Primary tumors of the small intestine

Primary

Tumors

of the Small Intestine

E. MEREDITH ALRICH, M.D., RICHARD H. BLANK, M.D., Charlottesville, Virginia, AND JOHN J. FOMON, M.D., Miami, Florida is hoped that by recording the protocoIs of the cases in sufhcient detail, these data may be useful in the compilation of a review study at some later date. (Tables I and II.)

From tbe Department of Surgery, University oj Virginia School of Medicine, Cbartottesville, Virginia.

EOPLASMSof the small intestine appear infrequently when compared with tumors involving other portions of the gastrointestinal tract. This fact is impressive when one realizes that the small bowel comprises approximately 75 per cent of the length and perhaps over 90 per cent of the internal surface of the alimentary canal. This apparent immunity to tumor formation has been attributed to several factors: (I) the intestinal content is liquid and alkaline, (2) there is essentially no stasis in the small intestine, (3) the incidence of embryonal rests is very low, and (4) a specific protective hormonal or chemical factor may be present in the intestinal mucosa [I]. The relative incidence of the various types of tumors of the small bowel is dificult to determine. When autopsy and clinica materia1 are combined, benign lesions are more frequent than malignancies [2,4,10]. However, if only those patients with chnical evidence of disease are investigated, there is a significant predominance of malignant tumors [z-5]. In spite of their rarity, these lesions occur with sufficient frequency to warrant consideration in the differential diagnosis of unexplained abdominal pain or gastrointestinal bleeding. The literature relating to tumors of the small intestine is extensive, and it hardly seems warranted to report small series of patients. However, on close inspection of the majority of reports it is apparent that the method of documenting cases is varied, and one has difhculty in making a survey study in the hope of evolving the natural history of the various types of lesions of the small bowel found cIinicaIly. It is for this reason that we have undertaken a review of the cases of neoplasms of the small bowel encountered at the University of Virginia Hospital during the past twenty-five years. It

N

UNIVERSITY OF VIRGINIA SERIES All cases of primary tumors of the small intestine seen at the University of Virginia Hospital since 1932 were reviewed, and the microscopic sections of these lesions were re-examined by Dr. David Smith. Cases of tumors found incidentally at surgery or autopsy were excluded. Six cases were eliminated because of inadequate histologic data. Those lesions which were primary in the ampulla of Vater, having their origin from duodenal mucosa, common duct mucosa or pancreas, were excIuded because, in our ,opinion, these tumors are not germane to the subject of tumors of the small intestine. Twenty-two tumors primary in the small intestine were considered suitable for this study and have been divided into a malignant and a benign group with subclassifications as noted in Table III. The predilection of specific tumors to various segments of the intestine can be noted in collected reports. Cameron observed that in 200 cases of carcinoma of the jejuno-ileum, the lesion appeared in the proximal jejunum in 40 per cent, the distal ileum in 28 per cent and in the intervening segment in 32 per cent [6]. Lymphosarcomas [7] and argentafhn tumors [8] occur more frequently in the ileum, while leiomyomas and leiomyosarcomas [g] have a more even distribution throughout the small intestine. The most characteristic symptom in both the benign and malignant group is para-umbilical pain, and this was true of 75 per cent of the cases in our series. Acute obstruction occurred in four of the twenty-two cases, while symptoms 33

American

Journal

of Surgery.

V&me

99, Jmuary,

r960

AIrich, BIank and Fomon TABLE MALIGNANT

TUMORS

Symptoms and PhysicaI Findings

I

OF

Operative

THE

SMALL

BOWEL

Findings

Procedure

Follow-up

Study

and Sex Reticulum

Cell Sarcoma

(TWO Cases)

T

1

6g,W,M AbdominaI pain and alter-

nating constipation and diarrhea for 2 mo.; 5 cm. hard mass in right upper quadrant for 2 wk.

---~ 2

s,W,F

Cramping pain in right Iower quadrant for 4 mo., confirmed on physical examination

Tumor mass in mid-iIeum, invading mesentery

Segmental resection, end-to-end anastomosis

Patient died 8 yr. postoperativety of heart disease

Tumor

Resection of terminal ileum, cecum, ascending coIon; iIeotransverse coIostomy

Patient died 3 days postoperativeIyof puImonary emboIus

of terminal

Adenocarcinoma

-

iIeum

(Five Cases)

3

65.WJ

Cramping abdominal pain. Tumor arising from terminal ileum fiIIing cuI-de-sal aIternating constipation and diarrhea, IO pound i weight loss for 8 mo.; bright bIood in stooIs; dis ,tended tympanitic abdomen, I 2 cm. narrowed sig moid on barium enema; stoo1, 2 pIus benzidinepositive

Resection of terminal ileum, iIeotransversc colostomy

Patient died 8 mo. postoperativeIy

4

49,NM

Epigastric fuIIness with oc. Tumor of proxima1 jejunum at Iigament of Treitz casiona1 nausea and vommetastasis iting for 4 yr.; persistent vomiting for I wk.; gastrointestina1 series shower rI jejuna1 obstruction ---__-

PaIIiative gastrojejunostomy

Patient died on sixth postoperative day

53,W,F

Abdominal distention, nausea and vomiting; fret fluid in peritoneal cavity

None

Patient died on thirteenth postoperative day

SegmentaI resection, end-to-end anastomosis

None

Palliative jejunojejunostomy and jejunotransverse colostomy

Patient died 2 mo. postoperativeIy

~__74,W,F

None; autopsy showed car cinema of proxima1 jejunum with compIete obstruction, perforation, Iocalized peritonitis, Laennec’s cirrhosis

--

Papillary carcinoma of inCramping pain in right traIumina1, proxima1 Iower quadrant, distention, nausea and vomiting : ileum with almost compIete obstruction for 1 mo.; temperature, IOI’F.

7

41,W,F

Tumor of proxima1 jejunum extending retroperitonealIy to invoIve major vessels

DuII pain in paraumbiIica1 region radiating to back and easy fatigabiIity for 3 yr.; 6 by 8 cm. firm, movabIe mass in left upper quadrant; hematocrit, 22 To ; st.001, 4 pIus for bIood; gastrointestina1 series showed carcinoma of jejunum (Fig. I)

-

34

Primary

Tumors TABLE

MALIGNANT Age (yr.), Race and Sex

Case No.

I (Continued)

TUMORS

Symptoms and Physical Findings

--

Procedure

Follow-up

Study

(Four Cases) SubtotaI

45,W>M

Cramping epigastric pain and sensation of reduced stomach capacity for 4 mo.; gastrointestina1 series showed potypoid mass in base of duodena1 bulb _~

PapiIIary Iesion (0.75 cm.) on wa.II of first portion of duodenum

Excision of Iesion with button of adjacent duodenal waI1

Patient we11 6 mo. postoperatively

61,W,M

Intermittent episodes of abdomina1 distention for 2 yr.; visibIe and paIpabIe peristaIsis in Ieft upper quadrant; fiIm of kidney, ureter and bIadder showec chronic smaI1 bowe1 obstruction (Fig. 2)

Large mass at jejunoileal junction invading large segment of mesentery

Ileum resected except for dista1 3 cm., endto-end anastomosis (tumor left)

Patient we11 7 mo. postoperatively

34,W,F

Mass appearing intermittentIy in Ieft lower quadrant and 6 cm. mass in left paraumbiIica1 region for 8 mo.

Palliative side-to-side anastomosis

Patient died 3 mo. postoperativeIy

Resection of invoIved jejunum and peritoneum, inadequate margin

None

Re-expIoration, 17cm. segment of iIeum removed at previous site of tumor

Patient we11 5 yr. postoperatively

-.

-. II

Findings

UIcer in first portion of duo denum

-. --

10

SMALL BOWEL

Epigastric pain and vomiting, tenderness in right upper quadrant for 3 mo.; gastrointestinal series showed questionable duodenat uIcer

47,W,F

9

OF THE

Operative

Carcinoids 8

of SmaII Intestine

gastrectomy

ExpIoration 4 yr. Iater for uIcer revealed no metastasis

~Tumor arising from midiIeum, serosa puckered, metastases to mesentery and liver

Leiomyosarcoma

I2

13

(Two Cases)

Tumor mass in d&a1 ieiunum attached to peIvic peritoneum

mW,M

Right paraumbiIica1 pain, constipation, weakness, weight Ioss for 18 mo.; hematocrit, 28 y0 ; stooIs, 4 pIus for bIood; gastrointestina1 series showed questionabIe duodena1 deformity ___--

53,W,F

No residua1 tumor Intermittent abdominal folIowing origina pain for 2 yr.; .two Iarge dure extraIuminaI tumors removed from bowe1 waI1 eIsewhere

I

,

2 mo. proce-

Continued on following page

35

AIrich, BIank and Fomon TABLE MALIGNANT

Case No.

Age

(yr.), Race and Sex

Symptoms and PhysicaI Findings

I

(Continued)

TUMORS

OF THE

Operative

SMALL BOWEL

Findings

Procedure

FoIIow-up Study

Lymp bosarcoma (Two Cases) 14

15,W,M

Cramping abdominaI pain with intermittent constipation and diarrhea for I yr.; smaI1 boweI study suggested tumor -~~_

Segment (5 cm.) of jejunum thickened to diameter of 4 cm.; metastasis to regionat nodes

SegmentaI resection, end-to-end anastomosis

Recurrence 6 yr. postoperativeIy

Recurrence symptoms

Lesion simitar to primary

Segmental resection, end-to-end anastomosis, subsequent x-ray therapy

Patient we11 5 yr. postoperativeIy

Mass (8 cm.) invoIving entire circumference terminal iIeum

SegmentaI resection, end-to-end anastomosis

None

of obstructive

18,W,M AbdominaI tenderness in

right lower quadrant for 4 days; 8 cm. firm mass

of

of intermittent partia1 obstruction were encountered in over 50 per cent of the series. Melena, or acute gastrointestinal bIeeding, was present in onIy three cases in this group. The most common change in bowel function was that of alternating constipation and diarrhea. The most frequent physical finding was a paIpa-

bIe mass. PhysicaI findings other than those associated with some degree of obstruction of the smaI1 bowe1 were rare and non-specific when present. Anemia was present in five cases, i.e., hemogIobin was I I gm. per cent or Iess; the stoo1 contained bIood in six cases. The x-ray findings were confirmatory or suggestive of the correct diagnosis in eight cases. In retrospect, there were x-ray findings m two additiona cases which should have suggested the correct diagnosis of neopIasm of the smaI1 bowel.

FIG. I. Case 7. Gastrointestinal series demonstrates a filling defect in the proximal jejunum. A preoperative diagnosis of jejunaI carcinoma was confirmed at operation.

FIG. 2. Case IO. Microscopic appearance of a carcinoid tumor invoIving the jejunum. Notice the we11 formed acini with uniformity of nucIear staining. There is no ceIIuIar pleomorphism or evidence of malignancy.

36

Primary

Tumors

of SmaII Intestine

TABLE BENIGN

Case No.

Age Race and Sex

Symptoms and PhysicaI Findings

-_ 16

17

TUMORS

II

OF THE

SMALL

Operative

Findings

Fibroma

(Two Cases)

BOWEL

Procedure

-

Follow-up

Study

-

55,W,M

Para-umbiIicaI pain for 6 yr.; mass in right Iower quadrant

Firm white tumor (6 cm.) mass arising from terminal iIeum --

LocaI excision

72,N,M

AbdominaI pain, nausea and vomiting for 2 days; mass in Ieft upper quadrant

Large tumor mass arising from waI1 of ileum

SegmentaI resection, end-to-end anastomosis

Patient asymptomatic for 9 yr.

-. None

Lipoma 18

82,W,M

(One Case)

Nausea and vomiting, deep ReticuIosarcomas of Iiver jaundice for II mo.; gaspreviousIy diagnosed by trointestinal series showed periphera1 node biopsy; 3 cm. polypoid mass in Iipoma, first portion of duodenum duodenum

Local excision

None

Tumor (4 cm.) arising fron antimesenteric border on broad pedicle

Segmental resection, end-to-end anastomosis

Patient we11 2 yr. postoperatively

Intussusception at region of jejunum by poIypoid tumor mass

Resection of intussusception of jejunaI segment incIuding tumor, end-to-end anastomosis

None

Segmental resection, end-to-end anastomosis

None

Segmental resection, end-to-end anastomosis

Patient wet1 I I yr. postoperatively

Leiomyoma

(Two Cases) I

Weight anemia enema fect of 20

78,W,M

Ioss, weakness and for I mo.; barium showed IiIIing dececum (Fig. 3)

Cramping abdominal pain, distended abdomen for 3 days; gastrointestina1 series showed questionabte smaI1 bowe1 tumor

Adenomu

21

2g,W,F AbdominaI pain for 24 hr. with distention

(OneCase)

IntraIuminaI

adenomatous

POIYP

Neurilemmoma

22

57,W,M

Intermittent tarry stools for 18 mo.; 4 plus blood in stooIs, anemia (hemogIobin, 45 mg. %); x-ray of smaI1 bowel showed questionabIe jejuna1 tumor

(One Case)

Tumor mass (3 by 4 cm.) extending both intra- and extraI&naIIy with uIceration of 0verIying mucosa

37

AIrich,

BIank

and Fomon TAI3L E -

No. of

Benign

Fibroma Lipoma . . Leiomyoma. Adenoma. Neurilemmoma

Total.

.

III

1No. of Cases

Malignant

Cases

Adenocarcinoma Carcinoid ;Leiomyosarcoma. 1Lymphosarcoma ReticuIum cel1 sarcoma...........

.

5 4 2 2

_

Total.

.. -

2

15 -

Second, the symptoms of partia1 obstruction are caused by gradua1 compromise of the Iumen of the bowe1 by encroachment of the tumor. This occurs Iess frequently and at first gives rise to vague intermittent symptoms (Cases 7 and 17). BIeeding may be acute with exsanguinating hemorrhage (Case 22) but usually is occuIt in nature and manifest by secondary anemia and weakness (Case 12). Gastrointestinal bleeding of undetermined origin shouId aIways suggest the possibility of the existence of a tumor of the smaI1 bowel, and appropriate studies should be instituted to substantiate or rule out this diagnosis. A Ieiomyoma or Ieiomyosarcoma is more IikeIy to give rise to bIeeding than are other tumors because of its tendency to undergo tissue necrosis with the development of an uIcer crater. On x-ray examination, this ulcer crater in the tumor is sometimes visualized as a diverticulum-Iike process. The symptoms arising from IocaI irritabiIity secondary to tumor are atypica1 and diffIcuIt to evaluate. Not infrequently the patient wiII have melena and relativeIy typica symptoms of an uIcer without satisfactory confirmatory roentgenoIogica1 evidence of such a Iesion. In these instances, as in patients with vague symptoms suggestive of “chronic appendicitis,” disease of the gaIlbladder or chronic pancreatitis, carefu1 and adequate roentgenoIogic study of the smaI1 bowe1 may Iead to a positive diagnosis at an early stage of the tumor (Case 14). The majority of these tumors are overlooked in the course of routine examination of the gastrointestina1 tract. In recent years the roentgenoIogist has intensified his interest in this group of Iesions and, as a result, there has been an increase in preoperative diagnosis by roentgenology. The x-ray studies must be made specificaIly for study of the small bowel. Scout

FIG. 3. Case 19. Appearance

at operation of a Ieiomyoma of the distal iIeum. In spite of the extraIumina1 growth of this lesion, there was a history and Iaboratory evidence of sign&ant intestinal bleeding in this patient.

SurgicaI resection was accompIished in a11 of the benign group. Curative resection was performed in nine cases and paIIiative resection in five cases in the maIignant group. Operation was refused by the remaining patient in the maIignant group; the patient died, and positive diagnosis was made at postmortem. In the operated group there were two hospita1 deaths, one from pulmonary embolus on the third postoperative day and one from advanced metastatic disease on the sixth postoperative day. AI1 patients in the benign group survived without recurrent symptoms. In the malignant group three patients have survived five years or longer without recurrent disease, and two patients have Iived from one to four years without recurrence. COMMENTS

These tumors, whether benign or maIignant, produce symptoms dependent upon both the type of tumor present and its location. The majority of symptoms are related to disturbances in the orderIy progression of the intestina1 content. These may vary from mere irreguIarities in peristaIsic waves to compIete obstruction. Secondary anemia and IocaI irritative phenomena of the Iesion contribute to the symptomatoIogy in a significant number of cases. The obstructive symptoms are produced in two ways. First, the acute symptoms of compIete intestina1 obstruction are the resuIt of intussusception of the bowel, the tumor being the cause of the intussusception (Case 20). 38

Primary

Tumors

of SmaII Intestine

fiIms of the abdomen, with the patient both in recumbent and upright positions, shouId be a prehminary to the visualization of the small bowe1 by opaque materia1. The cardinal roentgenological hndings consist of the foIIowing: (I) dilatation proxima1 to an area of constriction, (2) a filling defect or Ioss of the norma mucosal pattern, (3) deIayed passage of the barium meal (Ionger than eight hours) through the intestinal tract, and (4) disturbance of motility resulting in segmentation of the barium in the small bowe1. Symptoms of the gastrointestina1 tract such as those related herein should strongIy suggest the possibiIity of tumor of the smaI1 bowe1. Definitive x-ray studies shouId offer reIiabIe diagnostic confirmation, and resection of the tumor by a IiberaI margin in most instances with early detection wil1 offer a curative resuIt.

in the future might utilize them in compiling statistica conclusions. A brief summary of the findings in these cases is presented.

SUMMARY

‘950. 8. DOCKERTY, M. B. Carcinoids of the gastrointestinal tract. Am. J. Clin. Path., 25: 794, 1955. g. STARR, G. F. and DOCKERTY, M. B. Leiomyomas and Ieiomyosarcomas of the smaI1 intestine. Cancer, 8: IOI, 1955. IO. ELIAS, W. S., LUND, C. C. and YONEMOTOR,R. Neoplasms of the smaI1 intestine. Am. J. Surg., 88:

REFERENCES

1. FELDMAN, M. Multiple primary carcinomas of the smaII intestine: a review of the Iiterature. Am. J. Digest. Dis., 20: I, 1953. 2. RAIFORD, T. S. Tumors of the smal1 intestine. Arch. Surg., 25: 122, 1932. 3. DUNDON. C. C. Primarv tumors of the small intestine. Am. J. Roentge&l., 59: 492, 1948. 4. RANKIN, F. W. and NEWELL, E. Benign tumors of the small intestine. Surg., Gynec. ti Obst., 57: 501, 1933. 5. BERNSTEIN, J. S. and CHEY, W. Y. A cIinica1 study of 1og cases. J. Mt. Sinai Hosp., 25: I, 1958. 6. CAMERON,A. L. Primary maIignancy of the iejunum and ileum. Ann. Surg., 108: 2o3,-Ig38. . 7. MARCUSE. P. M. and STOUT. A. P. Primarv Ivmphosarcoma of the smaI1 intestine. Cancer, 3: 459,

Twenty-two cases of tumors of the smaI1 bowe1 treated at the University of Virginia HospitaI over a twenty-six-year period are reviewed. The primary purpose of this effort is to record in chart form the pertinent data concerning these cases so that Iarge clinical reviews

384. 1954.

39