Leiomyosarcoma of the duodenum

LEIOMYOSARCOMA J_

A.

HEYMANN,

M.D.

OF THE DUODENUM AND GORDON

11‘icbita Falls, Texas

T

HE duodenum is rareIy the seat of benign or malignant tumors. Although carcinoma of this region comprises about 3 per cent of the tota intestinal carcinomas, occasionally mahgnant tumors of the smooth muscle are encountered. Leiomyosarcoma follows a rather slow, progressive, malignant clinica course; hence these lesions offer a spIendid opportunity for surgica1 eradication. A review of the Iiterature shows that onIy sixteen cases have een reported’-I4 including the author’s case.* A study of the cases of this rare lesion reported during the Iast ten years would suggest that it is being diagnosed with greater accuracy and that the surgica1 management has greatly improved. A preponderance of cases occurs from the fourth to sixth decades of life and is more apt to affect the maIe than the femaIe in the ratio of 3 : 2. It has been suggested by Boyce and McFetridge ‘5 that trauma might be an etiologic factor. The symptoms of Ieiomyosarcoma of the duodenum resembIe in a genera1 way those of sarcoma of any other part of the small intestine. They- are chiefly pain, Ioss of weight, weakness and anemia. If the growth arises from the outer muscuIar layer, the tumor may become very Iarge and may be palpated early. If it arises from the inner coat, there is usually early bleeding into the intestinal tract. Diarrhea does not usuallv occur with sarcoma of the duodenum unless-it involves the ampuIIa of Vater; then it has the characteristics of disturbed pancreatic secretions. The diarrhea of intestina1 sarcoma elsewhere than in the duodenum is due to irrita* It is possible that other cases have been reported between ,944 and 1947, but the available literature did not reveal any. Harrington’s case reported in 1940 was operated in 1938. Later in 1940 C. W. hlayo reported all the cases of duodenal malignancy on record at the Rlayo Clinic. Two of these were Ieiomyosarcoma, and it is assumed that he reported the case listed above by I Ial-rington, but not counted in this report.

G.

CLARK,

M.D.

Iowa Park, Texas tion and hypermotiIity of the bowe1 and does not show evidence of pancreatic dysfunction. Intrinsic growths of the duodenum obstruct sufficientIy to cause pain, but never intussusception because they are fixed. High fever, jaundice and other symptoms are reported; however, these are not common. Perforation has been reported in two cases. The exact nature of this Iesion cannot be determined unti1 the abdomen is opened. A Iarge growth paIpated in the right upper quadrant should arouse suspicion. During the Iast few years considerabIe emphasis has been pIaced on the diagnosis of Iesions of the smaI1 intestine by means of x-ray. Feldman,‘6 Weintraub and Tugglen and others have emphasized this point. Positive x-ray findings plus the symptoms previousIy enumerated suggest a provisional diagnosis. Pathologists differ as to the origin of these tumors. It was suggested by ConheimlR that they deveIop from embryonal ceI1 rests. Obendorferlg beIieves that they may arise from benign Ieimyomas, whiIe Ewing20 states, “ It is not to be assumed without adequate proof that a malignant myoma represents the transformation of a previous benign tumor. Many and probabIy a11 these tumors are malignant from the first.” They tend to remain Iocahzed longer than other types of sarcoma of the bowel. The major part of them are subserous and may grow very large. The smaIIer submucous growths cannot grow very large before they produce symptoms requiring intervention. A few have been reported as peduncmated. Even the more frequent sessile type is not invasive. These growths are pale, noduIar, rather firm and tend to ulcerate early. The microscopic picture appears in the pathoIogic description of the case reported hereafter. The treatment is surgica1. When discovered earIy some of the growths may be excised Io-

120

I

2

FIG. I. Anteroposterior view; arrow is pointing into defect caused by tumor. FIG. 2. Oblique view; arrow is pointing into defect caused by tumor.

tally, but any surgeon approaching them should be thoroughly familiar with and capabIe of performing a pancreaticoduodena1 resection. The operation perfected by Whipple and Parsons21 or some modification thereof is the operation of choice. Every attempt shouId be made to preserve the external secretions of the pancreas. CASE

REPORT

B. B. (No. 8785), a white, married femaIe aged forty-two, was admitted to the Wichita General HospitaI on December 28, 1947, because of gradual weakness and failing strength. She stated that she had recentIy lost 4 pounds in weight. She had no pain or indigestion but had recently noted tarry stooIs. Her mother died from peIIagra and her father from “throat trouble.” Five brothers and two sisters were Iiving and well. There was no familial history of cancer or tuberculosis. Two years before the patient was admitted to the Wichita Genera1 HospitaI by one of the authors (G. G. C.), with a history of loss of strength and indigestion. She complained of miId epigastric pain which was relieved by soda and the ingestion of food. At that time she had tarry stooIs. GastrointestinaI x-ray fiIms were suggestive of a gastric uIcer on the Iesser curvature just above the pyIoric sphincter. The duodenum and the remainder of the intestinal tract were reported as normal. Her blood count was 2,400,000 red blood cells with 45 per cent hemoglobin, and ro,ooo white bIood cells with 78 per cent poIymorphonucIears and 22 per cent Iymphocytes. She remained in the hospital eight days and was given a diet and four blood transfusron of 500 cc. each. On discharge the red blood count was 4,450,ooo with 80 per cent he-

moglobin, and the white bIood count was 7,200 with 73 per cent poIymorphonucIears and 27 per cent Iymphocytes. The urine was normal. She did fairIy we11 until December, 1947, when she returned with the symptoms already retated. PhysicaI examination reveaIed a bright but paIe and underweight female 62 inches in height. Her norma weight was 105 pounds but her present weight was IOI pounds. Her temperature was 98.2%. and blood pressure 104/76. Examination of the head, neck, heart and Iungs revealed nothing abnormal. Her abdomen was flat and there was no tenderness. To the right of the midline about 2 inches beIow the gaIIbIadder a movable, elongated tumor was palpable. It was about 5 cm. long and 2 cm. in diameter. Her liver was not paIpabIe. The remainder of the physical examination was negative. Red blood count was 3,280,000, hemoglobin 7.3 gm., white blood count 5,700, Gg per cent polymorphonuclears, 26 per cent lymphocytes, 2 per cent eosinophiIes and 3 per cent monocytes. The urine was normal but the stools contained occult blood. The roentgenologic report revealed that the chest and heart appeared normal with the exception of a tenting of the left diaphragm due to adhesions. The esophagus, stomach and duodena buIb appeared normal. There was, however, a marked irreguIarity in the second part of the duodenum. The mucosa was definitely disturbed. The duodenal wall appeared fixed and there was a ragged filling defect involving about z inches of the descending duodenum. (Figs. I and 2.) These changes were strongIy suspicious of a malignant neoplasm of the duodenum despite the fact that such a condition is quite rare. American

Journal

of Surger)

Heymann,

CIark-Leiomyosarcoma

A severe inflammatory process might produce the changes described. There was no obstruction. Further study of the colon revealed nothing significant. Preoperatively, the patient was kept under observation for ten days. She received two blood transfusions of $00 cc. each. Exploratory operation was advised. On January 8, 1948, under pentothal@ and cycIopropane anesthesia (with curare) the abdomen was opened through a long right paramedian incision. Exploration revealed a normal liver, spIeen, pancreas, kidneys, coIon and peIvie organs. On the anterior surface of the duodenal bulb there was a peritonea1 scar suggesting previous uIcer. No enlarged nodes were found. The second part of the duodenum revealed a growth about 4 cm. Iong and about 2)s cm. in diameter. It extended from about I cm. below the ampulla downward. An attempt was made to mobiIize the duodenum, excise the growth and perform an end-to-end anastomosis; however, because of its proximity to the ampulla a pancreaticoduodenal resection was selected. The duodenum and head of the pancreas were easiIy mobilized, but in the process of mobiIizing a rather large uncinate process of the pancreas, the superior mesenteric vein was torn. It was closed with fine silk sutures without difficulty. The common bile duct was small and diffrcuIt to mobilize. It was cut and doubly ligated with siIk. The duodenum was then severed just proximal to its disappearance beneath the mesenteric vesseIs, and the Iower end cIosed with two rows of No. oo chromic sutures reinforced with tags of fat. The pancreas was severed through its neck, and the parenchyma closed up to its duct with fine silk sutures. The stomach was then amputated just proximal to the pyloric sphincter. A loop of jejunum was brought out and an entero-anastomosis done with two rows of No. oo chromic sutures. The stomach was anastomosed to this loop (end-toside) with two rows of No. 00 chromic catgut. Just above this the neck of the pancreas was anastomosed (end-to-side) with fine interrupted silk; the gallbladder was anastomosed to the proximal part of the Ioop with two rows of No. oo chromic sutures. (Fig. 3.) The wound was cIosed in layers with No. o chromic catgut, four tension sutures and dermal sutures. A rubber tissue drain was brought out through a stab wound. The operation lasted three and a half hours. The patient was given

July,

1950

of Duodenum

121

FIG. 3. Diagram of technica procedure; GB, gallbladder: C, common duct: S. stomach: P. oancreas: EA, entwokstomosis; Pi, proximal loop ,? jejunu,, and third portion of duodenum.

1,500 cc. of bIood during the course of the operation; at the close her puIse was 104 and blood pressure 120/70. The pathoIogic report reveaIed the following: GrossIy, the specimen consisted of 13 cm. of duodenum, the major portion of which was IargeIy replaced by a roughIy ovoid, cauIifIowerlike, uIcerated tumor. The ulcerated surface was hemorrhagic and necrotic. The bordering mucous membrane was thickened and roIIed but couId easily be peeled from the growth. The papiIIa of Vater was not demonstrable. On section the tumor presented a more or less diffuseIy uniform, fleshy and somewhat granuIar appearance. The growth was of rubbery consistency and appeared to merge with the muscuIaris of the intestine. The serosa was smooth and vascularized but apparentIy not infiItrated by the lesion. Another smaI1 segment of intestine attached to a piece of pancreas 3 by 4 by 5 cm. in diameter was present. In this specimen the ampulla of Vater was identified. Microscopically, the surface of the specimen showed extensive granuIating uIceration and a wide zone of hemorrhagic necrosis. The neoplasm presented a diffuseIy and highly cellular structure of elongated and fusiform cells. In areas the ceIIs were reguIarIy pIaced and resembIed smooth muscIe. EIsewhere the ceIIs were chaotically disposed and their processes interlaced indifferently. The nucIei varied from elongated rods to roughIy ovoid in shape. The nuclei aIso showed considerable variation in hyperchromatism. In the

Heymann,

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4

5

FIG. 4. Leiomyosarcoma merging with muscularis mucosa. FIG. 5. Higher magnitication of neoplasm showing mitotic

chaotic areas hyperchromatism was marked, anisonucleosis was present and occasional mitotic figures occurred. At the borders of the tumor the neoplastic cells merged inseparably with the muscularis of the bowel wall. (Figs. 4 and 5.) The diagnosis was leiomyosarcoma, grade 2. PostoperativeIy, nothing was given by mouth for ninety-six hours. Wangensteen suction was instituted. During the first ninety-six hours the patient was given two blood transfusions of 250 cc. each and was given IL. of 5 per cent gIucose every eight hours (one of these with saline each twenty-four hours). On the second day the drain was removed and profuse watery drainage followed. This ceased entirely after a few hours, however. The patient’s condition remained good at all times, her maximum temperature and the maximum pulse rate I IO. being 100.6’~. On two occasions folIowing administration of glucose she went into mild hypoglycemic shock, evidenced by profuse sweating. This ceased when amigen was substituted for glucose and given very slowly. After suction was discontinued the patient was pIaced on smaII amounts of Iiquid nourishment. This was rapidly repIaced by solid food. On the eighteenth postoperative day her red blood count was 5,460,ooo

X loo. figures.

X 560.

with hemogIobin 14.5 gm. The blood sugar was I 17 mg. per cent. She left the hospital on the nineteenth postoperative day after being up and about for aImost one week. The patient progressed nicely for about six months. She then showed evidence of liver metastasis in the Ieft Iobe. The upper part of the abdomen rapidIy IiIIed with the growth and the patient expired on May 15, 1949, a little over sixteen months after the operation. No autopsy was obtained. CONCLUSIONS

Leiomyosarcoma is a rare lesion of the duodenum, only sixteen cases having been reported including the present one. It is amenabIe to successful surgica1 management, particuIarIy when it grows into the lumen, because the symptoms and x-ray findings are such that it can be diagnosed reasonabIy early. It can be removed IocaIIy when properIy situated or by pancreaticoduodenal resection when the ampuha is invoIved. The patient’s nutritional state can be maintained at a higher IeveI if the external secretions of the pancreas are preserved. Acknowledgment: We iwish to thank Dr. Joseph G. Pasternack for his helpful suggestions in the preparation of this paper and for his as-

American

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Heymann,

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sistance in preparing the photomicrographs, and Miss Lou LeBus for her aid in assembling the material. REFERENCES

SALIS, H. A. Sarcoma of the duodenum, especiaIIy myosarcoma. Deutsche Ztschr. j. Cbir., Iho: 180, 1920. 2. BRDICZKA, I. G. Die Tumoren des Bulbus Duodein und ihre RontgenoIogische Diagnose. Kiintgenpraxis, 3: 625-629, 1931. 3. ANDERSEN, D. H. and DOUB, E. F. Leiomyosarcoma of the duodenum. Author’s reprint (with additions) from Arch. Path., 16: 795-802, 1933. 4. SILVERTONE, M. Sarcoma of the duodenum. Bit. J. Surg., 22: 332-336, 1934. 5. KLOPP, E. J. and CRAWFORD, B. L. Leiomyosarcoma of the smaI1 intestine. Ann. Surg., IO I : 726 I. VOX

733. 1935. 6. SEYMOUR,W. J. and GOULD, S. E. Leiomyosarcoma of the duodenum. Am. J. Cancer, 28: 572-578, 1936. 7. FOSHEE, J. C. and MCBRIDE, W. P. L. Leiomyosarcoma of the duodenum. J. A. M. A., I 12: 2497-25oop ‘939. 8. ITIKAWA, H. Leiomyosarcoma Duodenei. Arch. f. jap. Chir., 17: 1270-1271, 1940. 9. HARRINGTON,S. W. and GANSHORN, J. A. Leiomyosarcoma of the third portion of the duodenum. Proc. Staff Meet., Mayo Clin., 15: 75-76, 1940.

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