoms prior to surgica1 intervention in their series of patients deserves emphasis. Of the twenty-seven patients so evaIuated, fourteen had initia1 symptoms for six to tweIve months before diagnosis was established. With regard to the types and incidence of maIignant tumors in the smaI1 bowe1, carcinomas are found to be most frequent in many earIy reports. Our findings differ sIightIy in that carcinoid tumors (sixteen cases) were encountered more frequentIy than carcinomas (thirteen cases). Seven carcinoids were sympto-
Tumors
of SmaII
Bowel
bowe1 mesentery, obstructing a major tributary vein and encroaching upon one of the large intestinal arteries. There was complete excision of this mass. After removal of the tumor, the divided intestinal artcry and vein were reunited to prevent necrosis of any smaI1 bowel. The patient is still aIive and comfortabIe one year Iater. For years to come, tumors of the smaI1 intestina tract wiII remain a diagnostic chalIenge. This chaIIenge can onIy be met by a constant awareness of the physician towards the roIe which tumors of the smaI1 bowe1 may pIay in the various manifestations of abdomina1 disorders.
matic. The typica carcinoid syndrome was present in only two patients. None of these patients had been correctly diagnosed preoperatively. The finding of an unsuspected asymptomatic pheochromocytoma in one of the patients with symptoms reIated to a carcinoid tumor was a most unusua1 circumstance. In contradistinction to a11 other types of smaI1 bowel tumors in this study, with the exception c>fIeiomyosarcomas, none of the carcinomas was found unsuspectedIy at autopsy. AI1 thirteen carcinomas had caused definite symptoms or findings which Ied to operative intervention. In two cases, however, an emergency operation had been carried out under the presumptive diagnosis of acute appendicitis. In both instances a norma appendix was removed without further carefuI examination of the abdomina1 cavity. OnIy at subsequent re-expIoration one and two weeks Iater, respectiveIy, was the true nature of the iIIness in these two patients discovered and the patient treated accordingIy. These observations emphasize again the importance of a thorough, carefu1 expIoration and paIpatlon of the whoIe smaI1 bowe1 and other abdomina1 organs at every celiotomy, especiaIIy when an erroneous diagnosis has prompted the operation. A positive x-ray diagnosis was made in onIy nine of the thirteen symptomatic carcinomas. AIthough this appears to be a rather favorabIe incidence, it contrasts somewhat with the fact that of the fifteen symptomatic benign neopIasmas a positive x-ray diagnosis was made in tweIve instances. In view of the more pronounced symptomatoIogy of the carcinomas in this series, we had expected a higher incidence of diagnostic findings. Whether the absence of intraIumina1 projection in some of these tumors may account for this discrepancy cannot be decided from the data avaiIabIe to us. The operative treatment of these primary neopIasms is we11 estabIished and has been summarized in severa recent pubhcations [6,8]. AIthough metastatic tumors to the smaI1 bowe1 have not been incIuded in this study, a recent situation encountered at operation by one of us (J. F. R.) justifies mentioning, since it emphasizes the fact that certain metastatic Iesions to the smaI1 bowe1, seemingIy inoperabIe at first sight, may be amenabIe to Iong-term paIIiative treatment. In this particuIar instance a recurrent carcinoma of the iIeum had metastasized to the root of the smaI1
SUMMARY
A series of IOO cases of primary neopIasms of the smaI1 intestine encountered over a twenty year period (1944-1963) is reviewed. were benign (in&ding Fifty-nine tumors twenty-two cases of ectopic pancreas) and forty-one were mahgnant. As a resuIt of the inclusion of ectopic pancreata, the most common site of benign tumors was the duodenum, and the incidence of tumors diminished towards the ileoceca1 vaIve. Fifteen of the thirty-seven benign Iesions were symptomatic. In the group of forty-one maIignant tumors, the iIeum was the most frequent site of Iocaiization. Because of the Iarge number of carcinoids found incidentaIIy, onIy thirty-one neopIasms were symptomatic. Several brief case reports are incIuded to iIIustrate some of the compIexities which may confront the physician in the diagnosis and treatment of tumors of the smaI1 boweI. An improvement in the earIy recognition of tumors of the smaI1 bowe1 can only be achieved if the potentia1 presence of such neopIasms is given due consideration in the differentia1 diagnosis of abdomina1 disorders. REFERENCES I. RAIFORD, T.
S. Tumors of the smaI1 intestine. Arch.
Surg., 25: 122, 1932. 2. DARLING, R. C. and WELCH, C. E. Tumors of the smaI1 intestine. New England J. Med., 260: 397, 1959. 3. RIVER, L., SILVERSTEIN,J. and TOPE, J. W. Benign neoplasms of the smaI1 intestine. A critica comprehensive review with reports of 20 new cases. Internat. Abstr. Surg., 102: 1, 1956. 4. GOOD, C. A. Tumors of the smaII intestine. Am. J. Roentgenol., 89: 685, 1963.
275
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HoIIeran
BOCKUS, H. L. GastroenteroIogy, voI. 2, p. 124. PhiIadeIuhia. 1044. W. B. Saunders Co. 6. LONGMIRE;W.‘P.; jk. and ADAMS,R. M. Malignant Iesions of the smaI1 intestine. J. Kansas M. Sec., (Cancer Supplement), 5 I : 32a, 1950. 7. HARKINS, H. N., MOYER, C. A., RHOADS, I. E. and ALLEN, I. G. Surgery, PrincipIes and Practice, 2nd ed., p. 959. PhiIadeIphia, 1961. J. B. Lippincott co. 8. ROCHLIN, D. B. and LONGMIRE,W. P., JR. Primary tumors of the smal1 intestine. Surgery, 50: 586, 1961.
and Regan
which treatment is most urgentIy needed, a mass is often paIpabIe. Two cases have been cited in which a mass shouId have been felt but was overIooked, obviously because it was not carefully sought. X-ray studies are frequentIy of great heIp, particuIarIy if, as pointed out by the authors, the cIinician asks for a speciaI smaI1 bowe1 study. Even so, some wiI1 not be demonstrated. Therefore, I think that when our cIinica1 judgment indicates it, abdomina1 expIoration, without a definite preoperative diagnosis, is stiI1 a worthwhiIe operation. MILLARD S. ROSENBLATT (PortIand, Ore.): The smaIIest tumor of the smal1 bowe1 I have ever removed was present in a twenty-two year old woman who had no symptoms until the tumor started to bIeed. She was eight and a haIf months pregnant, Iost the fetus as a resuIt of exsanguinating hemorrhage and aImost Iost her own life from this tumor, which was Iess than I cm. in its Iargest diameter. FREDERICK P. SHIDLER (Men10 Park, Calif.): We recentIy had one such patient who had symptoms of intermittent obstruction. As Dr. Sprong pointed out, it is wise to perform the smaI1 bowe1 x-ray examination during the periods of pain. We observed her many times without noting any evidence of obstruction unti1 pain recurred. FiIms at this time showed a so-caIIed coiled-spring appearance of the smaI1 intestine, which is not unlike that of intussusception, aIthough these fiIms did not actuaIIy show intussusception. To confirm our finding, since the x-ray findings were not altogether conclusive, roentgenograms were taken during an acute attack and the same findings were demonstrated again. A peduncuIated benign adenoma measuring 2 cm. in diameter was found which produced a very demonstrabIe intussusception at surgery. JAMES F. REGAN (cIosing): As mentioned before, we were trying to stress symtomatoIogy, not treatment. We have not mentioned the age, sex, incidence or duration of symptoms. These have occurred in the same ratio as that reported by others. Our foIIow-up data over this twenty year period is incompIete so we cannot teI1 you exactIy the outcome of the operation in a11 patients. However, I wouId Iike to re-emphasize the possibiIity of a smaI1 bowel tumor in a patient presenting repeated rather vague obstructive symptoms and bIeeding and the importance of an examination during the acute phase.
5.
DISCUSSION DAVID H. SPRONG, JR. (Santa Monica, CaIif.): One of the interesting features of tumors of the smaIl bowe1 is their infrequent occurrence. The smaII intestine makes up about 75 per cent of the Iength and over go per cent of the interna surface of the aIimentary tract, yet Iess than 5 per cent of the tumors are found there. The reason for this reIative immunity of the smaI1 intestine to the deveIopment of tumors is not known, aIthough several theories have been suggested to expIain it. The symptoms of smaI1 bowe1 tumors are reIated mainly to: (I) obstruction and (2) bIeeding. The obstruction is usuaIIy intermittent and often foIIowed by diarrhea. Bleeding can be massive, but is more commonIy insidious, the main symptom being weakness resuIting from secondary anemia. Since maIignant tumors grow more rapidIy and show symptoms earher, it is not surprising that they predominate in most surgica1 series reported. On the other hand, autopsy series show a preponderance of benign tumors. Differences in cIassification account for some of the variation in relative incidence reported in different series. One might question the incIusion of accessory pancreatic rests among the tumors aIthough the cIinica1 probIem is the same. In patients in whom smaI1 bowe1 tumors produce symptoms, a mass is paIpabIe in nearIy haIf of the malignancies and in about one fifth of the benign tumors. Evidence of bIeeding is frequent among maIignant tumors, but is much Iess common in the benign group. The treatment of smaI1 bowe1 tumors is surgica1 remova1; anything Iess is paIIiative. I beIieve the emphasis of this paper on diagnosis rather than treatment is we11 pIaced. The oId medica cIichC, “to make the diagnosis, you must first think of it appIies. In maIignant tumors, in as a possibiIity,”
276