Neoplasms of the small intestine

Neoplasms

of the Small Intestine

WILLIAM SHIBLEY ELIAS M.D., CHARLES C. LUND, M.D., Diplomate, American AND ROBERT YONEMOTO, M.D.,Boston, Massachusetts From tbe Fiftb Surgical Service, Boston City Hospital, Boston, Massachusetts.

Board

of Surgery

entire gastrointestinal tract. Mayd13 reported 0.029 per cent for this autopsy ratio while Nothnage14 found an incidence of 0.051 per cent. In 1930 Rankin and Mayo5 reported that 0.62 per cent of a11 gastrointestinal malignancies were in the smaI1 intestine. LikeIy6 reported an autopsy incidence of 2.3 per cent. In 1932 Raiford’ reported 4.9 per cent for this ratio and Forgue and Chauvin8 reported 6 per cent. Up to 1943 Iess than 300 surgical and autopsy specimens of carcinoma of the smaI1 bowe1 had been reported. Raifordr in 1932 reported eighty-eight cases of tumors of the smaI1 intestine among I 1,500 genera1 autopsy records and 45,000 surgica1 specimens from the Johns Hopkins HospitaI. In 1940 Buckstein found sixty-nine cases in 22,810 autopsies at BeIIevue HospitaI. Shallow, Eger and Carthy,iO reviewing the Iiterature in 1945, coIIected a total of 137, I 74 genera1 autopsies. Among these there were 4,034 carcinomas of the large bowe1, an incidence of 3.6 per cent, and I 34 carcinomas of the smaI1 bowe1 or 0.098 per cent, but 2.4 per cent of carcinoma of the intestine. From this we can conclude that for every case of smaI1 bowe1 carcinoma there are approximately forty cases involving the Iarge bowe1. Age and Sex Incidence. The youngest reported case is that of a three and a haIf year old chiId. GeneraIIy the maIes are invoIved two and a haIf times as often as the females and the average age is approximately fifty years. Signs and Symptoms. There is no characteristic symptom complex which points definiteIy or with any degree of certainty to either benign or malignant tumors of the smaI1 intestine. GeneraIIy the symptoms are those of either obstruction or bleeding into the alimentary tract. Often the clinical picture is that of secondary anemia but some are diagnosed as cases of pernicious anemia and treated unsuccessfuIIy as such. The symptom complex has severa features in common with that of carcinoma of the large intestine, yet there are certain differences. These are more or less inde-

RECENT case of intestina1 obstruction due to intussusception, secondary to hemangioma of the jejunum, has Ied us to review the Iiterature and the cIinica1 and pathoIogic records of patients with tumors of the smaI1 intestine at the Boston City Hospital from 1933 through 1932. This study has been Iimited to those patients whose diagnoses were found in the index fiIe at the MaIIory Institute of Pathology. The hospita1 record room has no index fiIe of diagnoses during most of these years, so that patients seen on the ward and diagnosed either at operation or otherwise are not included unIess a specimen was examined. In spite of the generally accepted beIief that neopIasms of the small intestine are quite rare, there have been eighty-nine microscopicaIIy proved cases in the twenty-year period previousIy mentioned. Sixty-two of these were autopsy specimens, the vast majority of these patients did not manifest the nature of their disease cIinicaIIy, but died of incidental causes. Twenty-seven patients had enough symptoms to be subjected to ceIiotomy and primary resection. During the past few years the specia1 interest of Schatzki,‘* GoIdenzl and others has made US “smah bowe1 conscious.” Because of the better understanding of the cIinica1 manifestations and x-ray findings the diagnosis of such tumors shouId be considered whenever puzzIing gastrointestina1 pain, bIeeding or obstruction occurs. Such Iesions are frequentIy curabIe when the diagnosis is made and the operation performed before the obstruction or bleeding has become termina1. Incidence. The first articIe on carcinoma of the jejunum was written in 1824 by Sor1in.l The first autopsy series of maIignant Iesions of the smal1 intestine was reported by Leichtenstern2 in 1876. They made up approximateIy 0.093 per cent of the maIignant Iesions of the

A

384

Neoplasms

of SmaII Intestine differentiation is now extremeIy high. Weber and Kirklin” reported correct diagnoses in 94 per cent of duodena1 neopIasms, 85 per cent of jejuna1 neoplasms and 67 per cent in the iIeum in a series of sixty-two smaI1 intestine tumors. GeneraIIy speaking three separate and distinct methods are avaiIabIe to the roentgenologist in the study of these tumors. The first is the smaI1 intestine enema as described by Schatzki.12 This consists of passing a Levin tube into the second portion of the duodenum and fihing the smaI1 intestine with a barium suspension which is allowed to run through SIOWIY. The second method is a modified smaI1 intestine enema. A MiIIer-Abbott tube is passed into the smaI1 intestine as a therapeutic measure and the patient is decompressed. A smaI1 amount of thin or diluted barium suspension is then introduced just proxima1 to the obstruction and fIuoroscopicaIIy examined. The third method is caIIed a motility series. Thirty grams of barium suIfate are given orally at 8 A.M. and films are taken one-half hour Iater and repeated at haIf-hour intervaIs. These seria1 fiIms are examined immediateIy and the patient is Auoroscoped and spot films are taken as indicated. When a11 of the small intestine has been we11 visualized and the head of the coIumn has reached the cecum, the examination is terminated. Benign Iesions of the smal1 intestine are diffrcuIt to diagnose before any obstruction has occurred clinically. Large benign lesions invoIving one portion of the thin-waIIed structure may be hidden by the flexibility and norma appearance of the uninvoIved circumference. With a non-obstructive intraIumina1 lesion, subtraction of the normal Iumen may be expected. In the duodenum this is easy to find but in the jejunum and iIeum it must be activeIy sought. ExtraIuminaI growths are aIso diffrcuIt to diagnose earIy and usuahy can be diagnosed roentgenoIogicaIIy only after they have reached sufficient size to cause obstruction by pressing against the waI1 of the intestine, manifesting a Iarge intraIumina1 radioIucent defect. In the presence of maIignant tumors various degrees of fiIIing defects are demonstrated. UsuaIIy, this is of an annuIar constriction. Pathology. There is some difference of opinion as to the reIative frequency of the two principat groups of maIignant tumors of

pendent of the site of the Iesion and may be either acute or chronic in onset and may be present for variabIe periods of time. One of the most characteristic chnical features is that of intermittent obstruction. With high tumors vomiting occurs quite earIy. The vomitus usuaIIy contains blood, free hydrochIoric acid and gastric enzymes. Massive hemorrhage is not frequent aIthough the stool usuaIIy contains occuIt bIood. The more proxima1 the tumor, the earlier the gastrointestinal symptoms occur. Changes in bowe1 habits are common, occurring usually as increasing constipation though not infrequentIy as alternating bouts of constipation and diarrhea. Often these take the form of recurring attacks of intestina1 obstruction with severe abdomina1 cramps in the periumbiIica1 and suprapubic regions, accompanied by nausea and vomiting. Weeks or months may pass between each of these attacks with periods of quiescence. Pain is common and is usuaIIy cramp-like during these periods of obstruction. Loss of weight is another common feature. Pridgen et a1.24 from the Mayo CIinic reported that 70 per cent of patients with jejuna1 Iesions demonstrated some degree of either partia1 or compIete obstruction of the smaI1 bowel. They report a paIpabIe mass in 41 per cent of al1 cases in the jejunum and forty-eight per cent of cases in the ileum. The Iocation of the mass in the jejunum was usuaIIy in the Ieft Iower quadrant while those in the iIeum were predominantly in the right Iower quadrant. VisibIe peristaIsis was present in approximately 30 per cent of these obstructive Iesions; borborygmus was present in approximateIy 50 per cent. About 25 per cent of the patients with smaI1 bowel tumors compIained of passing excessive Aatus or belching gas. X-Ray Findings. In order to establish the diagnosis of smaI1 intestine tumors a special smal1 intestine x-ray examination is necessary; but, of course, no barium should be given by mouth if complete or partial obstruction is present. As a prerequisite, scout films with the patient in both the upright and the recumbent position are important. Every patient with a clinical picture of gastrointestina1 obstruction or bIeeding which cannot be explained on the basis of a pathoIogic condition found in either the esophagus, stomach or the intestine should have a smaI1 intestine roentgenologic examination. In the hands of a competent roentgenoIogist the accuracy of both Iocalization and 385

NeopIasms of SmaIl Intestine the small intestine, carcinoma and sarcoma. Fraser,13 Mayo, lg Cameron’4 and others report that carcinoma was only sIightIy more frequent. This is a very different situation from that of the large intestine or the stomach where carcinomas make up over 93 per cent of the cases. Lymphosarcoma has been the most frequently reported type of sarcoma invoIving the smaI1 intestine. Carcinoma. The invoIved portion of smaI1 bowe1 is usuaIIy quite short, rigid and often fixed. ProximaI to this lesion the intestine is frequently distended. At ceIiotomy the carcinoma is usuaIIy found to have encircIed the bowe1 compIeteIy in an annular or napkin-ring type of tumor and often invoIves the small intestine for a reIativeIy short distance of I or 2 inches; frequently this invoIvement may be simpIy a fraction of that area. By the time the tumor has been expIored at ceIiotomy it has usuaIIy brought about compIete or nearIy compIete occIusion of the smaI1 bowe1 with subsequent intestina1 obstruction. The Iate appearance of these cIinica1 features is accounted for by the fluid nature of the bowe1 contents in the smaI1 intestine and, therefore, many of these patients first present themseIves as cases of chronic or acute intestina1 obstruction of unknown etioIogy. As a consequence of this gradua1 obstruction, hypertrophy and diIation of the proxima1 loop is a frequent finding at ceIiotomy. OccasionalIy the carcinoma presents itseIf cIinicaIIy as an intussusception of the smaI1 bowel which at operation is not compIeteIy reducibIe. However, intussusception is more frequentIy an occurrence in poIypoida1 types of tumors. In spite of the fact that the intestina1 symptoms deveIop reIativeIy Iate, secondary metastasis occurs quite earIy. This is probabIy due to the abundance of the Iymphatic drainage and the vascular suppIy aIong with the absorptive functions of the ima intestine. Metastases occur to the reIated Iymph nodes and peritoneum, the Iiver, Iungs, Iong bones and to the dura in that order of frequency. In 1953 Ebert et aI. reported from the Boston City Hospital on tumors of the duodenum with detaiIed case reports of two recent operations for carcinoma of the duodenum. Some of their cases are aIso incIuded in this report. Treatment. The treatment of choice for any tumor or type of maIignancy in the smaI1 intestine is resection of the afnicted Ioop of bowe1

aIong with a wide wedge of related mesentery and contained Iymphatics. The continuity of the smaI1 intestine is re-estabIished preferabIy by end-to-end anastomosis. Because of its anatomic relationship the duodenum, however, presents a much more complicated surgical probIem than neopIasms of the ileum or jejunum. If the neopIasm manifests itseIf as acute intestinal obstruction, it is essentia1 that these patients be decompressed to a maximum degree before operation. Their fluid and elecanemia and vitamin and troIyte balance, pIasma protein defidiencies shouId be improved as much as possibIe preoperatively. Prognosis. Prognosis is good in cases of carcinoids and poor in cases of carcinoma and sarcoma. In 1947 Botsford and Seibe118 reported an average surviva1 of eleven and a haIf months among eighteen cases of carcinoma and six months among thirteen cases of sarcoma. In Cameron’s14 series in 1937 the average duration of Iife was 17.6 months foIIowing the resection. In Mayo and Nettrour’slg thirty-one cases of jejuna1 carcinoma reported in 1937 two patients survived for a period of seven a patient years. In 1932 WakeIyZo reported living and we11 eight years after resection of the termina1 ileum for adenocarcinoma. This was unexpectedIy found whiIe expIoring the abdomen for an umbiIica1 hernia. The resuIts of surgery for smaI1 intestinal maIignancy compare quite favorabIy with those of the Iarge bowe1. Since the maIignancies of the smaI1 intestine metastasize quite early and since the diagnosis is deIayed because of the reIative absence of symptomatoIogy unti1 late in the stage of the disease, it is usuaIIy beyond the point of operabiIity when expIored. Carcinoma of the smaI1 intestine seems to have a better prognosis than that of sarcoma. Because of the anatomic location and reIationship of the duodenum, primary malignancy in the duodenum is Iess favorabIe than that of the jejunum or ileum. PATHOLOGIC

MATERIAL

STUDIED

After reviewing the clinical and pathoIogic records of the proved neoplasms of the smaI1 intestine it was decided to present the data concerning the cases found incidentaIIy at autopsy separately from the rest of the cases. There were sixty-two in the group of those found at autopsy. TabIe I shows the Iocations in the intestine of the autopsy patients and shows __

Neoplasms

of SmaII Intestine the numbers are too small. It is of great importance, however, to notice that ten patients, or more than one-third, are under the age of forty. TabIe IV presents the data as to the Iocation of the tumor and the type of matignancy in

the different kinds of maIignant tumors found in these locations. Table II presents the same type of data for the benign lesions found at autopsy. It is interesting to note that practicaIIy three-fourths of these lesions found at autopsy were in the benign group, indicating TABLE I LOCATION OF MALIGNANT TUMORS IN AUTOPSY CASES

-

DUOdenum

Adenocarcinoma. Leiomyosarcoma. Lymphosarcoma. Malignant adenoma. Melanosarcoma. ReticuIum ceII sarcoma.

-

Jejunum

]ileum

I

-

rota1

1 o o o

Total.

Duodenum

II

8

_~

TABLE IV LOCATION OF MALIGNANT TUMORS IN OPERATIVE

-

Adenocarcinoma. Leiomyosarcoma Lymphosarcoma Mabgnant adenoma. Melanosarcoma. Reticulum cell sarcoma

2 I 0 0

o

2

9

16

-

--

Total.

Jejunum

CASES

Ileum

Total

3 0 0 0 0 0

9 3 0

I I 2 -_ 16

3

-

-

TABLE II LOCATIONOF BENIGN

-

TUMORS

Duodenum

Adenoma. Fibroma. Ganglioneuroma. Hemangioma. Leiomyoma. Lipoma. Lymphangioma. Neurofibroma. Polyp.

IN AUTOPSY

Jejunum

1lleum

2 2 2

4 4 0

2

IO

4 0 0 0

3 0

I9

16

-__

II

2 2

0 0

Adenoma

II 0

0

I

0

0

_.

-

46

Total.

IN OPERATIVE

,Jenum

Jejunum

0 0 0

2 0 0

0 0 0 0 0 0

3 0 I 0 0 0

0

6

.

F’b I roma. Ganglioneuroma Hemangioma. Leiomyoma. Lipoma. Lymphangioma Neurofibroma.. Polyp.

3 7 20

I

v

TUMORS

Duo-

-

-

OF BENIGN

Total

2

0

TotaI

LOCATION

T

0 0 0 2

I

TABLE

CASES

-

-__

I.leum

CASES

I

Total

I-

I-

TABLE III AGE

AND

Females(r4).

I

o

Males(r3)....

I

Age.

o--10,ok

SEX

I

DISTRIBUTION

z

z

0

0

the operative cases. There were sixteen patients in the group, of whom more than half had adenocarcinoma. Exactly half of a11 the patients had the lesion Iocated in the jejunum. TabIe v presents simiIar data concerning benign tumors in operative cases. Here there was no preponderant type but it is interesting that no Iesions were found in the duodenum of eIeven in this group. It can be seen that in the patients with Iesions found at operation 6g per cent had mahgnant diseases compared to 26 per cent of malignant disease found at autopsy. Unfortunately, the cIinica1 records of six cases in 1933 were unsatisfactory for further

3

4 I 5 0 ~0430 30-40 40-w so>60 f&-,0 70-80

that when a patient has a symptomIess tumor in the intestine it is most IikeIy to be benign. The data found by further study of this rather misceIIaneous group of patients were found to be reIativeIy insignificant. Operative Cases. There were twenty-seven patients in this group. TabIe III shows their age and sex distribution. In this tabIe the maIignant and non-maIignant are both included and are not shown separateIy because 387

Neoplasms

of SmalI Intestine biopsy for a patient with hemangioma of the smaI1 intestine. Three of the patients died poatoperativeIy. One had malignant adenoma of the ileum and died of a subdiaphragmatic abscess. The second postoperative death was a patient with adeno-

study. Therefore the data on symptoms were tabulated from the remaining twenty-one. Symptoms. As seen in Figure I, onIy eight of twenty-one patients complained of preoperative constipation in spite of the fact that there was intestina1 obstruction that was inCases

q case

8cases I’6

Cases

r -I II11Cases

3 cases

I I C

7Cases

Cases

+Case5

.- Pai n Jantipa Palpable

FIG.I.Symptomatology

RristalsIs

of twenty-one

5 Cases

QCases

cases with adequate records.

carcinoma of the duodenum which was resected. A jejunal fistula and peritonitis deveIoped. Both of these deaths occurred in 1938, in the pre-antibiotic era. The third death, which occurred in 1947, was that of a patient who had reticuIum ceI1 sarcoma of the ileum and who died after an extreme reaction secondary to intravenous protein hydroIysate feeding. The remaining eighteen patients recovered without major complication and Ieft the hospita1 in good condition.

complete in eIeven patients and compIete in five additiona cases. Nineteen of twenty-one patients complained of acute pain, eighteen of nausea and sixteen of vomiting. There was a definiteIy related loss of weight in six cases. We beIieve that an outstanding cIinicaI manifestation is the mass paIpabIe in eIeven, or over 40 per cent of the cases. In 33 per cent there was visibIe peristalsis. Again over 30 per cent had definite meIena aIthough the red bIood ceIIs and hematocrit were not significantly depressed except in three cases. Because our Iast clinica case presented as an intussusception, we were especiaIly on the lookout for this in the previous cases but couId find a total of onIy four cases or less than 20 per cent. Treatment. Twenty-one operations were performed, of which twenty consisted of resection with primary anastomosis and one was a

SUMMARY

Delay in the diagnosis and treatment of smaI1 bowe1 tumors occurs quite frequentIy and is due to the fact that the cIinica1 manifestations occur Iate in the disease because of the Iiquid character of the bowe1 contents in 388

NeopIasms

of SmaII

g. BUCKSTEIN, J. Clinical Roentgenology of the Alimentary Tract. PhiIadeIphia, 1940. W. B. Saunders Co. IO. SHALLOW, T. A., EGER, S. A. and CARTHY, J. B. Primary maIignant disease of the smaI1 intestines. Am. J.-Surg.;69: 372-383, 1945. I I. WEBER, A. M. and KIRKLIN, B. R. Roentgenologic manifestations of tumors of the smaI1 intestine. Am. J. Roentgen& 47: 243-253, 1942. 12. SCHATZKI, R. SmaII bowel enema. Am. J. Roentgenol., so: 743-750. 1943. 13. FRAZER. K. MaIisnant tumors of the small intestine. Brit. J. Surg., 32: 479-491. 1945. 14. CAMERON, A. L. Primary malignancy of the jejunum and ileum. Ann. Surg., 108: 203-220.

the small bowel. Often the patient is presented as a case of intestinal obstruction or intussusception, the obstruction often being of an intermittent type. In the presence of anemia of gastrointestina1 origin in which the stomach and large boweI have been thoroughIy investigated, a radioIogic examination of the smaI1 boweI is indicated. Twenty-seven cases have been recorded, with three deaths. In spite of the fact that many patients wiIl probabIy continue to come to surgery Iate in the course of the disease, a very small immediate mortality should be encountered in future operations of this nature.

1938. 15. CHEEVER, D. Tumors of the smal1 intestine. Ann. SUrg., 96: 911920, 1933. 16. FAULKNER, J. WI and DOCKERTY, M. B. Lymphosarcoma of the smaI1 intestine. Surs.. Gvnec. ~7 Obst., 95: 7684, rggz. 17. FITZGERALD, P. J. and PEARSON, C. Carcinoids of the small intestine. Cancer, 2: 1045-1060, 1948. 18. BOTSFORD. T. W. and SEIBEL. R. E. Benign and malignant tumors of the small intestine. New England J. Med., 236: 683-694, 1947. 19. MAYO, C. W. and NE~ROM, W. S. Carcinoma of the jejunum. Surg., Gynec. @ Obst., 65: 303-309, 1937. 20. WAKELY. C. P. G. Tumors of the smaI1 intestine. Brit. J. Surg., 14: 525-535, _ _-_ 1927. 21. GOLDEN, R. RadioIogic Examination of the SmaII Intestine. PhiIadeIohia. 1045. J. B. Liooincott. 22. NICKERSON,D. A. and WILLIAMS, R. H. MaIignant tumors of the smaI1 intestine. Am. J. Putb., 13: 53-64, 1937. 21. EBERT. R. E.. PARKHURST. G. F.. MELENDY. 0. A. and .OSBO~NE, M. P. ‘Primary tumors of the duodenum. Surg., Gynec. u Obst., 97: 135-139, ‘953. 24. PRIDGEN,J. E., MAYO, C. W. and DOCKERTY, M. B. Carcinoma of the jejunum and ileum excIusive of carcinoid tumors. Surg., Gynec.
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2.

3. 4. 5.

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7. 8.

Intestine

SORLIN, M. Observation d’un squirre d’une portion de jejunum chez ur sujet de quarante-neuf aus; morte a Ia suite de norubreux vomissemeus. J. gin. de mbd., cbir. et pbarm., 87: r89--196, 1824. LEICHTENSTERN, 0. Handbuch der speciehen. PathoIogie und Therapie, pp. 523-524. Leipzig, 1876. F. C. W. Vogel. MAYDL, C. Ueber den Darmkrebs, p. 7. Vienna, 1883. BraumueIler. NOTHNAGEL, H. In speciatle. Pathologie und Therapie, vol. 17, p. 310. Vienna, 1903. AIfred Hoelder. RANKIN, F. W. and MAYO, C. Carcinoma of the smal1 bowel. Surg. Gynec. ti Oh., 50: 939-47, ‘930. LIKELY, D. S., LISA, J. R., STITCH, M. N. and STEIN, H. D. Primary tumors of the small intestine. Arch. Int. Med., 82: 206-216, 1948. RAIFORD, T. S. Tumors of the small intestine. Arch. Surg., 25: 122-177, 1932. FORGUE, E. and CHAUVIN, E. Le cancer primitif et intrinseque (non vaterien) du duodenum. Rev. cbir., 50: 470-582, r914-1915.

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I

I