Lymphosarcoma of the stomach

LYMPHOSARCOMA REPORT JOHN

OF THE STOMACH*

OF A CASE WITH SOME CLINICOPATHOLOGICAL

B.

O’DONOGHUE,

AND

M.D.

Professor of Surgery, LoyoIa University Medical SchooI; Attending Surgeon, Cook County Hospital CHICAGO,

T

HE eadiest reported cases of sarcoma of the stomach found in the literature are those by SibIey,l in 1816, and by Bruch,z in 1847. Since that time interest has primariIy been centered on this type of neopIasm because of its reIative infrequent occurrence, and secondIy, the more favorabIe prognosis afforded to a patient with a malignancy arising from Iymphoid tissue, in comparison to malignant Iesions arising from epitheIia1 tissue of the stomach. TABLE I INCIDENCEOF SARCOMAIN MALIGNANTLESIONS THE

Author

Smithies and Ochsner’. Pack and McNee?. Cheeverg . . . . . Walters et at.‘“. Cameron and BresIichG Cook County Hospital” 11,882autopsies. Surgical PathoIogy

STOMACH

-

Year

919

No. Cases Carcinoma

No. Cases Sarcoma

921

4 5 9

400 628 6,242 I35

935 9’3-1932

907-1938 930-1940 930-1940 933-1940 ---

OF

._

TotaI.

110 2

272 180

6

8,778

I39

3

-

Masson reviewed the cases of maIignant Iesions of the stomach encountered at the Mayo CIinic from 1go8 to rgzo and found only thirteen proved cases of sarcoma in * From the Departments

MAURICE

B.

NOTATIONS JACOBS,

M.D.

Associate in Surgery, Loyola University MedicaI School; Associate Attending Surgeon, Cook County Hospital ILLINOIS

2,067 cases of maIignant Iesions of the stomach. Haggard4 found that up to 1920, onIy 244 cases of sarcoma of the stomach were reported in the Iiterature. In 193 I, coIIected ninety-one additiona Askey cases, and in 1941, Cameron and BresIich6 reviewed the Iiterature and found 104 isoIated cases of gastric sarcoma had been reported in the preceding ten years (1930 to 1940). In aI1, Iess than 500 cases have been reported up to the present time. (TabIe I.) CASE REPORT A. D., a white male, aged sixty-three, was admitted April zz, 1941, to the Cook County HospitaI (surgical service of Dr. J. B. O’Donoghue) with compIaints of anorexia and vomiting foIIowing the ingestion of food of two months’ duration. Marked generaIized weakness of six weeks’ duration was present. It was Iearned, however, that his immediate reason for seeking hospitaIization was because of persistent abdomina1 pain of four days’ duration which was described as cutting like, and Iocated to the right of the umbiIicus. Radiation of the pain across the epigastrium to the Ieft hypochondrium wouId occur upon the ingestion of food. At the onset of his iIIness, the patient was abIe to retain miIk and soup, but for three weeks preceding hospitalization, emesis wouId occur whenever any food with the exception of miIk was taken. A weight Ioss of 30 pounds had occurred during the past year; hematemesis or tarry

of Surgery, Cook County Hospital and LoyoIa University SchooI of Medicine. before the Chicago PathoIogicaI Society, January 12, 1942. 246

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stools had never been noted by the patient. past medica history was irreIevant. famili a1 history was of having a brother

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one examination of the stoor was made, this was negative for bIood. The bIood cevit amic acid IeveI was 0.1 mg.

FIG. I. Roentgenogram showing Iarge filling defect involving the pars prepyIorica and the distal half of the pars media of the stomach.

cumb to a stomach aiIment; however, the exact nature was not known to the patient. On physica examination, the patient appeared emaciated and exhibited a marked generaIized weakness. His temperature was 20 and blood 97.6%.; pulse rate 84; respirations pressure of 124/100. The essentia1 physicopathoIogica1 findings were limited to the abdomen which was scaphoid; tenderness on paIpation was present in the epigastrium. No masses were palpable, aIthough the Iiver edge was just made out below the right costal margin. Laboratory examination revealed the bIood hemogIobin to be 75 per cent (Sahli), the 4,200,000 and the erythrocytes numbered Ieuckocytes 10,000 ceIIs per cubic miIIimeter, respectively. The serologica reaction was negative, as was aIso the urine for aIbumin, sugar, biIe and bIood. Gastric anaIysis by the EwaId test mea1 reveaIed an absence of free hydrochIoric acid; the combined acid, however, was 53 degrees. BIood was absent both grossIy and chemically from the stomach contents. OnIy

A barium mea1 was administered and the roentgenoIogist , found a large, rigid, superimposabIe defect invoIving the entire pars prepyIorica and the dista1 haIf of the pars media. The opinion was expressed that the findings were indicative of an advanced carcinoma of the stomach. (Fig. I.) Gastroscopic examination confirmed the roentgenoIogicaI findings. A roentgenogram of the chest reveaIed no apparent metastasis. The patient was prepared for operation, the preoperative diagnosis being carcinoma of the stomach, aIthough the absence of blood in the stooI could not be correIated with the cIinica1 history and Iaboratory findings. anesOn May 6, 1941, under cycIopropane thesia the abdomen was opened through a Ieft para median epigastric incision. A large firm mass invoIving the pyloric and prepyIoric portions of the stomach was present. The perigastric Iymph-nodes were enIarged and firm. No apparent metastasis in the Iiver or to the peri-aortic Iymph-nodes were present. The lesion appeared resectable; accordingly, the

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stomach was mobiIized and a high subtota1 gastric resection was performed. The continuity of the gastrointestina1 tract was re-estabhshed by the P6Iya type of gastrojejunostomy. The resected specimen was submitted to the

FIG. 2. Low power photomicrograph taken from uIcerated area of the stomach showing diffuse lymphocytic infiItration of the stomach waII. (Hemotoxylin-eosin stain. X 99.)

Department of SurgicaI PathoIogy and was described as foIIows: Grossly: “In the mucosa of the distal end is a 5.5 X 6.5 cm. uIcer located on the Iesser curvature. The edges of this defect are firm and roIled, the base is cIean. Just proxima1 to the uIcer is a sIight na.rrowing of the Iumen and another uIcer, measuring 2.5 cm. in diameter is present whose edges are more irregular. SeveraI Iymph nodes, the largest measuring up to 1.2 cm. in diameter are attached to the pyIoric end. MicroscopicaIIy, sections taken from the uIcerated area reveaIs a reticuIum ceil sarcoma of the stomach.” The postoperative course was entireIy uneventful, multipIe bIood transfusions, parentera1 fluids, intravenous cevitamic acid together with genera1 supportive measures resuIted in his being afebriIe on the fourth postoperative day. On his fourteenth postoperative day, the patient was discharged from the hospita1 and

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referred to the x-ray therapy division where he received twenty-one treatments from May 26 to August 12, 1941, each treatment consisting of 300 r units, the rays being administered over five fields.

FIG. 3. High power photomicrograph showing infiItration of stomach wall by Iymphocytes. The nuctei are rich in chromatin and are surrounded by a narrow rim of a pate staining cytoptasm. Variations in size and shape are present. (HematoxyIin-eosin stain. X 670.)

The patient has since gained 30 pounds in weight, feeIs stronger and at the present time is working. A barium mea1 given on December 5, 1941, shows a we11 functioning stoma and no apparent neopIastic invoIvement of the stomach. (Figs. 2 to 6.)* DISCUSSION

The frequency of the sarcoma of the stomach was given in 1919 by Ewing12 as comprising 1 per cent of al1 gastric maIigThis is in conformation with nancies. operative and autoptic statistics from German clinics given by Ziesche and Davidson13 a decade earlier. * On September 14, 1942, this patient was contacted. He is going to business and is feeIing fine.

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In the ten-year period from January I, 1930 to January I, 1940, a total of I 1,882 consecutive necropsies were performed at

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were seen, compared to r80 cases of carcinema of the stomach, giving a ratio of I case of sarcoma to every 6o cases of

FIG.

4.

FIG.5.

FIG. 4. High power photomicrograph showing separation of the muscIe bundles of the stomach by invasion of the lymphosarcomatous ceIIs. (Hematoxylin-eosin stain. X 670.) FIG. 5. Low power photomicrograph of a perigastric Iymph-node. Only one lymph foIIicIe is present. The remainder of the architecture has been replaced by Iymphosarcomatous elements. (HematoxyIin-eosin stain. X 99.)

the Institute of PathoIogy of the Cook County Hospital. In this totaI, 278 cases of maIignant Iesions of the stomach were encountered, of these six cases were sarcoma. OnIy two of the Iatter were of the Iymphosarcoma type. SimiIarIy, the statistics of the Department of SurgicaI PathoIogy for the seven and a haIf period from JuIy I, 1933, to January I, 1940, revea1 that onIy three cases of Iymphosarcoma of the stomach

carcinoma. This ratio is in conformation with the most recent statistics of the Mayo CIinic’O comprising 6,352 operative cases of maIignant Iesions of the stomach observed in the thirty-one years eIapsing from 1907 to 1938, of which I IO cases were sarcoma, the ratio being I :58. ConsiderabIe disagreement in the terminoIogy and cIassification of neopIastic and neopIastic-Iike Iesions of the Iymphoid system exists because of the Iack of knowl-

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edge as regards the etioIogy and histogenes;is of these Iesions. Tr auma was e‘mphasized by early au-

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type of Iesion may be diffIcuIt is evidericed by a case cited by Kirshbaum14 and aIs by Eusterman and BaIfour15 which was

FIG. 6. Roentgenogram taken seven months after operation showing stoma of stomach with no apparent neoplastic involvement.

thors as the causative factor; the theory of degeneration of benign lesions of the stomach into maIignant ones, as poIyps to carcinoma, or myoma to sarcoma have been advanced. Other theories, as tuberculous Iesions of the stomach causing irritation in the presence of aberrant undifferentiated ceIIs in the submucosa, or the chronic irritation produced by peptic uIcer are specuIations that have been offered. Ewing is of the opinion that Iymphosarcoma may begin as an inflammatory process. In discussing the origin of this tumor in the stomach, Ewing states the foIIowing questions must be answered: Is it part of a Ieukemic process? Is it part of a gastrointestina1 pseudoIeukemia? Is it part of a generalized Iymphosarcoma, or is it soleIy a IocaIized tumor? That the pathoIogica1 diagnosis of this

reported by Freeman,16 wherein sections taken from a case of gastric sarcoma were submitted to severa noted pathoIogists, who presented various diagnosis of carcinoma, Iymphosarcoma, inffammatory tissue and chronic granuIoma. Ewing classifies sarcoma of the gastrointestina1 tract as (I) spindIe ceI1 myosarcoma, (2) misceIIaneous round ceI1 or mixed ceI1 aIveoIar sarcoma and (3) Iymphosarcoma. Primary Iymphosarcoma of the stomach may arise from any Iymphatic tissue in the organ. It is probabIe, however, that the Iesion begins most often in a Iymph foIIicIe in the submucosa. From this point of origin, it penetrates aIong the tissue spaces and infiItrates the various Iayers, particuIarIy, the muscle Iayers, each band of muscIe being separated by Iarge masses of tumor

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ceIIs. The submucosa is enormousIy thickened and this expIains the giant rugae that are sometimes seen in a roentgenogram. The mucosa, not being the site of the original growth does not show the early characteristic uIceration of carcinoma. However, Iater, ulceration does take pIace and characteristic uIcer niches and craters may develop. These tend to be more shaIIow than in carcinoma and frequentIy are muItipIe. InvoIvement of the serosa is usuaIIy a Iate manifestation but often assumes great proportions. Due, perhaps to compromise of the bIood suppIy by diffuse infiltration of a11 the Iayers, necrosis is frequent and for this reason perforation is not uncommon. Koucky” and his associates in reporting an unusua1 case of an acute perforation of a Iymphosarcomatous uIcer of the stomach occurring in a maIe, aged fourteen, with the cIinica1 picture of a perforated peptic uIcer are of the opinion that the Iargest number of perforations occur into adjacent structures with or without a sarcomatous peritonitis. They advocate the opinion expressed by McNeaIyls of the advisabiIity of taking a biopsy from every case of gastric perforation. From a study of sixty-seven cases of sarcoma of the stomach, Madding and WaItersl$ found the Iymphosarcoma as being the most common form. These are divided into two types: (I) reticuIum ceI1 sarcoma, (or the Iarge round ceI1 Iymphosarcoma and (2) maIignant Iymphocytoma (or the smaI1 round ceI1 Iymphosarcoma). This differentiation is further emphasized by Madding and Waiters as being of a practica1 and cIinica1 significance, inasmuch as the two types differ in their response to roentgen therapy. KrumbhaarzO is of the opinion that the reticuIum ceI1 sarcoma is more resistant to roentgen therapy than is the maIignant Iymphocytoma. The Iocation of the Iesion is most usuaIIy in the antrum, but generaIIy is far enough away from the pyIorus so that obstruction by the tumor is uncommon. Our case

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differed from this, in that the Iesion produced symptoms of pyIoric obstruction. CLINICAL

FEATURES

Sarcoma of the stomach occurs at a much earIier period of Iife than does carcinoma. From statistics of Iarge numbers of cases in the Iiterature, the average age of carcinoma of the stomach is given as sixty-one years, aIthough in our series of 272 cases of carcinoma of the stomach, the average age is found to be fifty-five years; whereas the average age of sarcoma of the stomach is found to be forty-one years. Case reports of sarcoma of the stomach occurring in the extremes of Iife are present in the Iiterature, FinIayson,*l reporting the youngest patient as three and one-haIf years of age, Hunt,22 reporting a case in a patient three years and eight months of age, and Gosset23 reporting the oIdest patient as being eighty-five years of age. BaIfour and ‘McCann24 in reviewing a series of fifty-four cases of sarcoma of the stomach found their youngest patient to be ten years of age and the oIdest sixty-seven years of age, the average for the group being forty-three years. As to sex, the frequency was predominateIy in the maIe sex, the incidence ranging from 60 to 70 per cent, which conforms to the maIe preponderance existing in maIignant Iesions of the stomach. Pain is the most common feature and is either generaIized throughout the abdomen, or, more often IocaIized to the midepigastrium. In many cases, the pain is similar to that seen in peptic uIcer. Other symptoms are beIching, anorexia, epigastric nocturna1 pain and Ioss of weight and strength. The reIationship of symptoms to Iesion is emphasized by Madding and Walters inasmuch as the reticuIum cell sarcoma arises from the reticulum ceIIs of the Iymph foIIicIes in the submucosa; and since the sarcoma originates in the submucosa, the mucosa itseIf is not earIy invoIved and uIceration does not occur as soon as it does in carcinoma because of the proximity of the sarcoma to the submucous

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pIexus of nerves, pain is a very common feature. The average duration of symptoms is found to be from two months to severa years. In the inoperabIe cases the average duration of symptoms is about thirty-seven months. Gastric anaIysis reveals an achIorhydria in the Iargest number of cases, aIthough norma acid vaIues were found in 20 per cent of the cases reported by Madding and Waiters. The blood picture is usuaIly that of a microcytic hypochromic type. BIood in the stoo1 is generaIIy absent, and is present onIy in the Iate cases when the mucosa has become uIcerated. Roentgen examination by barium mea1 is usuaIIy resultant in a diagnosis being made of carcinoma. HoImes, Dresser and Camp25 are of the opinion that nothing diagnostic is present roentgenoIogicaIIy in Iymphosarcoma of the stomach, but that in some cases, peristaltic movements are not as much interfered with as in cases of carcinoma. Gierez6 recentIy reported a case diagnosed correctIy by gastroscopic examination, one month after roentgen examination had faiIed to discIose the presence of any abnormaIity of the stomach. The prognosis is somewhat better in cases of sarcoma of the stomach than in carcinoma. The average Iife span in the Madding and Waiters series was a IittIe over three years, the Iongest surviva1 period for any patient being fifteen years. Jones and Carmodyz7 report the case of a patient who at the age of nine years underwent a subtota1 gastric resection for Iymphosarcoma of the stomach who is aIive and we11 nineteen years Iater. The treatment of sarcoma of the stomach might at first gIance seem a hopeless subject, but such is not the case. In fact, quite the opposite is true. At the onset, we can say that in cases of tumors of the stomach of comparabIe size, one a carcinoma, the other a Iymphosarcoma, that the Iatter wiII in the hands of surgery, together with

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postoperative x-ray therapy have a better prognosis and a greater Iongevity. We believe that a11 tumors of the stomach shouId have the opportunity of Iaporatomy inasmuch as the ratio of operabiIity of Iymphosarcoma has been reported by various authors as being from 60 to 85 per cent. The reason for this being, that Iymphosarcoma metastasizes Iate and then usuaIIy to the regiona Iymph-nodes in the gastrohepatic and gastrocolic omentum, aIso because the tumor mass usuaIIy remains within the stomach waI1 and does not become attached to adjacent viscera. This is not true for spindIe ceI1 sarcoma, Ieiomyosarcoma or fibrosarcoma, and IastIy most of the cases of Iymphosarcoma occur on the Iesser curvature in the Iower segment of the stomach, where, when the stomach is mobiIized, the tumor may be resected and the continuity of the gastrointestinal tract be re-estabIished. In our review of the literature it was found that more than 60 per cent of all the sarcomas of the stomach are Iymphosarcomas, and of these 50 per cent are maIignant Iymphocytomas, and 50 per cent are reticuIum ceI1 Iymphosarcomas. An important statement recentIy made by Desjardins28 was “That if Iymphosarcoma couId be diagnosed earIy, most of them couId be cured by radiation therapy aIone.” On this statement hinges the crux of the treatment of a11 tumors of the stomach, that is, earIy diagnosis. This is one of the perpIexing probIems of the medica profession, inasmuch as earIy diagnosis is diffIcuIt to accompIish because of the many factors which are hard or impossibIe to contro1. The first of these is the patient, who through medica education must be directed to the doctor for any digestive disturbance earIy in its course, hence eliminating the deIay of the period of self medication. The second factor, is the doctor, and the one mostIy at fault. This fact we are impressed with in reviewing the Iiterature and noting in the case reports, the Iong interva1 that eIapses between the

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time the patient first seeks medica attention and the date of instituting adequate medical help, namely, surgical intervention. EarIier diagnosis can be made onIy by a thorough and painstaking history, a complete physica examination, (which is usuaIIy negative), foIIowed by gastroscopic study and a radiological check-up. We beIieve that the greatest aid in making a diagnosis earIier wiII come through advancement of gastroscopic study, inasmuch as the involvement of the stomach wall by a Iymphosarcoma throws up rugae which are markedIy exaggerated and have been described as IobuIated without invoIvement of the mucosa. This finding, together with an x-ray deformity in an individua1 with a gastric complaint may Iead to the diagnosis of a neopIasm of the stomach. In the presence of these findings, a Iaporatomy is indicated and justified, since surgery suppIemented by roentgen therapy can and does offer these patients a cure. In conclusion, we wish to point out that Iymphosarcoma is not a rare condition, that it occurs in from I to 2 per cent of a11 gastric maIignancies, that it occurs at an earIier period of life than does carcinoma, that it metastasizes late and that it responds more favorabIy to surgery than does carcinoma, even when the Iesion appears hopeIess from a roentgenoIogica1 and gastroscopic viewpoint. REFERENCES I. SIBLEY. Quoted by Walton, A. J. SurgicaI Dyspepsias. zd Ed., p. 247. London, 1930. Edward ArnoId & Co. 2. BRUCH. Quoted by Pack, George T. and McNeer, Gordon. Sarcoma of the stomach. Ann. Surg., 101: 1206-1223, 1935. 3. MASSON, J. C. Ouoted bv Balfour, D. C. and McCann, J. C.Sarcoma”of the stomach. Surg., Gynec. +Y Obst., 50: 948953, 1930. 4. HAGGARD, W. D. Sarcoma of the stomach. Surg., Gynec. Ed Obst., 31: 505, 1920. 5. ASKEY, E. V., HALL, E. M. and DAVIS, K. S. Sarcoma of the stomach. West. J. Surg., 39: 830, 1931. 6. CAMERON, A. L. and BRESLICH, P. J. Surgery, 9: 916930, 1941.

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7. SMITHIES and OCHSNER. Cancer of the Stomach. P. 412. PhiIadeIphia, 1916. W. B. Saunders &Co. 8. PACK, GEORGE T. and MCNEER, GORDON. Sarcoma of the stomach. Report of nine cases. Ann. Surg., 101: 1206-1223, 1935. 9. CHEEVER, DAVID. CIinicaI aspects and treatment of primary Iymphosarcoma of the stomach and intestines. Ann. Surg., 96: 91 r-923, 1932. IO. WALTERS, WALTMAN, GRAY, HOWARD K. and PRIESTLEY, JAMES T. Malignant lesions of the stomach. J. A. M. A., 117: 1675-1680, 1941. I I. Cook County Hospital, Chicago. Statistica Data, Department of PathoIogy. Dr. Waiter SchiIIer, Director. 12. EWING, JAMES. NeopIastic Diseases. 3d Ed. PhiIadeIphia, 1928. W. B. Saunders & Co. 13. ZIESCHE and DAVIDSON.Mitt. a.d. Grenzgeb. d. Med. u cbir., 20: 377, 1907. Quoted by Haggard, W. D. Sarcoma of the stomach. Surg., Gynec. @ Oh., 31: 505, 1920. 14. ZAPH, S. O., OLIN, H. A. and KIRSHBAUM, J. D. Lymphosarcoma of the stomach; cIinica1 and roentgenoIogica1 aspects. Am. J. Surg., 36: 476 486, 1937. 15. EUSTERMAN, G. and BALFOUR, D. C. The Stomach and Duodenum. PhiIadeIphia, 1935. W. B. Saunders & Co. 16. FREEMAN, L. PartiaI gastrectomy for peptic ulcer and Iymphosarcoma of the stomach with recovery. Cal. Med., 2: 362, 1928. I 7. KOUCKY, JOHN D., BECK, WILLIAM C. and ATLAS, J. Acute perforation of Iymphosarcomatous uIcer of the stomach. Ann. Surg., 114: 1112-1116, 1941. 18. MCNEALY, R. W. and HEDIN, R. F. Perforation in gastric carcinoma, study and report of 133 cases. Surg., Gynec. ti Obst., 67: 818-823, 1938. 19. MADDING, GORDON F. and WALTERS, WALTMAN. Lymphosarcoma of the stomach. Arch. Surg., 40: 120-134, 1940. 20. KRUMBHAAR, E. B. The Iymphatoid diseases. J. A. M. A., 106: 286-291, 1936. 21. FINLAYSON, J. Case of sarcoma of the stomach in a chiId aged 334 years. &it. M. J., 2: 1535. 1899. 22. HUNT, VERNE C. Partial gastrectomy for Iymphosarcoma in childhood. Ann. Surg., 96: 21*214, 1932. 23. GOSSET. Presse m&d., 221, 1912. Quoted by Haggard, W. D. Sarcoma of the stomach. Surg., Gynec. @y Obst., 3 I : 505, I 920. 24. BALFOUR, D. C. and MCCANN, J. C. Sarcoma of the stomach. Surg., Gynec. @ Obst., 50: 948-953, 25. Hr%%, G. W., DRESSER, R. and CAMP, J. D. Lymphoblastoma. Radiology, 7: 44-50, 1926. 26. GIER~, C. NORMAN. Lymphosarcoma; diagnosed gastroscopicaIIy. J. A. M. A., I 17: 173-175, 1941. 27. JONES, T. E. and CARMODY, M. G. Lymphosarcoma of stomach: report of case with nineteen year surgicaI cure. Ann. Surg., IOI : I 136-1138, 1935. 28. DESJARDINS,A. U. Quoted by Eusterman, G. and BaIfour, D. C. The Stomach and Duodenum. PhiIadeIphia, 1935. W. B. Saunders 8r Co.