Primary carcinoma of the duodenum with a report of eleven cases

PRIMARY CARCINOMA OF THE DUODENUM* WITH

A REPORT CLYDE

Surgeon-in-Charge,

I.

OF ELEVEN ALLEN,

months;

and the Ioss of 27 pounds in five months. Physical examination showed moderate jaundice; evidence of Ioss of weight; the Iiver enIarged three fingerbreadths below the Costa1 margin. X-rays of the stomach and duodenum were negative, but the stoo1 was positive for occuIt bIood. The pre-operative diagnosis was acute exacerbation of chronic ChoIecystitis, probabIy with stones. Course. After a period of preparation, operation was done on June 26, 1930, and a large distended gall-bladder was found. Because of the patient’s poor condition a choIecystostomy onIy was performed, with a second operation seven weeks Iater when the patient’s genera1 condition had improved. The gaIIbIadder was removed and the common biIe duct was found to be greatIy enIarged. A mass, beIieved carcinomatous, was found, in what was thought to be the head of the pancreas, surrounding the termina1 portion of the common bile duct. The common biIe duct was drained. The patient died ten days after operation. Post-Mortem Examination. At the autopsy the fohowing features were observed: (I) a mixed squamous ceI1 and adenocarcinoma of the ampulla of Vater and the duodenum; (2) metastatic carcinoma in the regional Iymph gIands, Iiver and pancreas; (3) obstruction of the pancreatic and common bile duct. The tumor of the ampuIIa was a friabIe mass which extended through the papiIIa onto the surface of the duodenum as a Aat granuIar tumor 2 cm. in diameter. Comment. This condition was thought at operation to be primariIy a carcinoma of the but the diagnosis was proved by pancreas, autopsy to be a mistaken one. The inoperabiIity was demonstrated. CASE II. M. B., a female 62 years of age, was admitted to hospita1 JuIy 17, 1930, with compIaints of jaundice and pain in the right

rarity of carcinoma of the duodenum has been repeatedIy emphasized. WhiIe it is not common, the fact that eIeven cases were diagnosed in this clinic in the Iast ten years and that similar experiences have been reported in other cIinics, shouId remove it from the Iist of diseases thought hardIy to exist and pIace it among those for which some more generaIIy accepted attempt should be made at a diagnosis and cure. It has been reported by Eger’ and confirmed by others, that carcinoma of the duodenum is found in approximateIy 0.03 per cent of a Iarge series of autopsies. During the time of appearance of our eIeven cases, 154,613 patients were seen. This means that one case occurred for approximateIy each I 4,000 admissions to the cIinic. The accepted rarity of the condition has resuIted in too little attempt being made at diagnosis and perhaps, as suggested by Eger, in a Iack of decision on the part of the operator when the condition is unexpectedIy encountered. These tumors may occur in any portion of the duodenum, but are much more prevaIent in the region of the papiIIa of Vater. If we may consider these eIeven cases as representative, a carefu1 anaIysis of them shouId give an accurate picture of the condition. The first six cases have previousIy been reported by Mateer and Hartman.

CASE I.

admitted

REPORTS

S. R., a maIe, 44 years of age, was

to hospitaI May 26, 1930. He com-

pIained of: weakness for five months; and pain in right upper quadrant

Henry Ford HospitaI

MICHIGAN

HE

CASE

M.D.

Division of Genera1 Surgery, DETROIT,

T

CASES

jaundice for four

* From the Division of Genera1 Surgery, 89

Henry

Ford HospitaI.

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upper quadrant and epigasttiium of three months’ duration; weight Ioss of 25 pounds in three months, associated with anorexia and occasiona vomiting. the patient was thin, On examination, emaciated, jaundiced and weak. The Iiver was paIpabIe four fingerbreadths beIow the right costal margin. Rectal examination reveaIed a paIpabIe mass which was diagnosed as an adenocarcinoma, type 1, foIIowing biopsy. X-rays of the stomach and duodena1 cap were negative. Course. It was considered that the enIargement of the Iiver and biliary obstruction were due to a metastatic carcinoma from the rectum and that the condition was inoperabIe. The patient was, therefore, discharged from the hospita1. She was readmitted one month Iater in extremis, and died the foIIowing day. Post-mortem examination showed: (I) adenocarcinoma of the papiIIa of Vater with obstruction of both pancreatic and biIe ducts; (2) metastatic carcinoma of the mesenteric and retroperitonea1 Iymph gIands; (3) metastatic carcinoma of the liver and Iungs; (4) papiIIary adenocarcinoma of the rectum. Comment. This patient’s condition was considered inoperabIe and this was proved to be true at autopsy. The presence of an adenocarcinoma of the rectum, coincidenta with the carcinoma of the papiIIa compIicated the diagnosis. CASE III. E. H., a femaIe of 63, was admitted to hospita1 December 13, 1930, compIaining of Ioss of 80 pounds in four or five months; anorexia, nausea and vomiting for seventeen days; pain in the Iower quadrants of abdomen. Examination of the patient showed a moderate jaundice. She was obviousIy in extremis, had generahzed abdomina1 tenderness, and showed obvious Ioss of weight. Her white bIood count was 24,.500. The diagnosis made was probabIe generaIized carcinomatosis with acute generalized peritonitis. Course. The patient’s condition permitted neither further studies nor operation and she died the day folIowing admission. Post-Mortem Examination. A u t o p s y showed: (I) acute generaIized peritonitis secondary to rupture of galI-bIadder; (2) chronic choIecystitis and choIeIithiasis; (3) chronic interstitial pancreatitis; (4) adenocarcinoma

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of the papiIIa of Vater. No metastatic tumor was found. Comment. No more definite diagnosis couId be made in the case of this patient than generaIized peritonitis, since her poor condition did not permit any type of operation. The carcinoma of the duodenum was simpIy an interesting autopsy finding. CASE IV. G. M., female, 32 years of age, was admitted to hospital December zg, 1930. Two and a half years previousIy she had had a choIecystectomy eIsewhere because of what was diagnosed as galI-bIadder coIic accompanied by jaundice and a Ioss of I IO pounds. A IittIe over a year Iater a second operation was done eIsewhere, and a mass in the termina1 portion of the common biIe duct was found. This was removed and the biIe duct was anastomosed to the duodenum. On admission she compIained of tightness of the epigastrium which had been present for five months; anorexia of three months’ duration, vomiting which had been present for a few weeks; loss of IO pounds in the preceding thirty days. She had upper abdomina1 discomfort with the vomiting but had noted no jaundice. A firm mass couId be feIt in the right upper quadrant of the abdomen extending down to the IeveI of the umbiIicus. There was obvious weight 10~s. X-ray examination of the stomach showed a sIight irreguIarity in the antrum and the duodena1 cap did not fiI1 weI1. FIuoroscopicaIIy and on a11 the fiIms there was aIso noted a narrowing of the duodenum. There was persistent occuIt bIood in the stoo1. A diagnosis of probabIe carcinoma of the duodenum in an inoperabIe stage was made. This was based on the history, cIinica1 findings, the x-ray defect in the duodenum and persistent bIood in the stool. Course. The patient Ieft the hospita1 and returned three months Iater because of vomiting, bIood in the stool, and jaundice. She graduaIIy faiIed and died two months later. At post-mortem examination the following were noted: (I) primary adenocarcinoma of the papiIIa of Vater; (2) regiona1, mesenteric and retroperitonea1 metastatic adenocarcinoma; (3) hemorrhage into the intestina1 tract from the tumor; (4) muItipIe abscesses of the Iiver. of probabIe carComment. A diagnosis cinoma of the duodenum in an inoperabIe Five months Iater this stage was made.

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patient died amd autopsy reveaIed an inoperabIe condition. It is of some interest to specuIate as to when dul:ing this patient’s more than

FIG. I. Case XI, x-ray

showing

two and one haIf year illness the carcinoma developed. CASEv. H. S., a maIe 71 years of age, was admitted to the hospita1 JuIy 24, 193 I. He had been operated on eIsewhere three months previously because of gaIIstones, choIecystostomy being done. Previous to that for three or four years he had had duI1 right upper quadrant discomfort. Since his operation his discomfort persisted. He had bIack tarry stooIs at times; weakness; anorexia and weight Ioss. Upon examination there was evidence of weight loss. A Iarge paIpabIe mass in the right upper quadrant Iay directIy beneath the scar of the previous operation. There was rather marked anemia, the hemoglobin being 41 per cent. There was occuIt blood in the stooIs.

of Duodenum

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X-ray examination showed the stomach to be negative, but there was stasis in the sectsnd portion of the duodenum with dMatation prc3X1-

Ming

defect

in duodenum.

ma1 to this; there was stiI1 retention of barium in the stomach after six hours. Carcinoma of galI-bladder was diagnosed. Course. At operation a large mass, invoIving the duodenum and gaII-bIadder and considered to be in an inoperabIe stage, was found. There were nodules in the Iiver. A portion of one of these was removed and found to be metastatic carcinoma. The patient died four days foIIowing the operation. Post-Mortem Examination. The findings were: (I) adenocarcinoma of the duodenum 13 cm. heIow the pylorus and 4 cm. beIow the papiIIa of Vater, a Iarge crater-Iike uIcer 6 cm. in diameter extending directIy into the head of the pancreas; (2) metastatic carcinoma of the pancreas, regiona and retroperitonea1 Iymph gIands, the Iiver and both Iungs.

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Comment. A tion was found the persistence drainage of the

AIIen-Carcinoma

compIeteIy inoperable condiat operation. Here especiaIIy, of tarry stooIs foIIowing the gaII-bIadder shouId have been

FIG.

2.

Case

XI,

of Duodenum

whitish noduIes in the Iiver. Biopsy from the Iiver reveaIed the presence of metastatic carcinoma. The condition was inoperable. The patient died on the ninth post-operative day.

showing the unopened portion removed.

the cIue to the discovery that the troubIe had not been corrected. The diagnosis of carcinoma of the duodenum shouId have been made rather than a diagnosis of carcinoma of the galI-bladder. CASE VI. W. A., maIe age 64 years, was admitted to hospita1 November 24, 1931. He had had epigastric pain at intervaIs of a few months for one and a haIf years. This had been more persistent during the preceding two months. Recently nausea without vomiting began to accompany the discomfort. There had been a Ioss of 8 pounds in three weeks. Examination. Revealed slight jaundice; hemogIobin of 80 per cent; stooIs positive for occult blood; Iiver edge paIpabIe three fingerbreadths below the right Costa1 margin. X-ray of the stomach and duodena1 cap was negative. The pre-operative diagnosis was biIiary obstruction probabIy due to a carcinoma of the ampulla. Course. Operation was done on December g, I g3 I. A hard firm mass was found in the region of the termina1 portion of the common biIe duct and the head of the pancreas, with greatIy enIarged Iymph glands about the cystic and common duct. There were muItipIe hard

APRIL,,938

of the duodenum

which was

Post-Mortem Examination. In the first portion of the duodenum, 6 cm. beIow the pyIorus and 2.5 cm. above the papiIIa of Vater, was a circuIar uIcerated excavation in the wall which extended into the head of the pancreas. In the base of this an eroded bIood vesse1 was found. This was diagnosed as adnocarcinoma. There was aIso metastatic carcinoma of the Iiver, pancreas and regiona Iymph glands. Comment. An inoperabIe condition was found foIIowing the making of an essentialIy accurate diagnosis. In those cases with the tumor Iocated as in this case, in the first portion of the duodenum, the best chance for remova exists. An earIy diagnosis before metastases have occurred is imperative. CASE VII. M. S., femaIe aged 36 years, was admitted to the hospita1 August 2, 1927. During the preceding year she had two attacks of right upper quadrant discomfort with associated jaundice. The second attack began six months before her admission and persisted unti1 admission. She had had frequent nausea and vomiting during the preceding six months. There was a weight Ioss of 30 pounds during the year. Examination reveaIed Ioss of weight, moderate visibIe jaundice, non-filling gaII-bIadder on

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of Duodenum

choIecystograms. The stooIs were positive for occuIt bIood. Chronic ChoIecystitis with stone in the common duct and partia1 intestina1 obstruction of undetermined cause were diagnosed.

FIG.

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75 per cent. No B biIe was obtained on transduodenal biIiary drainage. X-ray of the stomach and duodena1 cap was negative; x-ray of the second portion of the duodenum showed some

3. Case XI, showing the opened specimen

with the tumor

in profile.

Course. Th e patlent ’ ’ contmued to vomit. After a period of preparation she was operated A Iarge mass was found in the on (8/13/27). transverse coIon which seemed to ascend from the mesentery into the waI1. The condition was considered inoperabIe, but a paIIiative iIeostomy was done. The patient died on the eighth post-operative clay. Post-Mortem Examination. Autopsy showed: (I) adenocarcinoma of the papiIIa of Vater with (2) metastatic carcinoma bile duct obstruction; of the transverse coIon and iIeocoIic Iymph glands. Comment. The partia1 obstruction of the Iarge intestine, as a resuIt of the metastatic tumor, confused this picture. The condition had reached an inoperabIe stage. CASE VIII. F. M., a maIe of 53 years, was admitted to the hospita1 February 24, 1933. He had had jaundice of four weeks’ duration, pain in the upper abdomen intermittentIy for two months. There was a Ioss of 35 pounds in three months. Other compIaints were anorexia, nausea and vomiting intermittentIy for six to eight weeks. Examination showed obvious weight Ioss and moderate jaundice. The hemogIobin was

irregularity. The stooIs were positive for occuIt bIood. The pre-operative diagnosis was obstructive jaundice, probabIy due to ChoIeIithiasis. A diagnosis of carcinoma of the duodenum or head of the pancreas was considered. Course. The patient was operated on on March 3, 1933. GaIIstones were found in an enIarged gaII-bIadder, which was removed. PaIpation of the termina1 portion of the common biIe duct reveaIed what was thought to be induration in the head of the pancreas. A piece of tissue was removed for microscopic examination and this reveaIed a norma pancreas. The patient did satisfactoriIy for a few days, but died on the seventh post-operative day folIowing a massive intestina1 hemorrhage. Post-mortem examination reveaIed: (I) adenocarcinoma (type 2) of the duodenum at the papiIIa of Vater with obstruction of the common biIe duct; (2) aIso a hemorrhagic pancreatitis. Comment. Th e pre-operatrve examination of this patient resuIted in a secondary diagnosis of possible carcinoma of the duodenum. This was based upon the irreguIarity of the second portion of the duodenum in the x-ray examination, the occuIt blood in the stoo1 and the

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this was ob$;trUCti ive jaLmdice. At operation faiIed to reveal bol rne in mlind and paIpation prc :sence of the tumor except for the finding of

of Duodenum

APRILw8 I.

condition had reached an inoperabIe stage and a paIIiative gastroenterostomy was (Jane. This patient was discharged on June 29, 1933. She

FIG. 4. Case XI, showing the opened specimen with a probe passed through the papiIIa at the upper border of the tumor.

induration in the head of the pancreas. The correct diagnosis could have been arrived at by transduodena1 examination of the region of the papiIIa. CASE IX. A. D., a femaIe of 71, was admitted to the hospita1 May 26, 1933. Her compIaints were upper abdomina1 pain and distress for the past year, which had become worse for the preceding two months; weakness; anorexia and occasiona nausea for two months; induced vomiting for two months. There was a greatIy enIarged stomach with aImost IOO per cent retention at the end of six hours. The duodenum was not visuaIized. The stool was positive for occuIt blood. The preoperative diagnosis was a probable carcinoma of the pyIorus with obstruction. Course. The patient was operated on June I, 1933. An infiItrating growth involving the waI1 of the duodenum and obstructing it was found at a point 3 cm. beIow the pyIorus. A biopsy from a nodule in the Iiver was diagnosed as a metastatic adenocarcinoma, type 2. The

died at her home on February I 8, 1934, approximateIy eight and one-haIf months after her operation. Comment. Metastases were present in the liver and the condition therefore was inoperabIe except for the paIIiative gastroenterostomy. The non-visualization of the duodenum might have suggested the correct diagnosis. The cIoseness of the tumor to the pyIorus resuIted in perhaps excusabIe error. CASE x. E. H., maIe, aged 37 years, was admitted to the hospita1 December 4, 1933. He compIained of discomfort in the right upper abdomen present intermittentIy for six months, accompanied by heart burn; Ioss of 20 pounds in six months, accompanied by increasing weakness; jaundice of three weeks’ duration; intermittent diarrhea for the preceding two weeks. There was obvious weight Ioss, moderate visibIe jaundice. Stool examination reveaIed the presence of occuIt bIood. X-ray examination reveaIed narrowing and irreguIarity in the third portion of the duodenum. No B biIe was

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obtained on transduodena1 biliary drainage. Hemoglobin was 66 per cent. PossibIe carcinoma of the duodenum, was diagnosed, based on the x-ray findings, the presence of occuIt bIood in the stoo1, the history, the biIiary drainage findings and jaundice studies. Course. FolIowing a period of preparation, the patient was operated on December I 6, r 935. The galI-bIadder and common biIe duct were found to be greatIy enIarged. A large tumor mass invoIving the duodenum and extending from the region of the papiIIa down to the junction of the second and third portions was paIpated. The growth did not compIeteIy surround the duodenum but nevertheIess caused marked obstruction. There were muItipIe noduIes in the pancreas. The duodenum was opened for about one and one-haIf inches to aIIow visua1 examination. A large tumor mass invoIving the posterior wall of the duodenum in the region of the papiIIa and extending downward from it couId be seen and feIt. A smaI1 portion of the tumor was taken for microscopic examination and was diagnosed as adenocarcinoma, type 2. A cholecystenterostomy was done, it being considered that the condition was inoperabIe and that probabIy a gastroenterostomy wouId be needed later. However, the patient died on the fifteenth postoperative day as a resuIt of pneumonia. Comment. A correct pre-operative diagnosis was made, but the condition was deemed inoperabIe on expIoration of the abdomen. The diagnosis was definiteIy made by transduodena1 expIoration and biopsy. This is probabIy the onIy way that a correct diagnosis may be arrived at at operation. CASE XI. L. P., male, aged 47 years, was admitted to the hospita1 August 13, 1937. He had had stomach troubIe for one year, with a weight Ioss of 22 pounds. Right upper quadrant pain had been present for 3 days. During the attacks of preceding year he had severa diarrhea with Iight coIored stools, abdomina1 distress and generaIized weakness. Examination showed muscIe spasm in the right upper quadrant of abdomen, liver duIness 4 cm. beIow the right costal margin. The hemogIobin was 70 per cent. The temperature was normal. The stooIs were positive for occult bIood, and the icterus index was g. X-ray examination of the stomach and duodena1 cap was normaI; the gaII-bladder showed non-fiIIing on cholecystograms. A pre-operative diagnosis of chronic choIecystitis was made.

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Course. ChoIecystectomy was done on August 3 I, 1937, operation having been postponed because of the deveIopment of fever and tenderness in the region of the right kidney. The gallbIadder was found to be distended, the waIIs moderateIy thickened. No stones were present. It shouId be noted that the stomach and duodenum were paIpated for the presence of ulcer and none was found. Except for an unusua1 amount of post-operative vomiting for a period of ten days, convaIescence was uneventfu1. The patient was discharged from the hospital September 18, 1937. FoIlowing this he continued to vomit at intervaIs of a few days and on October 22, 1937, there was visibIe gastric peristaIsis. At this time he gave a history of having recently vomited food that he had eaten the day before, that he had Iost an additional 12 pounds, and that he continued to have coIicky abdomina1 pain. He was again admitted to the hospita1 on October 25, 1937. On admission 2600 C.C. of Auid and food were aspirated from the stomach. X-ray examination on the day foIIowing admission reveaIed a Iarge hypotonic type of stomach. The duodenal cap was Iarge and we11 fiIIed, but there appeared to be an obstruction in the second or third portion of the duodenum resulting in I 00 per cent retention of barium. Because of these findings a diagnosis of probabIe maIignancy of the duodenum was made. FoIIowing a period of preparation a second operation was done on October 28, 1937. Because of the above findings the duodenum was mobilized to permit adequate paIpation and when this was done a tumor mass couId be paIpated in the duodenum below the IeveI of the papiIIa and entireIy separate from the pancreas. A radical resection was done by Dr. R. D. McClure. It was at first feIt that the tumor was we11 below the point of entrance of the common biIe duct, but it was eventuaIIy determined that it was so close that resection of the duct was necessary. This was therefore done. The duodenum was cut across about 3 cm. above and beIow the tumor mass, I I cm. of the duodenum being removed. It is of interest to note, in the light of subsequent events, that a carefu1 search was made for the pancreatic duct but it could not be visualized nor couId it be found on the specimen which was removed. The stumps of the duodenum were inverted and held in pIace by mattress sutures of fine siIk. A choIedochgastrostomy and posterior gastroenterostomy were done. A stab wound was made in the right Aank and a cigarette

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drain was brought out through this from the region of the resection. The patient’s post-operative course was unusuaIly smooth for so invoIved a procedure. On the day folIowing the operation, however, there began to be a rather profuse drainage through the wound in the flank. This was coIIected by inserting a tube in the wound, the amount averaging from 300 to 600 C.C. daily. It contained trypsin and Iipase and obviousIy was aImost pure pancreatic secretion. During the subsequent period an attempt was made to reintroduce this pancreatic secretion aIong with his food, but this was onIy partiaIIy successfu1. The patient refused the food which contained the secretion after a period of about two weeks. During this time he was also given pancreatin. On two occasions tpta1 fat determinations were made of twenty-four-hour stoo1 specimens, in each instance with the patient on a diet caIcuIated to contain IOO Gm. of fat. One determination reveaIed 0.29 Gm. and the other 2.4 Gm. of fat residua1 in the stool. The patient’s appetite remained very poor and, because he seemed to be graduaIIy faiIing and the drainage from the pancreatic fIstuIa remained at a high level, he was again operated on by Dr. McCIure on December 13, 1937. It had been originaIIy thought that an attempt wouId be made to transpIant the pancreatic fistuIa. He was doing so poorly, however, that it was decided that something must be done before the fistuIous waIIs were suffIcientIy strong to permit transpIantation. In view of Dr. WhippIe’s reported success with Iigation of the pancreatic duct it was feIt that it might be possibIe to find this and Iigate it even though attempts to find it before had been unsuccessfu1. At operation the head of the pancreas could easiIy be brought into view but the duct was not seen. Scattered over the surface of the transverse coIon and of the adjacent mesentery were ffat, whitish growths of tumor tissue, one of which was removed from the mesentery and reported as being composed of the same type of ceI1 as the origina duodena1 tumor. These implantations were aImost innumerabIe but no evidence of other metastatic tumor was found. FaiIing to visuaIize the pancreatic duct a doubIe ligature of heavy silk was pIaced around the head of the pancreas with an aneurysm needIe about I>& inches from its right sided termination. This was tied snugIy. There resuIted an aImost compIete cessation of drainage from the fistula for the

APRIL,rg38

of Duodenum

first day foIIowing

operation, and after this there remained a marked diminution of drainage, it being onIy about 200 C.C. daiIy. He was discharged from the hospita1 on December 23, 1937, and has since been folIowed at his home. The amount of drainage gradualIy decreased to a few cubic centimeters daily. PathoIogic examination of the tumor removed resuIted in a diagnosis of meduIIary carcinoma. On opening the resected portion of the duodenum, there was found a hard area in the waI1 just beIoti the papiIIa, about 3 cm. in diameter, raised from the surrounding mucosa1 surface. The surface of this tumor mass was supe&iaIIy uIcerated in part and covered with typica mucosa in the remaining area. Sections through the tumor showed invoIvement of the entire duodena1 waI1 to the serosa1 coat. The tumor was composed of a delicate reticulum, between the strands of which tumor ceIIs of wideIy varied size and shape and staining characteristics were Iying. Only occasiona mitotic figures were seen. There was apparentIy no attempt at gIanduIar formation and the ceIIs appeared to be growing in singIe irreguIar columns. Sections were stained with siIver and trichrome stain and none of the tumor ceIIs showed any argentifflne characteristics. Comment. The first diagnosis of chronic choIecystitis seemed reasonabIy we11 justified by the findings on physica examination, the history and the non&Iling gaII-bIadder on the cholecystograms. The presence of the occuIt bIood in the stoo1 was, of course, not the resuIt of the chronic choIecystitis. The very fact that this patient’s duodenum was carefuIIy paIpated and the tumor not found at the first operation is iIIustrative of the diffIcuIty of diagnosis. The second x-rays, taken when the obstruction was more severe, cIearIy showed the duodena1 defect and assisted in accurate diagnosis. The unusua1 type of ceIIuIar structure of this tumor is of considerabIe interest. COMMENT

ON THE

ELEVEN

PATIENTS

These patients represent an age distribution from the fourth to the eighth decades.

The sex distribution is of no significance, there being six maIes and five fernares in the group. The tumors were Iocated as foIIows: SuprapapiIIary area, two cases; papdIary

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area, seven cases; and infrapapiIIary area, two cases. The predominance of tumors in the region of the papiIIa is in accordance with the findings of others. Symptoms. AI1 patients compIained of abdomina1 pain which varied from miId distress to severe coIic, and in aImost every instance there were additiona compIaints of anorexia, weight Ioss, nausea, vomiting, and, in seven instances, jaundice. ObviousIy these compIaints wouId lead to no more definite concIusion than that the patient probably suffered from a disease of the gastrointestina1 tract, and in some instances of biIiary obstruction. Examination. The examinations of these patients aImost uniformIy reveaIed evidence of weight 10s~. One patient was obese. There was a paIpabIe mass in the right upper abdomen in two instances. The stooIs were positive for the presence of accuIt bIood in the ten instances in which this examination was done. Seven of the patients were jaundiced. In a11instances x-ray examinations of the stomach were negative and in four instances onIy were duodena1 defects noted. Some of these patients had a fairIy marked secondary anemia and with others it was only moderate. Diagnoses. The pre-operative or antemortem diagnoses arrived at were as foI10~s: (I) carcinoma of duodenum, 2 cases; (2) carcinoma of ampuIIa or head of pancreas; (3) carcinoma of galI-biadder; (4) generaIized peritonitis and carcinomatosis; (3) metastatic carcinoma of Iiver from carcinoma of rectum, the Iatter condition being proved; (6) galI-bIadder disease with obstructing stones, three cases; carcinoma of duodenum was considered in one case; (7) chronic choIecystitis; (8) carcinoma of pyIorus with obstruction. A study of our own and of reported cases Ieads one to the concIusion that a preoperative or ante-mortem diagnosis may be arrived at in most instances by the excIusion method onIy. A filing defect on the x-ray fXm of the duodenum should be of the greatest heIp, but often is not visuaIized. Perhaps more carefu1 attention to the

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duodena1 area and more frequent fiIms wouId resuIt in more positive diagnoses as suggested by Startz.5 Herman and Von GIahn6 have pointed out that the finding of an approximateIy norma gastric acidity in a patient who might otherwise be thought to have carcinoma of the stomach shouId make one very suspicious of the presence of carcinoma of the duodenum. This fact may be of considerabIe vaIue in differentiating the two conditions when the x-ray examinations are inconcIusive. In those instances where the tumor resuits in biIiary obstruction, the differential diagnosis between duodena1 tumor, tumor of the pancreas and common duct stone is dif&uIt. AI1 three conditions may produce jaundice, pain, anorexia, nausea, vomiting and weight 10s~. Pain is much Iess common with the pancreatic tumors. Deformities of the duodenum on the x-ray Mm, of course, favor a diagnosis of tumor of the duodenum. The presence of occuIt bIood in the stoo1 wouId greatly favor the diagnosis of intestina1 tumors and is probabIy the most vaIuabIe sign of aI1. The diagnosis and the differential diagnosis of infrapapiIIary duodena tumors from those Iower in the intestina1 tract, can be made to a certain extent from the cIinica1 picture, but the most reIiabIe aid wiI1 be the x-ray fiIIing defects. Operations. Of the eIeven cases, three were rightIy considered inoperabIe. The remaining eight were operated upon. In two the tumor was not found at operation, choIecystectomies being performed. In five cases the masses invoIving the duodenum and adjacent structures were paIpated, but the condition was considered inoperable because of metastasis to the Iiver in four cases and to the pancreas in one. OnIy three paIIiative operations were performed, an iIeostomy in Case VII, a gastroenterostomy in Case IX and a choiecystenterostomy in Case x. OnIy one radical operation was done, that of Case XI, in which a resection of a portion of the duodenum with transpIantation of the common duct into the stomach

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and a posterior gastroenterostomy were carried out. Treatment. It is obvious that a Iarge number of these patients were seen only after the disease had reached an inoperabIe stage. In such cases paIIiative measures for the reIief of biliary or intestina1 obstruction wiI1 sometimes give some measure of comfort for the remaining weeks or months of Iife. The probIems that must be soIved in a successfu1 resection of a tumor of the duodenum are numerous and diffIcuIt. They wiI1 vary somewhat with the Iocation. It seems obvious, however, that they are most diffxcuIt for tumors in the papiIIary area. This, of course, is the Iocation of a Iarge percentage of duodena1 tumors. The pancreas, pancreatic duct or ducts and the common biIe duct must be deaIt with. Biliary drainage must be provided for by an anastomosis of either the common biIe duct or gaII-bIadder to the stomach or intestine. Provision must be made for emptying of the stomach in those cases continuity is intestina1 where the interrupted. In the reIativeIy recent Iiterature there have appeared papers by advocates of two for tumors in the types of operation papiIIary area. One method is that advocated by Hunt and Budd,4 in which a one stage operation with transduodenal resection of peri-ampuIIary tumors with reimpIantation of the biIiary and pancreatic ducts was advised. WhippIe, after unsatisfactory experience with one stage operation, has worked out a procedure, the saIient points of which are: I. No attempt at a re-estabIishment of the continuity of the duodenum foIlowing the resection. 2. A two stage operation which incIudes a posterior gastroenterostomy, section and Iigation of the common biIe duct and ‘a choIecystgastrostomy in the first stage. The second stage, which is carried out three or four weeks later, incIudes a Iigation of the pancreaticoduodena1 and gastroduodena1 arteries and a resection of the

of Duodenum

APRIL, 19x8

invoIved portion of the duodenum together with a v-shaped excision of a portion of the head of the pancreas. The pancreatic duct or ducts are Iigated. It wouId seem that the two stage operation has many things in its favor, the most important of which is that it makes possibIe improvement of the patient’s condition by the reIief of biIiary and intestina1 obstruction at the first stage. The radica1 departure is the deIiberate Iigation of the pancreatic duct. WhiIe it is difIicuIt to consider IightIy the aIteration of so vita1 a physioIogic process as the secretion of pancreatic juice, WhippIe’s experience with two patients on whom this operation was done wouId seem to justify it. He has shown that even with the Ioss of drainage of pancreatic secretion into the intestina1 tract, fat metabolism is diminished by onIy about IO to 15 per cent. Even in those instances where the galI-bladder is found unsuitabIe for anastomosis, the transpIantation of the common biIe duct wouId be considerably simpIifLed. The reIationship of ulcer to carcinoma of the duodenum remains uncertain. Arisz’ has reported two cases of carcinoma which apparentIy arose on the basis of ulcer. Jefferson8 reported another case and coIIected thirty additiona ones. Startz5 has written on the reIationship of uIcer and carcinoma and so has Hinton.g WhiIe one must readiIy admit the possibIe reIationship in those instances where an uIcer has been known to exist for a considerable period before the probabIe occurrence of carcinoma, the fact remains that an extremeIy smaI1 percentage of uIcers become!? carcinomatous. It can be said safeIy that uIcer is not a great predisposing cause, if there is any causa1 reIationship at aI1. Ewing,‘O however, does fee1 that carcinomas in the first portion of the duodenum are secondary to uIcers and states that in this particuIar group stenosis and adhesions are common and that metastases are earIy and widespread.

Allen-Carcinoma

NEW SERIES VOL. XL, No. I

of Duodenum

The cehular structure of duodena1 carcinemas is usually a cylindrical cell adenocarcinoma. All of our eleven cases except one showed this type of tumor. One, Case XI, as noted in the case report, showed an unusual type of cellular structure and was diagnosed as medullary carcinoma.

Reported

-

REPORTS

Year

by

OF

RADICAL

Syme.

‘904

Infra-ampullary

Dewis and Morse (Morse).

1928

Supra-ampullary

Muller and Rade. maker.

1931

Ampulla of Vater

193’

Infra-ampuhary

4. Schoheld.

I

OPERATIONS

Location of Tumor

99

This is the only instance in our eleven cases where the tumor seemed to have arisen in the ampulla. We have included it because it did reveaI a dehnite tumor of the duodenal wali and is a good example of the diffkulty so often expressed of determining the exact origin.

CHART ADDITIONAL

American Journal of Surgery

FOR

CARCINOMA

Operation

OF THE

DUODENUM

ResuIt

Pathology -

RadicaI resection and end-to-end anastom o s i s, duodenum and jejunum I. Gastrojejunostomy 2. RadicaI resection first part duodenum and pyIorus I. LocaI remova with cautery 1924 2. Cholecystoduodenostomy 1926 3. Gastroenterostomy 1927 I. Duodenotomy and cholecystoduodenot-

Carcinoma

Operative

Scirrhous adenocarcinoma

Living and we11 fifteen months later

CyIindricaI ceI1 Died four years and adenocarcinoma eight months after first operation of recurrence

Adenocarcinoma

Died twenty-nine post-operative dena IistuIa

Adenocarcinoma from pancreatic rests Carcinoma

Operative

Adenocarcinoma

Died seven days postoperative

Adenocarcinoma

Died six hours postoperative Living twelve weeks after operation

omy

days duo-

2. Duodenotomy

5. Bookman.

I932

Supra-ampuIIary

6. Eger (Lower).

1933

Infra-ampuhary

7. Davis.

1935

Infra-ampulIary

8. Hoffman-Pack

1937

AmpuIIary

1938

Infra-ampuIlary

(Pickhardt). 9. Our Case XI (MC. Clure).

and impIantation of rador Local excision of tumor and gastrojejunostomy Radical resection infra-ampuIIary portion of duodenum Radical resection and anastomosis second part duodenum to jejunum Excision of growth

recovery

RadicaI resection; choledochgastrostomy; posterior gastroenterostomy

MeduIIary cinoma

car-

recovery

Died six years later of undetermined cause

-

The other partial exception was Case I which showed, in addition to the adenocarcinoma, squamous cell carcinoma. This patient had a friable tumor which seemed to originate in the ampulla and spread through the papilla onto the surface of the duodenal wall as a flat, granular tumor 2 cm. in diameter.

Outerbridge, l1 in 1913, collected a series of I IO cases which he grouped under the of the Papilla caption of “Carcinoma of Vater.” He admitted that some of the tumors may have had their origin elsewhere, but felt that an exact histoIogic differentiation was impossible and in fact useless.

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Ewing”’ states that except in very earIy cases it is diffrcuIt to determine the exact origin, that is, whether it is from the Iining of the ducts or the waII of the duodenum at the papiIIa of Vater. He adds, however, that the jaundice in those cases due to carcinoma of the papiIIa is “Iess severe and persistent than with carcinoma of the ampulla.” In each instance the structure of the tumor is usuaIIy a cyIindrica1 ceI1 adenocarcinoma and the treatment identica1. The differentiation as to origin, then, is IargeIy one of academic interest onIy. We have reviewed reports of 193 cases. Fifty-nine of these were colIected and reported by Cohen and CoIp,12 twenty-two by WhippIe and eighteen by Hunt and Budd.4 This last group incIudes eIeven cases aIso reported by WhippIe. The three groups were made up of a tota of eightyeight cases, on whom some attempt at a radica1 cure of carcinoma in the periampuIIary region of the duodenum had been made. To these groups may be added an additional nine cases including our Case XI (Chart I). WhiIe no Iarge portion of this tota of ninety-seven cases was benefited for a Iong period of time, twentyfive were reported as Iiving for a period of from one to twenty-two years foIIowing operation. l3 Of the remaining ninety-seven cases, fifty-five had paIIiative operations and forty-two had either no operation or abdomina1 expIoration onIy. The remainder of the case records which we reviewed are Iisted in the bibIiography under numbers 14 to 42. The pathoIogic diagnoses in our cases were made by Dr. Frank W. Hartman. CONCLUSIONS I. The diagnosis of carcinoma of the duodenum is extremely diffrcuIt. 2. The symptom compIex of our cases was not diagnostic of anything other than a disease of the gastrointestina1 tract. 3. The presence of occuIt bIood in the stoo1, not otherwise accounted for, shouId make one suspicious of a duodena1 tumor. It is probabIy the most vaIuabIe aid in the

APRIL, 1938

of Duodenum

differentiation of benign and malignant disease. Disregard of it wiI1 Iead to mistaken diagnoses. 4. X-ray hIIing defects in the duodenum are heIpfu1 in diagnosis, but are often not seen. The deveIopment of a specia1 technique for examination of the duodenum seems essentia1. 5. The operative treatment of duodena tumors is an invoIved and diffrcuIt procedure. It is deserving of further deveIopment. I am indebted to Dr. R. D. McCIure assistance

for his and for the use of his cIinica1 records. REFERENCES

I. EGER, S. A. Primary mahgnant disease of the duodenum. Arch. Surg., 27: 1087-1108, 1933. 2. MATEER, J. G., and HARTMAN, F. W. Primary carcinoma of the duodenum, cIinica1 and pathologica aspects with differentia1 diagnosis. J. A. M. A., gg: 1853-1859, 1932. 3. WHIPPLE, A. O., PARSONS, W. B., and MULLINS, C. R. Treatment of carcinoma of the ampuIIa of Vater. Ann. Surg., 102: 763-779, 1935. 4. HUNT, V. C., and BUDD, J. W. TransduodenaI resection of the ampuIIa of Vater for carcinoma of the dista1 end of the common duct. Surg., Gynec., TV Oh., 61: 651-661, 1935. 5. STARTZ, I. S. DuodenaI carcinoma; its reIationship to duodena1 ulcer. Radiology, 25: 688-697, 1935. 6. HERMAN, N. B., and VON GLAHN, W. C. Carcinoma of the supra-ampulIary portion of the duodenum. Am. J. M.Sc., 161: rrr-rrg, rgzr. 7. ARISZ, L. DuodenaI uIcer with carcinoma. Acta Radiol., 13: 41-42, 1932. 8. JEFFERSON, G. Carcinoma of the suprapapiIIary duodenum casuaIIv associated with preexisting uIcer. Brit. J. Surg., 4: zag, 1916. o. / HINTON., J. W. Does carcinoma of the duodenum ever arise from duodena1 uIcers? Report of cases. Am. J. M. SC., 181: 843-850, 1931. IO. EWING, J. NeopIastic Diseases. PhiIa., 1922. W. B. Saunders Co. I I. OUTERBRIDGE, G. W. Carcinoma of papiIIa of Vater. Ann. Surg., 57: 402-426, rgr3. 12. COHEN, I., and COLP, R. Cancer of the periampulIary region of duodenum. Surg., Gynec., Ed Obst., 45: 332-346. 1927. 13. MULLER, G. P., and RADEMAKER, L. End-resuIts in radica1 operations for carcinoma of periampullar region of duodenum. Ann. Surg., 93: 755-760, 1931. 14. SYME, G. A. Carcinoma of duodenum, resection and recovery. Lancet, I : 148, rgo4. 15. DEWIS, J. W., and MORSE, G. W. Primary adenocarcinoma of the duodenum; report of 12 proved cases; summary of literature. New England J. Med., rg8: 383-401, 1928.

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16. SCHOFIELD,J. E. Carcinoma of duodenum. Bit. J. Surg., 18: 84-90, 1930. 1’7. BOOKMAN, M. R. Carcinoma of the duodenum originating from aberrant pancreatic cells. Ann. Surg., 95: 464-467, 1932. 18. DAVIS, C. R. Carcinoma of the duodenum; case report. Am. J. Cancer, 23: 337-338, 1935. 19. HOFFMAN, W. J., and PACK, G. T. Cancer of duodenum; cIinica1 and roentgenographic study of 18 cases. Arch. Surg., 35: I 1-63, 1937. 20. Cabot Case Records. Case report, primary adenocarcinoma of duodenum. Boston M. IY S. J., 194: 267-270, 1926. 2 I. DARDINSKI, V. J. Primary carcinoma of duodenum. Am. J. Patb., IO: 313-318, 1934. 22. DEAVER, J. B., and RAVDIN, I. S. Carcinoma of duodenum. Am. J. M. SC., 159: 469-477, 1920. 23. HARBIN, W. P., HARBIN, W. P. JR., and HARBIN, L. Primary carcinoma of duodenum. Ann. Surg., IOI : 961-965, 1935. 24. HEAD, G. D. Primary carcinoma of third portion of duodenum. Am. J. M. SC., 157: 182, 1919. 25. HERRICK, F. C. Obstruction at the terminal portion of the common biIe duct due to cancer of the ampulla. J. A. M. A., 47: 1438-1440, 1906. 26. JONES, G. W. Melanoma of ampuIIa of Vater. J. A. M. A., 96: 1682, 1931. 27. LISA, J. R., LEVINE, J., and FITZHUGH, W. M. Primary carcinoma of the duodenum. J. Lab. ti C/in. Med., 20: 150-154, 1934. 28. MCGUIRE, E. R. and CORNISH, P. G. Carcinoma of the duodenum. Ann. Surg., 72: 600, 1921. 29. MORRISON, T. H. and FELDMAN, M. Autopsy report of a case of primary carcinoma in duodena1 diverticuIum. Ann. Ch. Med., 5: 326329, 1926. 30. NAGEL, G. W. UnusuaI conditions in duodenum and their significance. Arch. Surg., I I : 529-549, 1925.

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31. POTTER, E. B. SuccessfuI resection of common biIiary duct for carcinoma of amp&a of Vater. Ann. Surg., 98: 369-373, 1933. 32. SALA, A. M. Pathologica rarities in cancer; an unusua1 case (geIatinous carcinoma). Radiology, 25: 437-439, 1935. 33. SWENSON, P. C., and LEVIN, A. G. Primary carcinoma of the duodenum; case report. Am. J. Roentgenol., 31: 204-207, 1934. 34. VICKERS, D. M. Carcinoma of duodenum. Ann. Surg., 79: 239-243. 1924. 35. WILSON, J. A. and NOBLE, J. F. An instance of geIatinous carcinoma of the duodenum. Am. J. Digest. Dis. @ Nutrition, I : 840-844, 1935. 36. YLVISAKER, R. S. Carcinoma of third portion of duodenum with brief review of literature. Minnesola Med., 12: 351-354, 1929. 37. MORRISON, T. H. and FELDMAN, M. Carcinoma in a duodenal diverticuIum with consideration of duodena1 diverticuIosis. Ann. C/in. Med., 4: 403-414~ 1925. 38. MEYER, J., and ROSENBERG, D. H. Primary carcinoma of duodenum; report of 4 cases, with review of Iiterature. Arch. Int. Med., 47: 91794’, 1931. 39. EUSTERMAN, G. B., BERKMAN, D. M., and SWAN, T. A. Primary carcinoma of duodenum, report of 15 verified cases. Ann. Surg., 82: 153-163, 1925. 40. STEWART, H. L. and LIEBER, M. M. Six cases of carcinoma supra-papilIary portion of duodenum. Arch. Surg., 35: 99-126, 1937. 41. COOPER, W. A. Carcinoma of ampulla of Vater. Ann. SUrg., 106: 1009-1033, 1937. 42. LIEBER, M. M., STEWART, H. L., and LL~ND, H. Two cases carcinoma of infrapapiIIary portion of duodenum. Arch. Surg., 35: 268-289, 1937.