unfortunately this condition is rarely diagnosed prior

LYMPHO-SARCOMA OF THE SMALL INTESTINESBY FRED W. RANKIN, M.D.

OF LEXINGTON, Ky.

LYMPHO-SARCOMA of the gastro-intestinal tract is reported in the literatureas being found in every segment from the stomach to the rectum, but seemsto show a marked predilection for the ileal division of the small bowel. Whileof relatively infrequent occurrence as compared with carcinoma throughoutthe entire intestinal tract, it perhaps is found as frequently in the small bowelas any -form of malignancy. Broders and Mahle recently reported twelvecases of lympho-sarcoma of the stomach giving its incidence in comparisonwith carcinoma as I to 68. Their review of the literature showed only oneother series as large as their own, with scattering reports of an occasionalcase from various sources. The literature shows reported cases in the smallbowel and colon to be approximately 300; several relatively large series havingbeen collected by various writers, among them those of Libman and Crowther.Krugerz Boaz collected 37 cases of sarcoma of the intestines which weredistributed about equally between the large and small bowel; the rectum,however, being the site of a malignancy in i6 cases. DeNoyelles cites therelative frequency of this disease as compared to carcinoma as I to 20. Thestriking similarity in the distribution is noted when it is seen that the rectumand ascending colon, as in carcinoma, are the most frequently involved seg-ments of the large bowel.

Gerster and DeNoyelles report cases in the small bowel occurring high inthe jejunum and an occasional case involving the ileum, ileo-coecal valve andthe caecum. Crowther reports I2 cases occurring in the duodenum in a seriesof I9I cases, and Libman's review found 15 additional such cases in thesame location.

Syniptomnatology.-Unfortunately this condition is rarely diagnosed priorto operation and its most frequent recognition comes at the autopsy table.There seems to be no single line of symptoms which constantly occurs inthese cases. Either the attack is ushered in simulating an acute fulminatingabdominal infection, which is usually diagnosed acute appendicitis, or as ismore usual the findings of constitutional symptoms of anmemia, cachexia, andweakness predominate. Ochsner describes the early symptoms as indefiniteabdominal pain which persists and is not relieved by rest and starvation, as isthe case in the usual chronic abdominal infections. The character of the painis colicky; there is slight if any tendency to localize in any one definite spot.The fact that stenosis of the bowel does not occur until glandular involvementfrom the outside causes partial intestinal obstruction, militates also againstan early diagnosis. Ulceration being rare in the beginning of this disease,whose pathology is located first in the submucous coats of the bowel, precludes

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LYMPHO-SARCOMA OF THE SMALL INTESTINES

X-ray assistance being of much value in early diagnosis. The bowel not infre-quently is dilated proximal to the tumor because of paralysis of the muscu-lature from direct invasion of the malignancy. The fact that there is adilatation of the bowel about the growth rather than a stenosis produces anintermittent obstruction which probably accounts for a more constant chain ofsymptoms than any other one singlefactor. In the advanced stage of thedisease where tumefaction is distinctlyevident, obstruction is most likely tooccur and a diagnosis of malignancy bemade, but without hope from surgicalintervention. Cases are reported inwhich there has been an intussusceptionpresent which produced partial obstruc-tion, anorexia, and bloody diarrhcea.In one of our own cases the tumor massextended into the cwecum through theileo-caecal valve and the attempt of thebowel to rid itself of this bolus pro-duced an intussusception with partialobstruction which was diagnosed acuteappendicitis with abscess. In the chronictype of case the tumor mass, which isusually irregular and probably mostoften due to lymphatic involvement inthe mesentery, is variable in its locationand usually of a not too firm con-sistency. The latter condition whencachexia has taken place and metastasesare evident, must be differentiated from

FIG. I.-Case I. Gross specimen; most markedperitoneal tuberculosis, carcinoma, and portion of tumefaction is in ileum cecal band isthe granulomata. distinctly shown.

The age of the individual should have some bearing upon the diagnosis-a sarcoma always being looked upon as a disease of early youth or youngadult life. The distribution as far as sex is concerned is about equal. Oneof our own cases was a young man and the other a middle-aged woman. Theage incidence, however, is distributed over the first to the fifth diecade. Theprognosis is always gloomy; recurrence is rapid despite any treatment avail--able, and Baltzer quotes the duration of the disease as less than one year fromits time of onset.

Pathology.-Pathologists agree that lympho-sarcoma of the bowel beginsin the lymph follicles and may be either of a primary or secondary nature.The gradual extension of the growth into the mucosa and the other bowelcoats, except the peritoneal one, is progressive. This extension rarely causesperforation and resulting peritonitis. The ulceration through the mucous mem-

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FRED W. RANKIN

brane, however, occurs in a relatively high percentage of cases. Occasionallypedunculated growths are found. The microscopic picture is consistent. Thetype of cell which predominates usually resembles the lymphoid cells of themucosa, of which there are many variations. In the cases reported byDeNoyelles the predominating cell resembled the transitional mononuclear cell

of the blood. Bunting andH u s t o n show that thelymphocytes in the bloodstream migrate into the in-testinal mucosa to functionnormally.- Other writerssuggest that these lymph-oid cells in the presence ofsome irritation proliferatewi dl y and DeNoyellessuggests that a chronicirritation, possibly a spe-cific toxin, played animportant part in thegenesis of lympho-sarcoma.Because of the histologicalresemblance to an infec-tious granulomata he sug-gests that lympho-sarcoma"Is one of the many bi-zarre later p i c t u re s oflesions which were at onetime of the nature ofH o d g k i n's disease, orlymphoblastic or lympho-

FIG. 2.-Case I Gross specimen opened and showing the pro-trusion through the ileo-c2cal valve into the lumen of the large cy t i cleukwmia." Thebowel. lymph-glands w h i c h are

early infected grow rapidly and frequently attain the size of walnuts. Theirpressure upon the neighboring loops of bowel occasionally become sufficientto produce obstruction. The invasion of the mucous coats by the tumor itselfcauses paralysis and distention rather than stenosis.

Treatment.-Treatment by whatever methods undertaken usually yieldsunsatisfactory results. If the diagnosis be established before too muchlymphatic invasion has taken place, surgery seems to offer the best chanceof cure or at least the most palliation. Where the process is extensive andwhere a resection would involve the sacrifice of too wide an area of bowelsubstance palliative measures such as sidetracking operations are indicated.Radium in certain sarcomatous cases has given such excellent results thatsome observers have been stimulated to urge its use in this condition. Certainly

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the end results from surgery are poor, even from the standpoint of prolon-gation of life, to urge it as other than a last resort. Under any of the presentday treatments the prognosis is poor, and unless the early diagnosis of thiscondition can be made there is relatively little chance of instituting other thanpalliative measures. It is suggested that radiological evidence may be ofgreater value in an early diag-nosis of these lesions under amore improved technic than wehave at present. When we knowthe number of cases of sarcomaof the small bowel reported andconsider their uniformly fataloutcome a short time after beingdiscovered, and where an exhaus-tive study of the clinical syn-drome or laboratory investigationhave disclosed nothing helpfule x c e pt the after-thought thatX-ray examination might havefound the lesion earlier, it is evi-dent that the opaque meal shouldnot be restricted so largely tocases in which carcinoma ofthe stomach or large bowel issuspected.

The insidiousness of the dis-ease and its lack of a consistentsymptom-complex seem to showconclusively that we must look K _to the r6ntgenologist for prog-toetherintgekinogit frperoag- FIG. 3.-Case 1I. The gross specimen of the resectedress in making an earlier diag- smali bowei showing where the attachment and perforation

nosis, if we are to reduce the took place.mortality by any therapeusis now available. Appended are the case reportsof two cases which came under our observation and were operated' duringthe same week in I923.

CASE I.-H. R., male, age thirty-one, laborer. Chief complaint: Pain in right side ofabdomen; marked constipation for six weeks. Family history: Negative.

Personal History.-Measles and whooping cough in childhood with complete recovery;pneumonia ten years ago; typhoid fever four years ago, no complications or sequelawith either. General health good. Otitis media eight years ago, short duration and norecurrence. Frequent toothache. No dyspncea, cedema or cough. Frequent drenchingnight sweats, have been common during both winter and summer for many years.Appetite always good; no jaundice; troublesome constipation for six weeks previous toadmission. Frequent and urgent urination for last six months. Nervous system negative.Best weight I65; average and weight on entrance I63 pounds.

Present Illness.-Patient felt perfectly well until six months before coming intothe hospital. At that time he began having vague abdominal discomforts which he

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describes as indigestion. Cramping sensations would be frequently experienced afterthe drinking of cold water or after ingesting a heavy meal. This would be evidentsometimes-befbre the meal would be completed. The occurrence of this uneasiness wasdependent on the size of the meal rather than upon. any particular articles of food. Itwould be felt most plainly in the lower right quadrant of the abdomen and wouldpersist from thirty minutes to two hours after eating. This phenomenon was noticedabout two-thirds of the time. Eructation after eating was common. Small quantities of

food were occasionally regurgi-tated but no true vomiting occurred.During the five weeks previous toadmission a constant, dull pain hadbeen present in the right lowerquadrant. This was aggravated bywalking or bending over. Thiscondition b e c a m e progressivelymore annoying up to the time ofadmission. Pain and sensitivenesswere then so marked over the

-: affected area that the patient washurried to the hospital at seven

t.'Z'e o'clock Thanksgiving evening.Physical Examination.-Tem-

perature I00.5, pulse IO, respira-tion 24. Well developed and nour-ished white male thirty-one yearsold. Mucous membranes gave evi-

i dence of moderate anemia. Facialexpression anxious. Teeth in poorcondition. Tongue lightly coated.

* Neck, heart, and lungs negative.Abdomen: Distinct fulness in rightlower quadrant. No intestinal pat-terns seen. Rigidity present overlower half of the abdomen, moremarked on right. A tender masswas present over the csecal region

FIG. 4.-Case II. The specimen cut open showing the inside w i t h a c u t e sensitiveness overof the howel lumen. McBurney's point. This mass was

ovoid, fluctuant, and felt to be about the size of a grape fruit. Abdomen negative other-wise. Rectal negative.

Laboratory Blood Exarmination.-Red blood cells 4,000,000, leucocytes ii,ooO, poly-morphonuclears 68 per cent., urine negative.

Diagnosis.-Suppurative appendicitis, localized abscess. Immediate operation advised.Report of Operation.-Right rectus incision made. Large tumor mass was found

involving the terminal ileum and cacum. Enlarged glands were present in the mesenteryin this region. The terminal ileum for a distance of about eight inches, the caecum andascending colon were removed, and the terminal portion of remaining ileum was anas-tomosed end-to-side to the colon. All of the enlarged glands could not be removed.Abdomen was closed without drainage and aside from a wound infection the patient madean uneventful recovery from the operation.

Four months later, the patient returned to the hospital on account of return of abdom-inal discomfort, dyspneea, and increase in girth of abdomen. Examination of abdomen dis-closed masses that were probably the result of co'ntinued growth of the neoplastic process.

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Gross Description.-Specimen consists of a portion of ileum, cecum with attachedappendix, and portion of colon. The junction of ileum with colon is distorted andchanged from its normal appearance due to an invasion of the wall of the intestines with atumor mass. The appendix is I40 x I6 mm. adherent to the intestine with a firm, fibrousadhesion. Two centimetres toward colon from origin of appendix, the mucosa contains afairly smooth, rather firm, elevated tumor mass, about 30 mm. in diameter which tendsto circle the colon and extends well into the submucosa. From this mass the wall of theintestine to the appendixand backward towardileum, averages 22 mm.in diameter, being infil -

trated with a pale gray, 'fairly firm, somewhathomogeneous t u m o rmass. About 3 cm. -,from origin of appendixtoward the ileum, thetumor mass extends outinto the lumen in a cir-cular manner alImos toccluding the lumen of A

the intestine. The wallof the intestine towardthe ileum is invaded I2cm. from origin of ap-pendix from which place t,the ileum and its mucosaappears normal. T h ewall of the intestinetoward the colon is FIG. 5--Microphotograph from a section of Case I, showing delicate connec-

tive-tissue stroma supporting typical lymphoid cells.invaded 5 cm. f ro0mappendix from which place mucosa appears normal. The mucosa of the intestine overthe area of tumor invasion of its wall (between areas of normal mucosa of ileum andcolon) is dull purplish-red, roughened and indistinct as to its normal markings.

(2) Portion of mesentery contains few lymph glands which are fairly firm, dull redand on section varies from pale gray to purplish-red with loss of normal appearance oflymph gland. Cut surface resembles somewhat rice in water with dark shaded colors. Theglands average 20 mm. in diameter.

Microscopic Diagnosis.-Lympho-sarcoma.CASE II.-M. M., age forty-nine, housewife. Patient was a Slav with but slight

knowledge of English so that only a scant anamnesis could be obtained. Chief complaint:Pain in lower right side of abdomen. Family history: Negative as far as obtained.

Personal History.-No pulmonary troubles that were at all chronic. Patient wasable to do a full day's work up to the time of coming in to the hospital. She had sufferedwith constipation for the four years preceding. Never jaundiced. Frequency and burn-ing on urinating for six weeks previous to admission. Patient had been pregnant thirteentimes, twelve of which terminated normally and one miscarried. The menopause occurredsix years before admission.

Present Illness.-Two months before coming into the hospital patient experiencedgeneralized pain in the whole lower abdomen. This persisted for about a week and thenbecame localized in the lower right quadrant. It has been constant there since. Patienthas vomited three times during this whole period.

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FRED W. RANKIN

Physical Examination.-Patient is a moderately well nourished white woman forty-nine years old, and is in obvious discomfort. Color is fair. Head, eyes and nose negative.Oral sepsis present. Pharynx and neck negative. Thorax: Antero-posterior diameterincreased. Percussion note hyperresonant to 4th rib anteriorly and impaired with limitedexpansion at both bases. Breath sounds indistinct with occasional moist riles at bothbases posteriorly. Heart: Sounds muffled, otherwise negative. Abdomen: Generalizedg-g X D F sa *distention. Somewhat

a h. ~~~~~~~~~~~~tenderthroughout, mostmarked in lower rightquadrant with a secondarea of slightly increasedsensitiveness in u p p e r

part of upper right quad-rant. A mass was pal-pable in right side just

| t89below umbilicus and ap-parently extending into

*Vaginal Examina-tion.-O u t I e t relaxed.Yellowish discharge pres-

s; 23p*- ent. Cervix laceratedand pointed up to sym-physis. Uterus turnedback. There was a

smooth mass extendingfrom uterus up into re-

FIG. 6--Microphotograph of Case II. Picture similar to Case gion of caecum. Thiswas quite adherent and

manipulation brought forth such -pr'otests from the patient that thorough examinationcould not be made at the time. A tentative diagnosis of fibroid tumor with dense adhe-sions of bowel was made. Under anmsthesia, however, it was found that the pelvic tumorcould be moved somewhat without disturbing the position of the uterus to a correspondingextent and appeared to be primary in region of caecum.

Laboratory Data-.Urine negative. White blood cells 7,200 with 75 per cent.polymorphonuclears.

Diagnosis.-Tumor of cacum with adhesions to pelvic organs.Operative Findings.-Right rectus incision made. A tumor of the small gut was

found situated about i0 inches from the caecum, densely adherent in the pelvis and stuckto back of uterus. During delivery from pelvis it was opened at one point. It wasresected together with six inches of normal bowel on the proximal side and four incheson the distal side. Numerous hard glands were present in the mesentery. An end-to-endanastomosis was made with catgut and silk. About ten inches proximal to the anastomosisand ileostomy was done after the method of Witzel. No metastases other than glandularwere made out. Abdomen closed without drainage. The patient rallied and did well forseveral days but died on the twelfth day.

Partial autopsy showed no peritonitis. The anastomosis was healed. Numerouslymphatic glands throughout the bowel mesentery were found to be invaded. Death wasdue to bilateral broncho-pneumonia.

Description of Specimen.-Gross: Specimen consists of portion of small bowel withattached mesentery and lymph-nodes.

(a) Forty millimetres from one end of the gut. The gut wall is enlarged into atubular mass, incised, 50 mm. in diameter and total length of I40 mm. which is firm,

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yellowish to pinkish-gray and mottled with red. On section, the wall of the tubular massiS 20 mm. thick and is lined with a purplish-red mucosa, wall of which measures 3 mm.in thickness. Mucous surface of normal gut is pinkish-gray, mottled with red. Cutsurface shows i8 cm. of gut with apparently normal mucosa, while the remainder showsno evidence of folds in the mucosa, and is rather smooth and pale to deep reddish-gray.The wall of latter portion is 8 to 20 mm. thick, gray, firm and contains a fewfibrous striations.

(b) Attached mesenteric lymph-nodes which vary from 6 to 35 x 20 mm. moderatelyfirm. Cut surfaces vary in being gray, yellowish-gray and light brown. All are moder-ately smooth.

Microscopic Diagnosis.-Lympho-sarcoma.

BIBLIOGRAPHYBroders and Mahle: Jour. Lab. and Clinical Med., vol. vi, I921.Libman: Amer. Jour. Med. Sciences, I905, vol. cxxix.Krugerz Boaz: Bull. System of Practice of Surgery, vol. iv.Gerster, Jour. A. M. A., vol. lxxxii, I924.DeNoyelles: ANNALS OF SURGERY, vol. lxxvi, July-Dec., 1922.Ochsner: Surgical Diagnosis and Treatment, vol. iii.Bunting and Huston: Jour. Exper. Med., vol. xxxiii.

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unfortunately this condition is rarely diagnosed prior

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