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Leiomyoma of the duodenum
Leiomyoma
of the Duodenum
R. E. CAMPBELL, M.D. AND J. M. YOUNG, M.D., Diplomate, Memphis,
From tbe Surgical and Laboratory Services, Veterans Administration Medical Mempbis, Tenn.
Teaching
Group
ENIGN
CASE REPORTS CASE I. L. N., a twenty-five year oId white maIe construction worker, was admitted for the first time January 8, 1953, with the compIaints of tarry stooIs, weakness and dizziness. He had been in exceIIent heaIth unti1 one week before admission when a coId with non-productive cough deveIoped, foIIowed three days Iater by tarry stooIs. These continued at the rate of four a day for four days. Two days before admission he vomited undigested food containing cIots of dark bIood. No history of abdominal pain, discomfort or jaundice was obtained. He was a moderate drinker of aIcoholic beverages, consuming one or two pints of whiskey each week. On physical examination bIood pressure was 130/58, temperature IOI’F., puIse 130 and AmericanJournaloj Surgery,Volume
88, October,
rwo
Board of Patbology,
respiration 20. The patient was a we11 deveIoped, we11 nourished, paIe white maIe who appeared acuteIy iI1. Inspiratory wheezes and scattered rhonchi were present in the lungs biIateraIIy. The Iiver was paIpabIe one fingerbreadth below the right costal margin and was tender. The spleen was not paIpabIe. The laboratory reports can be noted in TabIe I. Bedside chest x-ray on admission was essentiaIIy normaI. On January 2rst x-ray study of the upper gastrointestinal tract showed an indentation of the Ieft IateraI margin of the stomach due to an enIarged spIeen. Esophagram and smaI1 intestina1 fiIms were within norma Iimits. On ffuoroscopy ten days Iater there was some diffIcuIty in fiIIing the duodenal buIb with rapid transit of the barium through this region. A smaI1 intestina1 series of fiIms was again normaI. The patient was immediateIy given whole bIood transfusions and received 9,500 cc. the first eight days, 4,000 cc. of which was given in the first three days. During this period his red bIood count rose to 3,800,000 and his hemogIobin to 10.4 gm. per IOO cc. The hematocrit reached 45 per cent. He was pIaced on a bIeeding ulcer diet, banthine@, ascorbic acid, calcium carbonate and procaine peniciIIin. After tweIve days his stooIs became brown in coIor and guaiac-negative. Esophagoscopy was negative. Caffeine gastric anaIysis was reported 18 mEq. free HcI in the haIf-hour specimen and 20 mEq. in the two-hour specimens. AI1 liver function tests were normaI. He was discharged on an ulcer regimen to return in two months for further observation and study. The patient remained we11 after discharge, but Iost 20 pounds in weight. One week before his second admission, April 28, 1953, sharp mid-epigastric cramping and a desire to defecate deveIoped after eating chiIi. He became nauseated and vomited, but the vomitus contained no bIood. His stooIs for the
Hospital,
and maIignant tumors of the small intestine are uncommon Iesions. In 1932 Raifordl reported a Iarge series of tumors of the gastrointestina1 tract in which there were fifty benign and thirty-eight maIignant Iesions arising in the smaI1 intestine. These fifty benign tumors constituted about 24 per cent of the entire benign group, whiIe the thirtyeight malignant tumors made up onIy 5 per cent of the maIignant group. Leiomyoma is the most common benign Iesion of the smaII intestine, and about 80 per cent of these occur in the jejunum and iIeum and the remaining 20 per cent in the duodenum.2 If one considers only the duodenum, adenomas are sIightIy more common than Ieiomyomas3 In 1952 Straus and 0’Kane4 coIIected fifteen cases of Ieiomyoma of the duodenum and added a case of their own. To this tota one can add nine cases reported by Olsen et aI., two by Oberhelman et aI., and two by Ebert et aI. We wish to report two other cases because of their rarity and some unusua1 features.
B
American
Tennessee
618
Leiomyoma
of Duodenum
next three days were hard and black. He began to have recurrent mid-epigastric pains on an empty stomach, relieved by milk. The pain frequently wakened him at night. Two days before admission the patient began to notice some change in skin color. TABLE SUMMARY
HEMATOLOGY
OF
perature came down to 99%. He then remained afebrile. He had received 3,500 cc. whole blood during this two-week period and showed no signs of bleeding after the seventh hospital day. The patient was definitely jaundiced at the I
LABORATORY
II
On physical examination blood pressure was 105/70, temperature IOO.~‘F., pulse 120 and respiration 18. The patient appeared acutely and chronically ill, and there was a sallow color to the skin. There were many “cafe au Iait” spots over the back and extremities. There were two small, freely movable, firm nodules, each 0.3 cm. in diameter, just under the skin, one in the left flank and the other over the right anterior chest. No definite jaundice was present. The liver was barely palpable and smooth. The spleen was enlarged three fingerbreadths below the left costal margin in the mid-clavicular line. There was tenderness to palpation in the right upper quadrant just to the right of the umbilicus. BIack stool was present in the rectum. The laboratory data are tabulated in Table I. During the first two weeks in the hospital the patient’s fever spiked to IOI’F. each day, and became progressiveIy more toxic. MultipIe blood cultures and enteric and heterophil agglutinations were negative. Penicillin and aureomycin were started after tweIve days, and graduaIIy over a six-day period the tem-
DATA,
CASE
BLOOD
I
CHEMISTRY
I
height of his fever on May 7, 1953, his tenth hospital day. Urine specimen was 4 plus for bile on the Harrison spot test. This cleared within two days. Following this his appetite increased, he became stronger, gained weight and became ambulatory. He no longer showed any signs of progressive disease, and his stools were negative for occult blood. Biopsy or one of the skin masses was reported as neurofibroma. Upper gastrointestinal roentgenograms and chest x-ray were normal, as were cholecystogram and intravenous pyeIogram films. On July 3, 1953, with the patient under endotracheal cyclopropane and curare anesthesia, an exploratory Iaparotomy was performed. A firm, rubbery tumor mass in the wall of the second part of the duodenum was found. This was encroaching upon the head of the pancreas and displaced the common duct. An attempt was made to remove the tumor by local excision, but this was not possibIe. A pancreatico-duodenectomy was required for removal of the tumor. Splenic enIargement was noted. During the operation there was generalized oozing of blood throughout the operative
Leiomyoma
of Duodenum
2
1
FIG. I. Case I. Anterior projecting
from
FIG. 2. Case tumor.
surface stomach, wall of duodenum.
I. Duodenum
opened,
duodenum
showing
sinus
and upper tract
leading
jejunum into
showing lumen
from
Iobulated central
tumor part
of
compIeteIy surrounded by the tumor. Most of the tumor Iay outside the duodena1 muscuIar waI1, but smaI1 Iobulations extended into the submucosa, especiaIIy near the entrance of the common duct and duct of Santorini. MicroscopicaIIy the tumor was composed of moderate-sized, uniform, spindIe-shaped ceIIs with abundant eosinophiIic cytopIasm and ovoid to round nucIei. No mitoses were seen. There was much necrosis and hemorrhage in the centra1 part. The tissue was very vascuIar with whorIing and interIacing of the fibers. (Fig. 3.) The tumor did not invade the pancreatic parenchyma. A Masson trichrome stain showed the tumor to be of muscIe origin. When the peritonea1 cavity was opened at autopsy, it contained 4,000 cc. of cIotted and liquid bIood. Dissection of the region of the duodenum discIosed no Iarge open vesseIs, but extravasation of bIood throughout the tissues was marked. The spIeen weighed 4go gm. and was very soft; the cut surface showed marked diflluence. The Iiver weighed 1600 gm. and was paIe brown in coIor. The choIedochojejuna1 anastomosis was patent and intact, as was the gastrojejunostomy. The remaining dista1 part of the pancreas weighed 6o gm. and measured 15 cm. in length. The anastomosis between the pancreatic duct and the bowel was intact.
field. The patient withstood the procedure well and received 1,500 cc. of whoIe bIood during the four and a half hour operation. Upon his return to the ward the bIood pressure was rzS/go and puIse 130. He remained thus stabiIized for three hours, when hisbIood pressure dropped to 1oz/80 and the puIse became 144. AIthough he was given 9,500 cc. of whoIe bIood and 9,000 cc. of intravenous fluids with neo-synephrine@ and norepinephrine added, he dropped into irreversibIe shock. In addition, he received adrenal cortex extract, and aqueous cortisone, desoxycorticosterone Iipo-adrenal extract without favorabIe response. He died twenty-seven hours after surgery. The gross specimen consisted of the distal portion of the stomach, the duodenum and the proximal portion of the jejunum. (Fig. I.) Attached to the second portion of the duodenum was a firm mass 4 by 6 by 5 cm. with the head of the pancreas Iymg directIy posterior. On section the tumor was a homogeneous mass of coarse IobuIated tissue having central hemorrhage and necrosis. There was a sinus tract leading into the duodenum. (Fig. 2.) The centra1 portion of the tumor lay 3.0 cm. above the ampuIIa of Vater and the inferior border was directIy adjacent to the common duct, displacing it and partly surrounding it near the ampuIIa of Vater. The duct of Santorini was
620
Leiomyoma
FIG. 3. Case muscle fibers.
I. Photomicrograph
of Duodenum
showing
interlacing
bundIes
of smooth
norma Iimits. StooI examination showed strongIy positive guaiac. On December 12th x-ray study of the upper gastrointestina1 tract revealed narrowing of the duodenum just beyond the buIb with a 2.5 cm. rounded defect at the beginning of the descending portion of the duodenum. BeIow this at the junction of the second and third portions of the duodenum, on the media1 contour, a 1.0 cm. projection with a narrow mouth was noted. RoentgenoIogic impression was that the fiIIing defect represented a tumor and the projection was either a diIated ampuIIa of Vater or a diverticuIum. The patient was pIaced on a bleeding MeuIengracht diet, bedrest, atropine and given whoIe blood transfusions. He vomited dark cIotted bIood and passed both Iiquid, dark, bIoody stooIs and formed, tarry stooIs. He received 6,500 cc. of whoIe bIood during the first three days, and during this time his red bIood count rose to 4, IOO,OOOand his hemogIobin to 12.2 gm. He showed no further signs of bIeeding after his third hospitaI day, and after six days his stooIs became brown in coIor and guaiac-negative. On December 27, 1949, with the patient under endotrachea1 cycIopropane and curare anesthesia, Iaparotomy was performed, revealing an ovoid mass about the size of a large marble just beneath the mucosa in the anterior waI1 of the duodenum. A smaI1 segment of the
Microscopic examination of the organs discIosed widespread infarction and necrosis throughout the Iiver, pancreas, spleen and kidneys, interpreted as a resuIt of prolonged shock. CASE II. A. W., a fifty-two year oId pipefitter, was first seen December 8, 1949, with the chief compIaint of sudden onset of upper abdominal pain, nausea and vomiting of two days’ duration. He gave a history of four simiIar attacks during the past year, each Iasting from two to three days. There was no history of jaundice or hematemesis, but he had noted frequent tarry stools both during and between these attacks. On physica examination bIood pressure was 150/64, temperature IOO.~‘F., puIse 104, respiration 20. The patient was a we11 deveIoped, we11 nourished white man who was extremeIy pale and appeared chronicaIIy i11. The abdomen was sIightIy distended, and there was tenderness to pressure in the epigastrium and in the left Iower quadrant. Tarry stool was present in the rectum. Laboratory reports reveaIed that on admission the red bIood count was 1,850,000, with a hemogIobin of 5.6 gm. The white blood count was I 1,650 with 65 per cent neutrophils and 35 per cent Iymphocytes. The tota proteins were 4.4 gm. with 2.9 gm. of aIbumin and 1.5 gm. of gIobuIin. Non-protein nitrogen was 39 mg. Liver function tests were a11 within 621
Leiomyoma
of Duodenum The majority of these tumors do not project into the Iumen of the bowe1 but extend into the subserosa1 region. The submucosa1 tumors are much smaIIer than the subserosa1 ones. Necrosis and hemorrhage in the center of the neopIasm gives an increase in size, and the gastrointestina1 hemorrhage foIIows when the sinus connects with the bowe1 Iumen. The treatment of choice is surgica1 excision of the tumor. When the tumor is present in the first portion of the duodenum, simpIe excision is desirabte, as in our Case II. If this is not possibIe, a duodena1 resection must be performed. When the tumor invoIves the second portion of the duodenum and the common duct, pancreaticoduodena1 resection is necessary, as in our Case I. Of the other eleven in which operative procedures are specified, fIve6~8~grequired simpIe excision, five4~6~8duodena resection and one gastroenterostomy.*
anterior duodena1 waII incIuding the tumor was excised IongitudinaIIy. The duodenum was then cIosed transverseIy. The postoperative course was uncomphcated. Postoperative x-rays reveaIed an irreguIar contraction in the first portion of the duodenum approximateIy 2.5 cm. dista1 to the buIb. The progress of the barium was unimpeded. The patient was discharged with no compIaints on January 16, 1950. On June 16, 1953, the patient was re-examined and found to be compIeteIy asymptomatic. His red bIood count was 5,130,000 and hemogIobin 15 gm. The specimen consisted of an ovoid mass of firm tissue partiaIIy covered by mucosa, measuring 1.5 cm. in diameter and 2 cm. in Iength. On section it was a homogeneous white mass of coarse IobuIated tissue. MicroscopicaIIy, the tumor was composed of bundIes of interIacing fibers of smooth muscIe. The nucIei were spindIe-shaped and of uniform size. One surface was covered by duodena1 mucosa with an intact muscularis. No maIignant changes were present.
SUMMARY
Two cases of Ieiomyoma of the duodenum are reported. These benign tumors usuaIIy occur in the first portion, give deformity or fiIIing defect on roentgenographic study and produce a variety of symptoms, the most consistent being meIena, hematemesis and massive gastrointestina1 hemorrhage. Most of the tumors project from the serosa1 surface and not into the Iumen of the bowe1. The treatment of choice is surgica1 excision.
COMMENTS
Leiomyoma of the duodenum usuaIIy arises in the first portion. OnIy three cases have been reported with origin given in the second portion (our Case 1).4*~ Some of these tumors are asymptomatic and are incidenta findings at autopsy. The nineteen symptomatic proven cases had symptoms varying from vague abdomina1 distress to massive gastrointestina1 hemorrhage. In one case6 the symptomatoIogy Ied to a preoperative diagnosis of choIecystitis, but jaundice has not been reported except in our Case I. These tumors may occur at any age. The ages of those with symptoms ranged from fourteen to sixty-three years with the symptomatoIogy covering periods of severa months to fifteen years. Roentgenographic study of the upper gastrointestina1 tract usuaIIy reveaIs significant changes, akhough they are seIdom diagnostic. In eight of the cases barium study discIosed deformity of the bulb and/or a fiIIing defect. DiIatation of the buIb with retardation of ffow, diffkulty in fiIling the duodena1 buIb, rapid transit of the barium through the duodenum, and other changes interpreted as mucosal foId defects and duodena1 uIcer have been reported.
REFERENCES I. RAIFORD, T. S. Tumors of the smaI1 intestine. Arch. SUTg., 25: 122, 1932. 2. ACKERMAN, L. V. and DEL REGATO, J. A. CancerDiagnosis, Treatment and Prognosis, p. 556. St. Louis. 1047. C. V. Mosbv Co. 3. HOFFMAN, B.-p. and GRAYZE-L, D. M. Benign tumors of duodenum. Am. J. Surg., 70: 394, 1945. 4. STRAUS. F. H. and O’KANE. C. R. Leiomvoma of ” duodenum. Surgery, 32: 86, 1952. 5. OLSEN, J. D., DOCKERTY, M. B. and GRAY, H. K. Benign tumors of smaII bowel. Ann. Surg., 134: 195. ‘951. 6. OBERHELMAN, H. A., CONDON,J. B. and GUZALJSKAS, A. C. Leiomyoma of gastrointestina1 tract. S. Clin. Nortb America, 32: I I I, 1952. 7. EBERT, R. E., PARK&J&T, G. F.:-MELENDY, 0. A. and OSBORNE. M. P. Primarv tumors of duodenum. Surg.,.Gynec. @ Obst., 97: 135, 1953. 8. BRECHOT, A., UHRY, P. and WIMPHEN. Une observation de Ieiomyome du duodenum. Medicine, 20: 529, 1939. o. Case records from the Massachusetts General Hospital. New England J. Med., 243: 970, 195~.
622
of the Duodenum
R. E. CAMPBELL, M.D. AND J. M. YOUNG, M.D., Diplomate, Memphis,
From tbe Surgical and Laboratory Services, Veterans Administration Medical Mempbis, Tenn.
Teaching
Group
ENIGN
CASE REPORTS CASE I. L. N., a twenty-five year oId white maIe construction worker, was admitted for the first time January 8, 1953, with the compIaints of tarry stooIs, weakness and dizziness. He had been in exceIIent heaIth unti1 one week before admission when a coId with non-productive cough deveIoped, foIIowed three days Iater by tarry stooIs. These continued at the rate of four a day for four days. Two days before admission he vomited undigested food containing cIots of dark bIood. No history of abdominal pain, discomfort or jaundice was obtained. He was a moderate drinker of aIcoholic beverages, consuming one or two pints of whiskey each week. On physical examination bIood pressure was 130/58, temperature IOI’F., puIse 130 and AmericanJournaloj Surgery,Volume
88, October,
rwo
Board of Patbology,
respiration 20. The patient was a we11 deveIoped, we11 nourished, paIe white maIe who appeared acuteIy iI1. Inspiratory wheezes and scattered rhonchi were present in the lungs biIateraIIy. The Iiver was paIpabIe one fingerbreadth below the right costal margin and was tender. The spleen was not paIpabIe. The laboratory reports can be noted in TabIe I. Bedside chest x-ray on admission was essentiaIIy normaI. On January 2rst x-ray study of the upper gastrointestinal tract showed an indentation of the Ieft IateraI margin of the stomach due to an enIarged spIeen. Esophagram and smaI1 intestina1 fiIms were within norma Iimits. On ffuoroscopy ten days Iater there was some diffIcuIty in fiIIing the duodenal buIb with rapid transit of the barium through this region. A smaI1 intestina1 series of fiIms was again normaI. The patient was immediateIy given whole bIood transfusions and received 9,500 cc. the first eight days, 4,000 cc. of which was given in the first three days. During this period his red bIood count rose to 3,800,000 and his hemogIobin to 10.4 gm. per IOO cc. The hematocrit reached 45 per cent. He was pIaced on a bIeeding ulcer diet, banthine@, ascorbic acid, calcium carbonate and procaine peniciIIin. After tweIve days his stooIs became brown in coIor and guaiac-negative. Esophagoscopy was negative. Caffeine gastric anaIysis was reported 18 mEq. free HcI in the haIf-hour specimen and 20 mEq. in the two-hour specimens. AI1 liver function tests were normaI. He was discharged on an ulcer regimen to return in two months for further observation and study. The patient remained we11 after discharge, but Iost 20 pounds in weight. One week before his second admission, April 28, 1953, sharp mid-epigastric cramping and a desire to defecate deveIoped after eating chiIi. He became nauseated and vomited, but the vomitus contained no bIood. His stooIs for the
Hospital,
and maIignant tumors of the small intestine are uncommon Iesions. In 1932 Raifordl reported a Iarge series of tumors of the gastrointestina1 tract in which there were fifty benign and thirty-eight maIignant Iesions arising in the smaI1 intestine. These fifty benign tumors constituted about 24 per cent of the entire benign group, whiIe the thirtyeight malignant tumors made up onIy 5 per cent of the maIignant group. Leiomyoma is the most common benign Iesion of the smaII intestine, and about 80 per cent of these occur in the jejunum and iIeum and the remaining 20 per cent in the duodenum.2 If one considers only the duodenum, adenomas are sIightIy more common than Ieiomyomas3 In 1952 Straus and 0’Kane4 coIIected fifteen cases of Ieiomyoma of the duodenum and added a case of their own. To this tota one can add nine cases reported by Olsen et aI., two by Oberhelman et aI., and two by Ebert et aI. We wish to report two other cases because of their rarity and some unusua1 features.
B
American
Tennessee
618
Leiomyoma
of Duodenum
next three days were hard and black. He began to have recurrent mid-epigastric pains on an empty stomach, relieved by milk. The pain frequently wakened him at night. Two days before admission the patient began to notice some change in skin color. TABLE SUMMARY
HEMATOLOGY
OF
perature came down to 99%. He then remained afebrile. He had received 3,500 cc. whole blood during this two-week period and showed no signs of bleeding after the seventh hospital day. The patient was definitely jaundiced at the I
LABORATORY
II
On physical examination blood pressure was 105/70, temperature IOO.~‘F., pulse 120 and respiration 18. The patient appeared acutely and chronically ill, and there was a sallow color to the skin. There were many “cafe au Iait” spots over the back and extremities. There were two small, freely movable, firm nodules, each 0.3 cm. in diameter, just under the skin, one in the left flank and the other over the right anterior chest. No definite jaundice was present. The liver was barely palpable and smooth. The spleen was enlarged three fingerbreadths below the left costal margin in the mid-clavicular line. There was tenderness to palpation in the right upper quadrant just to the right of the umbilicus. BIack stool was present in the rectum. The laboratory data are tabulated in Table I. During the first two weeks in the hospital the patient’s fever spiked to IOI’F. each day, and became progressiveIy more toxic. MultipIe blood cultures and enteric and heterophil agglutinations were negative. Penicillin and aureomycin were started after tweIve days, and graduaIIy over a six-day period the tem-
DATA,
CASE
BLOOD
I
CHEMISTRY
I
height of his fever on May 7, 1953, his tenth hospital day. Urine specimen was 4 plus for bile on the Harrison spot test. This cleared within two days. Following this his appetite increased, he became stronger, gained weight and became ambulatory. He no longer showed any signs of progressive disease, and his stools were negative for occult blood. Biopsy or one of the skin masses was reported as neurofibroma. Upper gastrointestinal roentgenograms and chest x-ray were normal, as were cholecystogram and intravenous pyeIogram films. On July 3, 1953, with the patient under endotracheal cyclopropane and curare anesthesia, an exploratory Iaparotomy was performed. A firm, rubbery tumor mass in the wall of the second part of the duodenum was found. This was encroaching upon the head of the pancreas and displaced the common duct. An attempt was made to remove the tumor by local excision, but this was not possibIe. A pancreatico-duodenectomy was required for removal of the tumor. Splenic enIargement was noted. During the operation there was generalized oozing of blood throughout the operative
Leiomyoma
of Duodenum
2
1
FIG. I. Case I. Anterior projecting
from
FIG. 2. Case tumor.
surface stomach, wall of duodenum.
I. Duodenum
opened,
duodenum
showing
sinus
and upper tract
leading
jejunum into
showing lumen
from
Iobulated central
tumor part
of
compIeteIy surrounded by the tumor. Most of the tumor Iay outside the duodena1 muscuIar waI1, but smaI1 Iobulations extended into the submucosa, especiaIIy near the entrance of the common duct and duct of Santorini. MicroscopicaIIy the tumor was composed of moderate-sized, uniform, spindIe-shaped ceIIs with abundant eosinophiIic cytopIasm and ovoid to round nucIei. No mitoses were seen. There was much necrosis and hemorrhage in the centra1 part. The tissue was very vascuIar with whorIing and interIacing of the fibers. (Fig. 3.) The tumor did not invade the pancreatic parenchyma. A Masson trichrome stain showed the tumor to be of muscIe origin. When the peritonea1 cavity was opened at autopsy, it contained 4,000 cc. of cIotted and liquid bIood. Dissection of the region of the duodenum discIosed no Iarge open vesseIs, but extravasation of bIood throughout the tissues was marked. The spIeen weighed 4go gm. and was very soft; the cut surface showed marked diflluence. The Iiver weighed 1600 gm. and was paIe brown in coIor. The choIedochojejuna1 anastomosis was patent and intact, as was the gastrojejunostomy. The remaining dista1 part of the pancreas weighed 6o gm. and measured 15 cm. in length. The anastomosis between the pancreatic duct and the bowel was intact.
field. The patient withstood the procedure well and received 1,500 cc. of whoIe bIood during the four and a half hour operation. Upon his return to the ward the bIood pressure was rzS/go and puIse 130. He remained thus stabiIized for three hours, when hisbIood pressure dropped to 1oz/80 and the puIse became 144. AIthough he was given 9,500 cc. of whoIe bIood and 9,000 cc. of intravenous fluids with neo-synephrine@ and norepinephrine added, he dropped into irreversibIe shock. In addition, he received adrenal cortex extract, and aqueous cortisone, desoxycorticosterone Iipo-adrenal extract without favorabIe response. He died twenty-seven hours after surgery. The gross specimen consisted of the distal portion of the stomach, the duodenum and the proximal portion of the jejunum. (Fig. I.) Attached to the second portion of the duodenum was a firm mass 4 by 6 by 5 cm. with the head of the pancreas Iymg directIy posterior. On section the tumor was a homogeneous mass of coarse IobuIated tissue having central hemorrhage and necrosis. There was a sinus tract leading into the duodenum. (Fig. 2.) The centra1 portion of the tumor lay 3.0 cm. above the ampuIIa of Vater and the inferior border was directIy adjacent to the common duct, displacing it and partly surrounding it near the ampuIIa of Vater. The duct of Santorini was
620
Leiomyoma
FIG. 3. Case muscle fibers.
I. Photomicrograph
of Duodenum
showing
interlacing
bundIes
of smooth
norma Iimits. StooI examination showed strongIy positive guaiac. On December 12th x-ray study of the upper gastrointestina1 tract revealed narrowing of the duodenum just beyond the buIb with a 2.5 cm. rounded defect at the beginning of the descending portion of the duodenum. BeIow this at the junction of the second and third portions of the duodenum, on the media1 contour, a 1.0 cm. projection with a narrow mouth was noted. RoentgenoIogic impression was that the fiIIing defect represented a tumor and the projection was either a diIated ampuIIa of Vater or a diverticuIum. The patient was pIaced on a bleeding MeuIengracht diet, bedrest, atropine and given whoIe blood transfusions. He vomited dark cIotted bIood and passed both Iiquid, dark, bIoody stooIs and formed, tarry stooIs. He received 6,500 cc. of whoIe bIood during the first three days, and during this time his red bIood count rose to 4, IOO,OOOand his hemogIobin to 12.2 gm. He showed no further signs of bIeeding after his third hospitaI day, and after six days his stooIs became brown in coIor and guaiac-negative. On December 27, 1949, with the patient under endotrachea1 cycIopropane and curare anesthesia, Iaparotomy was performed, revealing an ovoid mass about the size of a large marble just beneath the mucosa in the anterior waI1 of the duodenum. A smaI1 segment of the
Microscopic examination of the organs discIosed widespread infarction and necrosis throughout the Iiver, pancreas, spleen and kidneys, interpreted as a resuIt of prolonged shock. CASE II. A. W., a fifty-two year oId pipefitter, was first seen December 8, 1949, with the chief compIaint of sudden onset of upper abdominal pain, nausea and vomiting of two days’ duration. He gave a history of four simiIar attacks during the past year, each Iasting from two to three days. There was no history of jaundice or hematemesis, but he had noted frequent tarry stools both during and between these attacks. On physica examination bIood pressure was 150/64, temperature IOO.~‘F., puIse 104, respiration 20. The patient was a we11 deveIoped, we11 nourished white man who was extremeIy pale and appeared chronicaIIy i11. The abdomen was sIightIy distended, and there was tenderness to pressure in the epigastrium and in the left Iower quadrant. Tarry stool was present in the rectum. Laboratory reports reveaIed that on admission the red bIood count was 1,850,000, with a hemogIobin of 5.6 gm. The white blood count was I 1,650 with 65 per cent neutrophils and 35 per cent Iymphocytes. The tota proteins were 4.4 gm. with 2.9 gm. of aIbumin and 1.5 gm. of gIobuIin. Non-protein nitrogen was 39 mg. Liver function tests were a11 within 621
Leiomyoma
of Duodenum The majority of these tumors do not project into the Iumen of the bowe1 but extend into the subserosa1 region. The submucosa1 tumors are much smaIIer than the subserosa1 ones. Necrosis and hemorrhage in the center of the neopIasm gives an increase in size, and the gastrointestina1 hemorrhage foIIows when the sinus connects with the bowe1 Iumen. The treatment of choice is surgica1 excision of the tumor. When the tumor is present in the first portion of the duodenum, simpIe excision is desirabte, as in our Case II. If this is not possibIe, a duodena1 resection must be performed. When the tumor invoIves the second portion of the duodenum and the common duct, pancreaticoduodena1 resection is necessary, as in our Case I. Of the other eleven in which operative procedures are specified, fIve6~8~grequired simpIe excision, five4~6~8duodena resection and one gastroenterostomy.*
anterior duodena1 waII incIuding the tumor was excised IongitudinaIIy. The duodenum was then cIosed transverseIy. The postoperative course was uncomphcated. Postoperative x-rays reveaIed an irreguIar contraction in the first portion of the duodenum approximateIy 2.5 cm. dista1 to the buIb. The progress of the barium was unimpeded. The patient was discharged with no compIaints on January 16, 1950. On June 16, 1953, the patient was re-examined and found to be compIeteIy asymptomatic. His red bIood count was 5,130,000 and hemogIobin 15 gm. The specimen consisted of an ovoid mass of firm tissue partiaIIy covered by mucosa, measuring 1.5 cm. in diameter and 2 cm. in Iength. On section it was a homogeneous white mass of coarse IobuIated tissue. MicroscopicaIIy, the tumor was composed of bundIes of interIacing fibers of smooth muscIe. The nucIei were spindIe-shaped and of uniform size. One surface was covered by duodena1 mucosa with an intact muscularis. No maIignant changes were present.
SUMMARY
Two cases of Ieiomyoma of the duodenum are reported. These benign tumors usuaIIy occur in the first portion, give deformity or fiIIing defect on roentgenographic study and produce a variety of symptoms, the most consistent being meIena, hematemesis and massive gastrointestina1 hemorrhage. Most of the tumors project from the serosa1 surface and not into the Iumen of the bowe1. The treatment of choice is surgica1 excision.
COMMENTS
Leiomyoma of the duodenum usuaIIy arises in the first portion. OnIy three cases have been reported with origin given in the second portion (our Case 1).4*~ Some of these tumors are asymptomatic and are incidenta findings at autopsy. The nineteen symptomatic proven cases had symptoms varying from vague abdomina1 distress to massive gastrointestina1 hemorrhage. In one case6 the symptomatoIogy Ied to a preoperative diagnosis of choIecystitis, but jaundice has not been reported except in our Case I. These tumors may occur at any age. The ages of those with symptoms ranged from fourteen to sixty-three years with the symptomatoIogy covering periods of severa months to fifteen years. Roentgenographic study of the upper gastrointestina1 tract usuaIIy reveaIs significant changes, akhough they are seIdom diagnostic. In eight of the cases barium study discIosed deformity of the bulb and/or a fiIIing defect. DiIatation of the buIb with retardation of ffow, diffkulty in fiIling the duodena1 buIb, rapid transit of the barium through the duodenum, and other changes interpreted as mucosal foId defects and duodena1 uIcer have been reported.
REFERENCES I. RAIFORD, T. S. Tumors of the smaI1 intestine. Arch. SUTg., 25: 122, 1932. 2. ACKERMAN, L. V. and DEL REGATO, J. A. CancerDiagnosis, Treatment and Prognosis, p. 556. St. Louis. 1047. C. V. Mosbv Co. 3. HOFFMAN, B.-p. and GRAYZE-L, D. M. Benign tumors of duodenum. Am. J. Surg., 70: 394, 1945. 4. STRAUS. F. H. and O’KANE. C. R. Leiomvoma of ” duodenum. Surgery, 32: 86, 1952. 5. OLSEN, J. D., DOCKERTY, M. B. and GRAY, H. K. Benign tumors of smaII bowel. Ann. Surg., 134: 195. ‘951. 6. OBERHELMAN, H. A., CONDON,J. B. and GUZALJSKAS, A. C. Leiomyoma of gastrointestina1 tract. S. Clin. Nortb America, 32: I I I, 1952. 7. EBERT, R. E., PARK&J&T, G. F.:-MELENDY, 0. A. and OSBORNE. M. P. Primarv tumors of duodenum. Surg.,.Gynec. @ Obst., 97: 135, 1953. 8. BRECHOT, A., UHRY, P. and WIMPHEN. Une observation de Ieiomyome du duodenum. Medicine, 20: 529, 1939. o. Case records from the Massachusetts General Hospital. New England J. Med., 243: 970, 195~.
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