smooth muscle tumors of the alimentary canal *

Smooth Muscle Tumors of the Alimentary Canal *

JOHN H. MORTON, M.D., SAMUEL J. STABINS, M.D., JoHN J. MORTON, JR., M.D.Rochester, New York

From the Surgical Services, Strong Memorial, Rochester Municipal and Genesee Hospitals, Rochester,New York and the Department of Surgery, University of Rochester Medical Center,

Rochester, New York

THE NUMBER of leiomyomas and leiomyo-sarcomas of the alimentary canal whichproduce clinical symptoms is small enoughso that few surgeons gain a wide experi-ence in treating these interesting lesions.Since, however, they are readily confusedwith more common conditions, it is im-portant to recognize these tumors and tounderstand their clinical behavior. Thepresent report is based on a study ofpertinent literature and a review of casesseen at Strong Memorial, Rochester Mu-nicipal and Genesee Hospitals.

INCIDENCE

Small leiomyomas are found at post-mortem examination in a high percentageof cases when a careful search for them ismade. Meissner 70 found gastric leiomyomasin 46 per cent of 50 adult autopsies. Theselesions were found commonly in the fundus,and most were between four and six milli-meters in diameter. Rieniets,86 in 200 con-secutive autopsies, found 43 small leiomyo-mas in 16 stomachs. In two of these cases anassociated esophageal leiomyoma was pres-ent and similar tumors were found oncein the duodenum and once in the jejunum.Chi and Adams 18 considered leiomyomasthe most common benign tumors of theesophagus, but the majority of them wereasymptomatic autopsy findings. In 15,100autopsies at the Strong Memorial and Roch-ester Municipal Hospitals, asymptomaticleiomyomas in the gastro-intestinal tract

Presented before the American Surgical As-sociation, White Sulphur Springs, West Virginia,April 12, 1956.

487

were discovered in 70 cases. These weredistributed as follows: esophagus 10, stom-ach 42, duodenum 1, jejunum 9, ileum 5,colon 2, rectum 1.The frequent presence of these small

lesions at autopsy is of interest, but tumorsof this type have no clinical importance.The group of smooth muscle tumors inthe alimentary canal producing symptomsis a much smaller one, although many caseshave probably gone unreported or un-recognized. The stomach is the most com-mon site for symptomatic lesions, but theyhave been found all the way from thecervical esophagus 56 to the lower rectum.Meckel's diverticulum and the appendixare included among the sites where symp-tomatic tumors may occur.An approximation of the relative fre-

quency of these tumors can be obtainedfrom the review by Oberhelman and hiscolleagues.7 In the literature they found1,105 cases reported in the gastro-intestinaltract with 66 from the esophagus, 705 fromthe stomach, 225 from the small bowel and109 from the colon. They noted that ap-proximately 30 per cent of the lesions werefound incidentally at autopsy or at opera-tion. It is apparent that the stomach is themost frequent site of involvement.

PATHOLOGY

An excellent review of the pathologicalfindings in gastro-intestinal leiomyomas hasbeen written by Golden and Stout.36 Thetumors arise in the muscular layer of thegastro-intestinal wall, and they are usuallysingle and well circumscribed although a

488 MORTON, STAB

small number of multiple tumors are onrecord. The smaller tumors are rubbery,firm, round nodules which have a whorled,gray or pink appearance on cut section.The larger ones become lobulated. Sincethe tumors are relatively avascular, de-generative changes such as fibrosis, ne-crosis, cavitation, hemorrhage, abscess orcyst formation and calcification are com-mon. Because the secondary fibrosis is fre-quently such a prominent feature, it hasbeen proposed that these tumors be termed"fibromyomas.'91 Since the fibrosis is adegenerative phenomenon, this terminologyhas met with little encouragement frompathologists. The degenerative changesfrequently lead to mucosal ulceration withgastro-intestinal hemorrhage and rarely tofree perforation into the peritoneal cavity.

Proper fixation and the use of differentialstains are important in the diagnosis ofthese tumors. In most cases they are histo-logically similar to smooth muscle tumorselsewhere. They are composed of spindle-shaped cells arranged in interlacing bun-dles. The nuclei commonly palisade, andthis finding plus the presence of areas offocal necrosis has led to confusion of theselesions with neurilemmomas. Golden andStout pointed out, however, that gastro-in-testinal neurilemmomas are always en-capsulated and that a true microscopiccapsule is missing in the smooth muscletumors of the gastro-intestinal tract. Theyemphasized that this absence of a capsulemakes the leiomyomas appear to be in-filtrating growths and has led to their mis-classification by some authors as malignanttumors. They found that the small inci-dental leiomyomas discovered at autopsyshare this characteristic lack of capsule,and study of the microscopic material fromour cases confirms this opinion as far aslesions of the stomach and bowel are con-cerned. However, the leiomyomas of theesophagus are well enough encapsulatedso that most of them can be shelled outreadily,55 and Anderson and his associates 2

WINS AND MORTON Annals of SurgerySeptember 1956

reported that the benign lesions of thistype in the rectum are well encapsulated.

Differentiation of benign from malig-nant smooth muscle tumors on histologicalgrounds is not always easy. Golden andStout stated that the smooth muscle cellsin some malignant tumors may be quitenormal in appearance and that bizarre orgiant cells, although sometimes indicativeof atypical growth, may indicate merelydegenerative change. Mitoses when fre-quent are usually associated with malig-nancy, but the absence of mitoses providesno assurance that a tumor is benign.

Starr and Dockerty1( studied thosetumors arising in the small bowel. They feltthat generally the malignant leiomyomasare larger tumors and that they are moreprone to such features as ulceration, ne-crosis, hemorrhage, fistula or intussuscep-tion. Two large tumors in their series, whichmight have been considered benign his-tologically, recurred after therapy andwere, therefore, diagnosed malignant. Theyfelt that microscopically the tumors arecharacterized by spindle cells with ratherplump nuclei, and they listed certain his-tological criteria for malignant change. Ofthese they considered mitotic activity theone most important feature.Melnick 71 reported the interesting case

of a 50-year-old man who died after amassive gastro-intestinal hemorrhage. Atautopsy he was found to have an ulceratedlesion in the proximal end of the stomachand a solitary two centimeter nodule inthe right lobe of the liver. Both the gastriclesion and the liver nodule had the typicalmicroscopical appearance of leiomyomawithout invasion or anaplasia, and theauthor felt the tumor to be a benign onedespite the metastasis. Cowdell 23 recentlyreported a similar case in a 19-year-oldman with a primary tumor high in thegastric fundus and diffuse liver metastases.Histologically, the tumor appeared benignboth in the stomach and the liver. Massand Kirshbaum 65 described a case, his-

MUSCLE TUMORS OF THE ALIMENTARY CANAL

tologically benign, showing perforationfrom the stomach into the peritoneal cavityand direct extension to liver and perigastriclymph nodes. Most authorities agree atpresent that the histological diagnosis ofbenign or malignant smooth muscle tumormust occasionally be modified to fit thebiological behavior of the individual tumor.The malignant leiomyomas may metas-

tasize, but many of them kill by local in-vasive growth without distant spread. Theliver and the peritoneal surfaces are themost frequent metastatic sites althoughspreading to lung and bone has been re-

ported in a few cases.94 If metastasis toregional lymph nodes occurs at all, it isextremely rare. Several cases which are

mentioned in the literature as having lymphnode metastases actually had nodal in-volvement by direct extension from theprimary tumor. One case reported byMarvin and Walters 64 apparently had a

single regional node showing blood streaminvasion.

It is not easy to determine the per centof malignant leiomyomas which developmetastatic spread since there is consider-able variation among different authors indetermining the presence of malignancy.Lumb 58 felt that a better figure could beobtained by listing the per cent of allsmooth muscle tumors with metastases, andhe placed this figure at about six per centof gastro-intestinal leiomyomas.

Generally, leiomyosarcomas in the gas-tro-intestinal tract are slowly growing tu-mors which offer some hope for surgicalarrest. Occasionally, however, a case runs

a rapid and fulminating course with earlyspread or recurrence after operation.

CLINICAL TYPES

Even when dealing with a symptom pro-

ducing tumors, the physician is rarely ableto establish the correct diagnosis beforeoperation, and the operating surgeon may

share in this difficulty. In certain areas,

notably the esophagus, an accurate preop-

erative diagnosis may be highly importantin guiding intelligent therapy. In many

cases, careful roentgen examination of thegastro-intestinal tract should aid in ac-

curate preoperative diagnosis. Baker andGood4 pointed out that it should be pos-

sible by roentgen study to determine thata tumor is an intramural rather than a

mucosal one even though the exact typeof mural tumor could not be predicted, andthey gave criteria for making this distinc-tion.

According to their location in the ali-

mentary tract these tumors may be dividedinto four general types. (1) The intramuraltumors are the common, small, benign,asymptomatic lesions found at autopsy or

incidentally at surgical exploration. (2)The intraluminal tumors are the lesions,usually solid, which extend into the lumenwhere they may produce obstruction or

ulceration. (3) The extramural tumors are

those which grow outside the wall of thegastro-intestinal tract. They are commonlylarge and prone to cystic degeneration.(4) The hour-glass tumors combine thefeatures of the two preceding groups.

There are interesting differences in thesmooth muscle tumors at different levelsof the alimentary canal. These differenceswill be brought out by considering thevarious levels separately.

A. Esophagus. Figures from the MayoClinic 4 indicated that 85 per cent of theleiomyomas of the esophagus are benign.As compared with esophageal carcinomas,however, they are rare according to Piacen-tini 81 who stated that carcinomas are over

100 times more frequent. In a review ofthe literature through 1951, Lewis andMaxfield 55 found 47 surgical cases. Sincethat time scattered reports 13, 61, 81, 92 andthe collective review by Storey andAdams 101 have increased this number be-yond 120. Males were affected in approxi-mately two thirds of reported cases. Nocases below ten years of age have beenreported, but they have occurred at all ages

Volume 144Number 3 489

490 MORTON, STAB]

beyond the first decade. They are more

common in the middle and lower thirds ofthe esophagus, a fact which might beanticipated since part of the musculatureof the cervical and upper thoracic esopha-gus is striated.55 These tumors, which maybe multiple, usually lie in an intramuralpocket and are only rarely fixed to themucosa except in the cardioesophagealgroup which will be discussed separately.Symptoms are usually attributed to thegrowth impinging on the esophageal lumen.

Clinically, Sweet and his associates 103

found that most symptomatic tumors were

over five centimeters in diameter, and inthe esophagus size was not a good indica-tion of malignancy. Kenney45 reported a

19 by 15 centimeter tumor of the loweresophagus reaching to the right lateralchest wall. It weighed 1,420 grams andwas benign. A surgical resection was car-

ried out successfully. Symptoms attributedto these tumors by Sweet and his col-leagues were pain, dysphagia and variousdigestive disturbances occurring withroughly equal frequency. The dysphagiaoccasionally led to an erroneous diagnosisof cardiospasm. Symptoms were frequentlypresent for many years, but occasionallyasymptomatic tumors have been removedwhen they became large enough to bevisualized as a mediastinal mass on a

routine chest roentgenogram.Since the benign tumors are usually not

adherent to the mucous membrane, at-tempts at esophagoscopic biopsy are ill-advised.8 Taking advantage of this lackof fixation, surgeons have been able toenucleate many of these tumors successfullyfrom the esophageal wall without enteringthe lumen, and this is the method of choicewhen it is possible. Surgical complicationsmay arise when an unnecessarily exten-sive procedure is carried out because ofan incorrect diagnosis.10' It is, therefore,important to differentiate these lesionsfrom the carcinomas of the esophaguswhich demand a much more radical at-

INS ')'AND MORTON Ancals of SurgerySeptember 1956

tack. Results with enucleation or resec-tion in this group of tumors have beenvery good.Leiomyosarcomas are rare in the esopha-

gus. Harrington 38 performed the first re-ported successful resection of such a lesionin 1945. The tumor involved the entirecircumference of the lower esophagus in awoman of 60 years, and there was overlyingmucosal ulceration. The patient was livingand well three years later. Lyons and Gar-lock 59 reported an interesting case in awoman of 58 who had a pedunculated tenby four centimeter mass projecting into theesophageal lumen. The mass broke off atoperation and was retrieved from the stom-ach, but at latest report 33 the patient wasalive seven years after resection. Ovensand Russell 79 reported a woman of 56who underwent esophageal resection fordysphagia. The pathological specimenshowed a carcinoma in the middle thirdand a leiomyosarcoma just above thecardia. She was living and well two yearspostoperatively.

In reporting a fatal tumor of the cervicalesophagus eroding into the lung, Lip-schultz and Fisher 56 found only 11 re-ported cases, eight from autopsy materialand the three surgical cases mentionedabove. Seven of the 11 tumors were in thelower third of the esophagus. Their pa-tient, a man of 32, was the youngest onrecord and he had symptoms for only onemonth before death.

According to Sweet and his associates,pain was a much more characteristic find-ing in these patients than in those withesophageal carcinoma, and weight loss wasfrequently out of proportion to the degreeof obstruction. These authors cited a suc-cessful removal of a leiomyosarcoma fromthe cervical esophagus by Resano andHojman. Watson 105 reported four of thesetumors with one patient living and wellseven years after enucleation and anotherliving and well five years after biopsy,gastrostomy and roentgen therapy. Al-

v - - - - - v w . * ws w


though reported experience is too limitedto evaluate prognosis, these few reportsappear to justify vigorous surgical attemptsat extirpating these neoplasms.

In our clinical series only one patientwith two resected esophageal leiomyomaswas found. A 46-year-old man underwentresection of a symptomatic diverticulumof the lower esophagus. The diverticulumwas 2.5 centimeters in length and at itstip were two separate one centimeter firmyellow nodules which proved to be leio-myomas histologically. There have beenoccasional previous reports of leiomyomasin association with diverticula both in thecervical and the thoracic esophagus. In-deed, Sweet and his co-workers felt thatthe diverticula might occasionally be sec-

ondary mechanical phenomena producedby the tumors. The leiomyomas in our

case appeared to be purely incidentalfindings.

B. Cardioesophageal Junction. Tumorsin this area present enough differencesfrom leiomyomas elsewhere in the esopha-gus to warrant brief mention. Schnug o6was able to find reports of only seven suchcases. They usually present as large lobu-lated neoplasms completely encircling thelower end of the esophagus producingdysphagia. The tumors arise from theesophageal musculature and, like otheresophageal leiomyomas, do not involve themucosa of the esophagus. However, theyare characteristically fixed to the gastricwall and ulceration of the overlying gastricmucosa is common. Because they surroundthe esophageal lumen and are fixed to thestomach wall, surgical therapy usually in-volves partial esophagogastric resection.Brock9 and Kenworthy and Welch4" re-

ported cases of this nature. The case re-

ported by Harrington and Moersch39 dif-fered in that it was an extramural pedun-culated mass rather than a circumferentialtumor, but the mass produced dysphagiaby protruding up through the esophagealhiatus.

C. Stomach. Non-epithelial tumors makeup only a small per cent of gastric neo-plasms 108 but the leiomyomas are themost prevalent of these unusual lesions.As mentioned previously, the stomach isthe most common site in the gastrointestinaltract both for leiomyomas and for leiomyo-sarcomas. Considering a group of 931 be-nign tumors of the stomach, Minnes andGeschickter 72 found the leiomyomas to bethe most prevalent lesions. Of the totalgroup, 36.6 per cent were leiomyomas,19.5 per cent polyps and 10.9 per centneurofibromas. Everts and Kazal30 foundthat leiomyomas made up 40 per cent ofa group of benign gastric tumors mani-festing themselves clinically, and Elliottand Wilson 29 reported that leiomyomasrepresented 4.2 per cent of their seriesof clinically significant gastric neoplasms.Chaffin 15 reported that 77 per cent of hiscollected group of 363 smooth muscletumors of the stomach were benign and23 per cent malignant, but Golden andStout felt that statistics of this sort pre-sented a false picture. In considering onlythe tumors which produced clinical symp-

toms, they decided that the incidence ofmalignant leiomyomas was much greaterthan this figure. The rarity of these tumorswas emphasized by the report that at LaheyClinic the ratio of operated gastric car-cinomas to gastric leiomyomas was 62: 1.63Benign gastric leiomyomas are recorded

equally in both sexes with an age spreadof seven to 90 but with most of the cases

between ages 31 and 70. Palmer 80 reportedthat the tumors occurred anywhere in thestomach, being most common in the pars

media and antrum involving both curva-

tures and either gastric wall. In about tenper cent of cases, the tumors were mul-tiple. One of Appleby's patients 3 had 14separate gastric leiomyomas.The greater share of the tumors are sub-

mucous in position with considerable varia-tion in size. Most benign tumors are underten centimeters in diameter, but Palmer


492found that increase in the size or durationof the tumor did not correlate well withthe presence of malignancy. The tumorsare usually spherical or ovoid with pedun-culation and coarse lobulation common.They are usually sharply circumscribedbut without a well formed capsule.24The most common symptom found with

these tumors is gastro-intestinal bleeding,22'61,93 either hematemesis or melena. Morerarely, patients with slower bleeding pre-sent as cases of unexplained anemia. Theother symptom complex commonly re-ported is vague upper abdominal discom-fort which can be confused with the symp-toms of peptic ulceration. Kirshen, Wein-berg and Cohen 48 reported a case treatedas a duodenal ulcer for three years anddiagnosed roentgenologically as a papil-lary carcinoma which proved at operationto be a gastric leiomyoma.

Pyloric obstruction, the presence of an

asymptomatic abdominal mass, or symp-toms associated with an acute abdomendue to perforation of the tumor occur lessfrequently, but some unusual cases withthese symptoms have been reported. Sincelarger tumors frequently undergo centralnecrosis, perforation may be merely fromthe center of the tumor with intraperitonealhemorrhage rather than from the gastriclumen.19 Sherman, Long and Caylor 97 de-scribed an asymptomatic greater curvatureleiomyoma in a mobile stomach whichwas discovered only on pelvic examination.Pyloric obstruction may occur from thecontinued growth of a local tumor in thepyloric region, but a ball valve obstructionis perhaps more common. Muir 74 reportedsuch a case. Barnett 6 described an extremesituation of this kind with a pedunculatedleiomyoma of the gastric fundus leadingan intussusception of the entire gastric wallthrough an enlarged duodenum into theupper jejunum. The patient, a man of 54,had symptoms for two years before a suc-cessful operation was performed. Matas 66

reported a case, quite possibly a leiomyoma,

Annals of SurgerySeptember 1956

with symptoms of intense epigastric pain,shock, vomiting and melena occurringwhen the tumor prolapsed periodicallyinto the duodenum.

Obstruction from an extramural tumorwas reported by Ker.47 His 77-year-old manhad symptoms of dyspepsia on recliningover a period of eight years. When he wasforced to bed with a fractured hip, the dys-pepsia progressed to complete gastric ob-struction. At operation he was found tohave a large tumor arising on a pediclefrom the lesser curvature. With the patientsupine, the tumor slipped to the left of thespine and produced a volvulus of thestomach. Following removal of the tumor,his dyspepsia was relieved. Lorenz57 de-scribed a similar lesser curvature tumorwhich grew through the foramen of Wins-low and impinged on the common duct,the presenting symptoms being those ofacute chloecystitis without jaundice.

Preoperative diagnosis, when possible, isusually based on proper interpretation ofroentgen films. Abrams 1 wrote that thedemonstration of a smooth, round, sharplydemarcated, sessile gastric tumor with cen-tral ulceration, normal adjacent mucosaand normal peristalsis should suggest thepossibility of leiomyoma. Rigler and Erick-sen 87 and Elliott and Wilson also describedthe roentgen manifestations of these lesions.The former authors found that 25 per centof a large group of clinically significantgastroduodenal tumors were benign, butthey were unable to demonstrate many ofthe lesions roentgenographically. Many lei-omyomas show microscopical calcification,but gross calcification visible on ordinaryabdominal roentgenograms is unusual.Leigh53 described such a case, however,and others were reported by Garbarini andPrice 32 and by Koloski and his associates.49Cressman 24 included a case in which thetumor was first suspected because of a de-fect in the gastric gas bubble on a roent-genogram of the chest.

It is uncertain whether or not benign lei-

MORTON, STABINS AND MORTON

Volume 144 MUSCLE TUMORS OF INumber 3

omyomas ever undergo malignant change.Marshall and Cherry 61 felt that malignantchange, although not definitely proven, wasstrongly suggested by the facts. Dixon andKratzer 25 described a case in which a gas-tric resection for leiomyoma was followed23 years later by a secondary operationfor a leiomyosarcoma in the same location.In view of the difficulty with histologicaldiagnosis, it is remotely possible that thislesion was a malignant one at the time ofinitial operation.

Unless obvious metastases are present, itis extremely difficult to distinguish a lei-omyosarcoma of the stomach from a benignleiomyoma even at exploration. Among ma-lignant gastric lesions they are uncommon,making up only about eight per cent of thesarcomas reported 42 and occurring, accord-ing to Poskanzer and Schmidt,82 only oncefor every 1,000 gastric carcinomas. Becausethese tumors are so uncommon, no onesurgical center can offer a large seriessusceptible to statistical analysis. Most ofthe reported patients were middle aged,and males were somewhat more commonlyaffected. Lesions have been discovered inall parts of the stomach, the majority beingof the intraluminal type.62 A case with twoseparate gastric leiomyosarcomas was men-tioned by Marvin and Walters. The num-ber of these tumors which give evidenceof metastatic spread is open to dispute.Poskanzer and Schmidt concluded thatmetastases occurred eventually in about 50per cent of cases but most authors 61, 64, 94concur more closely with Horley's 41 figureof under 20 per cent metastatic spread.There is general agreement that metastasesare late and that these tumors infiltrate lessextensively than gastric carcinomas.

Everts and Kazal remarked that an er-roneous diagnosis of advanced gastric car-cinoma might deny a patient with leiomyo-sarcoma the benefit of surgery and that nosuch lesion should be considered inoper-able without an accurate histological diag-nosis. A case reported by Ripstein and

[TH E ALIMENTARY CANAL 493

Flint88 bore this out. Their patient, a manof 69, had gastro-intestinal bleeding nineyears before at which time roentgenogramsshowed a lesser curvature ulcer. Bleedingrecurred at the time of admission and atlaparotomy the ulcer was found in the cen-ter of a leiomyosarcoma. Even after nineyears no metastases were present. Theydescribed two other cases without metas-tases, 24 and 18 months respectively afterthe onset of symptoms. Hoerr and Crile 40reported a 41-year-old man who had re-quired many transfusions over an 18 yearperiod of repeated gastric hemorrhage.Fourteen years previously laparotomyshowed an "undifferentiated carcinoma"and a hopeless prognosis was given. Anabdominal mass was palpable five yearsbefore admission. At operation he was dis-covered to have an enormous tumor whichwas removed by wide dissection with cau-terization of a direct extension to the liver.No metastases were found and histolog-ically the tumor proved to be a low gradeleiomyosarcoma with ulceration. The pa-tient was working without further hemor-rhage over five year later. Coburn andBaker 20 described a similar case in a 61-year-old woman who refused operation forfour years after her tumor was visualizedroentgenographically yet had no sign ofmetastases when laparotomy was finallyundertaken.

Gastric leiomyosarcomas sometimes reachamazing proportions. The patient describedby Glenn and Douglas 35 had symptoms ofjaundice for only 48 hours before death,yet the tumor between the stomach andthe liver measured 30 by 17 by 10 centi-meters at postmortem. Although the pa-tient succumbed later to metastases, Ed-wards and Wright 27 reported the success-ful removal of a 37 by 23 by 16 centimetertumor weighing 6,180 Gm. and arising bya pedicle from the greater curvature.The treatment of benign or malignant

gastric leiomyomas is surgical, roentgentherapy being of little value.82 Lahey and

494 MORTON, STABINS AND MORTON Annals of SurgerySeptember 1956

TABLE I. Symptomatic Benign Leiomyomas of the Stomach, Strong Memorial,Rochester Municipal and Genesee Hospitals

Major Symptoms Roentgenographic OperativePt. Age Sex and Signs Report Operation Diagnosis Pathology Result

JS 66 M 2 wks. of abd. pain and Neoplasm, mid Subtotal Carcinoma, Leiomyoma Uneventfulbelching. Tarry stools. portion of stomach gastrectomy lesser with recovery.Hgb. 7 Gm. curvature necrosis Lost to

follow-upPF 48 F 5-6 yrs. of epigastric Leiomyoma Wedge Leiomyoma, Leiomyoma No recurrence

gnawing. Hematemesis resection high lesser with 41 yrs.on day of adm. Hgb. curvature necrosis12.5 Gm.

OM 60 M 2 yrs. of epigastric pain Intraluminal Wide local Leiomyoma, Leiomyoma Carcinoma ofand abd. bloating tumor excision antrum stomach 5 yrs.later. No re-currence 3Jyrs. after 2ndoperation

FM* 54 M 4 mos. of vague gastro- No tumor seen Shelled out Benign Leiomyoma GI symptomsintestinal symptoms tumor, better.with unexplained anterior Anemiaanemia wall unchanged

Ss 85 F Repeated gastro-intes- Not done; Wide local Bleeding Leiomyoma No furthertinal hemorrhage, emergency excision tumor, with bleeding.massive. Shock. surgery high lesser ulceration Died 6 yrs.BP 70/40 curvature later of other

causesFS 56 M 4 wks. tarry stools. Malignant neo- Subtotal Carcinoma Leiomyoma No recurrence

Hgb. 7 Gm. plasm, mid- gastrectomy fundus with 8 mos.portion of stomach ulceration

CH 59 M Weakness-tarry stools Not done None Clinical Leiomyoma Died1 day. Dyspneic, impression, withashen peptic ulcer ulceration

* Note Tumor only 0.4 cm. in diameter. Probably tumor incidental and symptomatic improvement unrelated to its removal.

Colcock 51 urged that high subtotal resec-tion be carried out in all cases, for theyfelt that any lesser procedure entailed toogreat a risk of recurrence. Most authorsfeel, however, that a more conservative re-moval with a generous margin of normalstomach is sufficient.The prognosis for the gastric leiomyo-

sarcomas has never been well established,for too few long term reports are available.It is generally agreed that the outlook isbetter than in the case of gastric carcinomaor even gastric lymphosarcoma.54 From theLahey Clinic 61 five year survival was re-ported in 12 of 16 cases treated, but mostsurvival figures are closer to those reportedby Jordan and his colleagues." In review-ing 103 cases from the literature theyreached a five year survival figure of 37per cent as compared to 27 per cent for281 lymphosarcomas.Our series includes ten symptomatic gas-

tric smooth muscle tumors, seven benign

and three malignant. In addition, four otherleiomyomas were incidental findings atoperation. The symptomatic benign tumorsare summarized in Table I, and two of themore interesting cases plus the three sar-comas are recorded in more detail.

Case 1. OM, a 60-year-old white man, wasadmitted to Strong Memorial Hospital February2, 1947 with a chief complaint of epigastric pain,upper abdominal bloating, heart burn and eructa-tion for 2 years with increasing symptoms in thepast few months. He was known to have a mildlabile hypertension. Physical examination showedonly prostatic hypertrophy and chronic bronchitiswith emphysema. Free acid was present in the gas-tric contents after histamine stimulation. During agastro-intestinal series he was found to have a4 cm., constant, intraluminal ifiling defect in theprepyloric area (Fig. 2A). At operation a noduleof stippled red appearance was found in the gas-tric wall nine centimeters from the pylorus project-ing into the lumen like a thumb. It was excisedwith a good margin, and a posterior gastroenteros-tomy done. This tumor had not ulcerated. No otherpathologic condition was present in the stomach.His symptoms regressed.

Volume 144Number 3

MUSCLE TUMORS OF THIK ;. : .......................~~~~~~~~~... ..........FIG. 1. Typical gastric filling defect produced by

leiomyoma in patient PF.

He was next admitted July 7, 1952 complain-ing of paraumbilical pain after eating, plus weightloss. Roentgenograms of the stomach at this timeshowed an annular carcinoma approximately atthe site of the previous leiomyoma (Fig. 2B).Laparotomy was performed and the pyloric end ofthe stomach removed. Microscopic sections re-

vealed an adenocarcinoma.At present, 9 years after the first and over 3½Y

years after the second operation, he is well with-out evidence of recurrence of either tumor.

Case 2. CH, a 59-year-old man, was admittedto Genesee Hospital July 3, 1941 complaining ofweakness, fainting and mild indigestion for 24hours before admission. He had passed severaltarry stools the previous evening. He was ashenand somewhat dyspneic, but his blood pressure

was 120/70 and his hemoglobin level 11 Gm. percent. Shortly after admission he vomited one

liter of dark fluid. It was concluded that he hada peptic ulcer, esophageal varices, or a gastricmalignancy, and it was decided to treat him med-ically. He refused feedings and by the followingday his hemoglobin level had dropped to 6 Gm.per cent. He lapsed into shock and, despite limitedtransfusions, died July 6, 1941.

Postmortem examination showed only a 4 cm.

tumor mass in the posterior gastric wall about 7

E ALIMENTARY CANAL 495

cm. from the pylorus. There was ulceration of themucosa over this tumor and the gastro-intestinaltract was filled with old blood. On microscopicalsection it was found to be a benign leiomyomawith ulceration.

Prompt surgical intervention in this case wouldprobably have been curative.

Case 3. LA, a 48-year-old white male, wasadmitted to Strong Memorial Hospital December31, 1953 with a chief complaint of dizziness of4 months' duration. The dizziness had been pre-ceded by 2 weeks of tarry stools. His physicianstarted an ulcer diet after obtaining a roentgeno-gram showing a lesion at the cardia. Subsequentgastro-intestinal series (Fig. 3) showed progressionof the lesion to involve the proximal third of thestomach, and surgical treatment was advised. Ex-amination showed him to be an obese pale manwith guaiac positive stools and a hemoglobin levelof seven grams per cent. A thoracoabdominal totalgastrectomy with esophagojejunostomy was carriedout uneventfully. The specimen showed an 11cm., hard, lobulated and ulcerated lesion arisingfrom the posterior wall of the stomach. The opera-tive impression was carcinoma but the microscopicsections showed a leiomyosarcoma without nodemetastases. Since operation he has done well andnow, over two years postoperative, he is withoutevidence of disease.

Case 4. WS, a 57-year-old white man, wasadmitted to Strong Memorial Hospital in July 1952with a chief complaint of weakness for 8 months.During this time he had intermittent episodes ofdizziness with one period of syncope which led tohospitalization elsewhere. He was found to havetarry stools and the diagnosis of duodenal ulcerwas made. He was hospitalized for one month butwas referred here because of continued symptoms.Physical examination was not remarkable. Gastro-intestinal series (Fig. 4) showed a large centralfilling defect in the stomach with sharp cut-off ofthe marginal rugae. It was felt to be a benign tu-mor and the surgeon at operation concurred. Atlaparotomy a purplish red nodule was found highon the lesser curvature and a 5 cm. globular massextended from this area down into the gastriclumen. A subtotal gastric resection was done, en-compassing the lesion with a wide margin. Micro-scopically the lesion was found to be a low gradeleiomyosarcoma. His postoperative course was un-eventful. He is living and well over 3½h years afteroperation.

Case 5. AF, a 62-year-old white woman, wasadmitted to Rochester Municipal Hospital January20, 1943. For 2 years she had been suffering fromchronic fatigue, weight loss and progressive pallor.Past history revealed a possible carcinomj of the

MORTON, STABINS AND MORTON Annals of SurgerySeptember 1956

A B

FIG. 2. Patient OM with adenocarcinoma of stomach developing in area of resection of a benignleiomyoma-2A, the pedunculated leiomyoma; 2B, the subsequent carcinoma.

left ovary 3 years previously for which radiationtherapy had been given. Examination revealed achronically ill, very pale woman with a hemo-globin level of 5 Gm. per cent and a guaiac posi-tive stool. A gastro-intestinal series (Fig. 5)showed a filling defect on the greater curvatureof the antrum. This was interpreted as a car-cinoma. A laparotomy was done February 15,1943. At this time no disease was evident in thepelvis. There was a 6 cm. mass in the gastric wallfor which a subtotal gastrectomy was done. Fortechnical reasons the transverse colon was alsoresected. The leiomyosarcoma had a central ulcera-tion 2 cm. in diameter. No lymph nodes in thespecimen were involved. Her postoperative coursewas smooth. She has remained well and is with-out evidence of recurrence over 13 years since heroperation. It is of interest that the attending physi-cian on the medical service was violently opposedto this operation in 1943, stating that the case wasa hopeless one.

D. Duodenum. In our series symptomaticduodenal smooth muscle tumors are com-pletely absent. Campbell and Young 12

found that leiomyomas were the most com-mon benign tumors of the small bowel, butthat only 20 per cent of them occurred inthe duodenum where they involved pre-dominantly the first portion. River and hisassociates,89 in reviewing 1,399 benign smallbowel tumors, found adenomas and lipomasreported slightly more frequently, but manyof these lesions were asymptomatic. Theywere able to find 32 benign duodenal lei-omyomas in the literature. Patients of bothsexes were reported, largely in the 40 to60 age group. Cole 21 considered adenomasmuch more frequent in the duodenum. Thenumber of leiomyosarcomas here is rela-tively large, making up from 20 per cent2'to 60 per cent'100 of the smooth muscletumors. Weinstein and Roberts,10' in re-

viewing the malignant lesions, found 28on record, and approximately 15 more havebeen reported since their review.26 43, 100

496


FIG. 3. Leiomyosarcoma of gastric fundus inpatient LA, thought preoperatively and at op-eration to be a carcinoma.

The symptoms produced by the duodenallesions are largely due to ulceration or ob-struction. Gastro-intestinal bleeding, gnaw-ing epigastric distress and other symptomsof peptic ulceration are the most common

presenting complaints. Complete obstruc-tion is unusual although nausea and vomit-ing occur frequently. Golden and Stoutfound occasional partial occlusion of theinferior vena cava or superior mesentericartery by the tumor mass with a significantincidence of complications from mesentericthrombosis or pulmonary embolism. Theyfound a palpable tumor in 25 per cent ofcases with free perforation into the peri-toneal cavity occurring more frequentlythan with similar lesions in the stomach.Campbell and Young described jaundicein a patient with a benign leiomyoma inthe second portion of the duodenum sur-

rounding the common duct. Like the otherleiomyosarcomas, those in the duodenumgrow slowly with metastasis, usually to theliver, reported in only about 25 per centof cases.

The results of surgical treatment of theselesions, except for the incidental asympto-matic ones, have not been good. Some ofthe patients with benign lesions have hadfatal hemorrhage or perforation 107 withthe tumor being discovered only at autopsy.Scattered reports 14, 21, 24 of successful local

FIG. 4. Leiomyosarcoma of lesser curvature ofstomach in patient WS, thought roentgenographi-cally and at operation to be a benign intramuraltumor.

FIG. 5. Leiomyosarcoma of gastric antrum inpatient AF, now living and well over 13 yearsafter resection of the lesion which was consideredhopeless preoperatively by the attending physician.

resections have been recorded. In Cress-man's case the tumor was missed at thefirst operation and a subtotal gastrectomydone with the incorrect diagnosis of bleed-ing ulcer. The tumor was removed fromthe duodenal stump at a second procedure


MORTON, STABINS AND MORTON

after further hemorrhage. In the case re-ported by Straus and O'Kane 102 a proximalduodenal resection was successful for alesion in the medial wall proximal to theampulla of Vater. In reviewing 28 leiomyo-sarcomas of the duodenum Weinstein andRoberts found only seven alive at the timeof report, the longest postoperative follow-up being 36 months. McCullough 68 re-

corded the case of a 44-year-old woman

who died after a febrile illness. At autopsyshe was found to have an ulcerated lei-omyosarcoma of the third portion of theduodenum with infected sinus tracts run-

ning through the tumor. There was a singlesmall tumor nodule in the liver, but thecause of death was metastatic abscesseswithout tumor in the liver, lung and heartmuscle. The longest recorded postoperativesurvival was Zintel's patient'09 who lived44 months after resection of the fourth por-

tion of the duodenum with end-to-endanastomosis only to succumb to livermetastases.

In reviewing available pathological de-tails 26, 106 it is evident that distant spreadwas not the main cause for failure sincemetastatic growth was present in only 25per cent. The site of the tumors was suchthat inadequate surgery was carried outwith subsequent recurrence or that majorsurgical extirpation resulted in postopera-tive fatality. The pathological characteris-tics of these rare lesions are such that a

pancreatico-duodenectomy should offer cer-

tain of these patients a favorable outlook.The chance of permanent arrest of theselesions by radical surgery would certainlyseem better than the situation with car-

cinoma in the head of the pancreas wherespread beyond the limits of resection occurs

so early.E. Jejunum and Ileum. Olson and his

associates 78 found leiomyomas the largestsingle group in their study of symptomaticbenign small bowel tumors. Starr andD0ckerty placed leiomyomas among themost common neoplasms in the small bowel

and reported that over 50 per cent of the300 they reviewed were malignant. Cherryand Hill 17 found metastases in only 16 percent of the total group, and almost all ofthese cases were patients with the extra-mural type of tumor growth. In reviewing986 tumors of the gastro-intestinal tract atJohns Hopkins, Raiford 84 found only threeleiomyomas, all benign, in the small bowel.Rabinovitch and his associates 83 alsopointed out the relative rarity of the malig-nant leiomyomas by their review of cases

over a 25-year-period at the Jewish Hos-pital of Brooklyn. During that time theyfound 48 carcinomas of the small bowel,1954 carcinomas of the colon and rectumand only seven leiomyosarcomas, three inthe small bowel and four in the colon.

In the small bowel the ileum 89 was themost frequent site for benign and the jeju-num43 for malignant smooth muscle tu-mors. All ages were affected with the tu-mors somewhat more frequent in males.100Roth and Farinacci9' reported a malignantlesion in the jejunum of a newborn, andcases in later life, asymptomatic until sud-den major symptoms developed, have beenrecorded.31' 52 Bleeding, frequently severe,into the bowel lumen or from necrotic tu-mor into the peritoneum is the most com-

mon symptom. Intestinal obstruction due tointussusception or, less commonly, to vol-vulus is the other major presenting com-

plaint. A palpable mass was noted in 30per cent and rupture with generalizedperitonitis in five per cent.100 Barb5 re-

ported a patient with pain similar to pepticulceration in whom gastro-intestinal serieson several occasions showed deformity ofthe duodenal cap. At operation no signof ulcer was present, and a midjejunal lei-omyoma was found with relief of symptomsafter its removal. Barb felt that the duo-denal deformity was secondary to the path-ologic change elsewhere in the gastro-in-testinal tract and urged that this possibilitybe considered when no ulcer could be dem-onstrated at surgery. Cherry and Hill em-

498 An,nals of SurgerySeptember 1956


phasized the same point, and Rankin andNewell 85 also noted the presence of painof this type occasionally with a leiomyoma.

In Meckel's diverticulum smooth muscletumors, especially sarcomas, are among themore common neoplasms. For instance,Nygaard and Walters 76 found 20 malignanttumors reported in the literature of whichfive were leiomyosarcomas and five others,listed as spindle cell sarcomas, may havebeen of smooth muscle origin. Barrett,7Moses,73 and Mathews 67 reported other lei-omyosarcomas here. None of the lesionsshowed distant metastases although localrecurrence was noted five years after opera-tion in Mathews' case.

Starr and Dockerty noted that the sizeof the tumor was an important factor indetermining whether small bowel leiomyo-omas were benign or malignant, and theyconsidered lesions over five centimeters indiameter to be suspicious even when micro-scopically benign. Like most gastro-intes-tinal leiomyosarcomas, those in the smallbowel usually grow slowly. Horsley andMeans 43 found one case with intermittentgastro-intestinal bleeding for 17 years andanother with a palpable mass for 15 years.Starr and Dockerty reported resection ofa large tumor in the ileum with extensiveperitoneal and omental recurrence provedduring an incidental operation 13 yearslater. Six years after the second operationhe was still living and well.

Diagnosis preoperatively is seldom madebut might be suggested by the presence ofa mid-abdominal mass with small bowelroentgen study occasionally helpful in lo-calizing the tumor.37 The pathological char-acteristics of these tumors affect the typeof surgical therapy which should beadopted. A wide local resection shouldoffer a good chance of cure when no ob-vious metastatic spread is present. This isespecially true in the submucosal groupwhere metastases are rarely reported.'1798Since metastases to draining lymph nodesdo not occur, Starr and Dockerty concluded

that removal of a wide wedge of mesentery,possibly interfering with blood supply inthe remaining small bowel, was not neces-

sary. Among their 26 patients with malig-nant tumors available for long term studythey found that 13 had survived five years,but three of this group had evidence of re-current tumor. That these tumors were fre-quently overlooked was emphasized by thefact that eight of their 76 patients hadundergone previous unsuccessful surgicaltreatment because of melena and requireda second operation for resection of the neo-plasm. Kustrup and his colleagues 50 re-

cently reported a similar case in which ableeding malignant tumor of the jejunumwas overlooked at the first operation.

In our series there is a single patient withtwo incidental leiomyomas of the ileum atoperation for an unrelated small bowel ob-struction. In addition there are two symp-tomatic cases which are presented briefly.

Case 6. CA, a 57-year-old white woman, wasadmitted to Rochester Municipal Hospital June 14,1949 with a chief complaint of right lower quad-rant pain with nausea and vomiting for 24 hours.The pain was rhythmical and severe. Physical ex-amination revealed a thin white woman with atemperature of 38.30 C. Her abdomen was softand with active peristalsis, but right lower quad-rant tenderness was present. Her white blood countwas 9,200, hemoglobin level 11 Gm. per cent andstool guaiac negative. A roentgenogram of theabdomen showed mild paralytic ileus. Five hoursafter admission an exploratory laparotomy wasdone with the preoperative diagnosis of acute ap-pendicitis. The appendix was not remarkable buta 6 cm. cystic mass was found in the pelvis. Ondelivery this proved to be a gray, friable, hemor-rhagic tumor with central necrosis. It was locatedon the antimesenteric border of the upper ileum.Resection with end-to-end anastomosis was done,and her postoperative course was uneventful. Thespecimen proved to be a typical leiomyoma withmucosal ulceration and tumor necrosis. Two yearslater a right colectomy was carried out for a car-

cinoma of the cecum. The site of small bowelanastomosis was visualized and no recurrencefound. Four years after the second and 6 years

after the initial operation she is in good health andshows no signs of abdominal disease.


500 MORTON, STAB]

Case 7. SP, a 68-year-old white woman, wasfirst admitted to Genesee Hospital August 15, 1954because of progressive enlargement of the lowerabdomen for one month. There had been no othersymptoms. Her past history was significant in thatshe underwent a resection of a leiomyosarcoma ina Meckel's diverticulum at Rochester GeneralHospital 7 years previously. At that operation a

spongy extraluminal mass about the size of a base-ball was found attached to the ileum. It wasmobilized from the cul de sac with difficulty andwith some tearing of the capsule. On the GeneseeHospital admission she was found to have a cysticmass in the lower abdomen rising out of the pelvis.Intravenous pyelography and barium enema

showed evidence of extrinsic pressure on uretersand colon from the mass. An exploratory laparot-omy was done August 20, 1954 at which time a

cystic hemorrhagic mass the size of a volleyballwas encountered. It was attached to the ileum andits mesentery and involved the right ovary. Nu-merous nodules were studded through the mes-

entery and omentum. The major mass was removedby doing a total hysterectomy, bilateral salpingo-oophorectomy and resection of ileum. Microscopicsections showed recurrent leiomyosarcoma. Shedid well until January 1956 at which time a largepelvic mass was again noted. The patient statedthat she had some lower abdominal discomfortand some recent difficulty moving her bowels.She had not noticed bloody stools. On examinationit was evident that two masses were present, one

in the left lower quadrant and a second in thepelvis impinging on the anterior rectal wall. ByMarch 1, 1956 the symptoms of bowel obstructionhad become more pronounced and a third ab-dominal exploration was done. There were foundto be metastatic implants over the bowel, mesen-

tery, omentum and in the liver in addition to thelarge palpable masses. Since the disease was so

extensive, a transverse colostomy to prevent totalbowel obstruction was done. Her postoperativeconvalescence has been unremarkable. She is nowliving with the generalized abdominal involvement9 years after the first operative removal of theleiomyosarcoma of Meckel's diverticulum.

F. Colon. Smooth muscle tumors in thecolon above the rectum are pathologicalrarities. At the Mayo Clinic about three per

cent of the myomatous tumors of the gas-

tro-intestinal tract, excluding the esophagus,occurred in the colon.2 Broders, Phillips andStinson 10 analyzed 1,202 malignant neo-

plasms of the large bowel, finding onlythree leiomyosarcomas. Two were inoper-

IN AND MORTON Annals of SurgerySeptember 1956

able tumors in the rectal segment, but thethird, a six centimeter nodule in the ascend-ing colon, had been resected and the pa-tient was without recurrence five yearslater. Golden and Stout included two co-lonic lesions in their paper and reportedthat these tumors occurred more frequentlyin the right colon with ulceration and hem-orrhage. Obstructing lesions in the leftcolon were also noted. However, in a morerecent review MacKenzie and his col-leagues 60 found a greater number of tu-mors, especially the benign variety, in thesigmoid. They recorded from the MayoClinic and the literature 27 benign andten malignant leiomyomas in the colon ex-clusive of the rectal segment. They foundintraluminal growths in the majority ofcases. Lesions of this type are usually proneto product abdominal pain, probably byleading an intussusception. In contrast, theextraluminal tumors are usually large be-fore symptoms occur. These authors did notnot find that large size here necessarily in-dicated malignancy. They referred to a casereported by Heurtaux in which a peduncu-lated tumor sloughed from the hepatic flex-ure and impacted at the anal outlet withresultant bowel obstruction, and they men-tioned a patient of their own who passeda fleshy mass per rectum three years beforea colonic leiomyosarcoma was excised. Freeperforation and localized abscess formationalso occurred in their series.

In their study Rabinovitch and his col-leeagues found four leiomyosarcomas inthe colon, one each in the appendix, cecum,transverse colon and sigmoid. Ryan9' re-ported a benign leiomyoma in the trans-verse colon of a five year old child withsymptoms of abdominal pain and vomitingfor two years. Trygstad 104 reported re-section of a malignant tumor in the cecumleading an intussusception in a man of 19years. Successful resection was recordedbut no data concerning the patient's subse-quent course was given. Ripstein and Flintalso described a sarcomatous lesion lead-

- - -


ing an intussusception in the cecum of awoman of 56. Eighteen months after rightcolectomy she showed evidence of abdom-inal metastases and died 27 months afteroperation. Marshall and Cherry found onlythree lesions in this area in their study ofthe smooth muscle tumors treated at theLahey Clinic. A leiomyoma from the sig-moid and single leiomyosarcomas from thetransverse and sigmoid colons were re-

ported. They also recorded a sarcoma inthe sigmoid, treated elsewhere, which ledan intussusception with external prolapse.Our series includes no cases in the colonabove the rectum. It is generally agreedthat wide local resection is the treatmentof choice in these rare lesions. Becauselymph node metastases are unknown here 60the outlook for curative resection shouldbe fairly good, but there are too few re-

corded cases for accurate determinationof prognosis.

G. Rectum. Although the rectum is notcommonly itivolved by myomatous tumors,they are considerably more common herethan elsewhere in the colon. In a completereview Anderson, Dockerty and Buie were

able to find 20 cases at the Mayo Clinic,10 benign and 10 malignant. The majorityof the benign lesions were in women andof the malignant tumors in men. The be-nign lesions were all less than five centi-meters in diameter. They were sphericalencapsulated tumors which were asymp-

tomatic findings on routine rectal examina-tion. No mucosal ulceration was presentand mucosal attachment only once. Allwere removed by local excision without re-

currence. In contrast most of the malignantlesions were larger and mucosal ulcerationwas present in half the cases. All were

sessile rather than polypoid lesions withbleeding, change in bowel habit and rectalpain the most common symptoms. Althoughno case showed lymph node metastases, re-

sults of surgical therapy were poor. Of theten cases nine patients died and one was

lost to follow-up. Two of the cases were

treated by abdominoperineal resection andthe others by lesser procedures. They feltthat all submucosal nodules should be con-

sidered dangerous lesions with immediateexcision and that all sarcomas should haveradical operation. When the large malignanttumors do not produce mucosal ulceration,they may be mistaken for presacral der-moids." Isolated reports of benign lesionsare on record 61, 95 including the 3.5 centi-meter tumor reported by Charles and Mc-Carty 16 which was excised from the analsphincter. Ghabrial 34 reported a ten centi-meter benign leiomyoma with mucosalulceration and bleeding in the rectal am-

pulla. A local resection was done with goodimmediate result. Reports of this type mustbe read with suspicion unless longer fol-low-up information is given. For instance,Neuman 75 reported enucleation of an ap-parently benign leiomyoma followed by re-currence and partial removal four years

later. At the second operation the tumoragain appeared benign but a recurrence

three years later showed definite sarcoma-

tous change. An abdominoperineal resec-

tion was then done, and the patient was

well two years later. Similar recurrent cases

were recorded by Soler-Roig and his asso-

ciates 99 and by McEachern and Arata.69 Inboth cases abdominoperineal resection was

carried out in the treatment of a secondrecurrence. Golden and Stout concludedthat the myomatous tumors of the rectumwere dangerous lesions and that abdomino-perineal resection was the treatment ofchoice for all except the pedunculated tu-mors. In their review they found poor re-

sults with local management of even theapparently benign lesions and felt that themore radical procedure was justified in an

attempt to improve the prognosis.Our series includes two rectal tumors,

one benign and one malignant. The benigntumor was a two centimeter, round firmpolyp discovered on routine rectal examina-tion just inside the sphincter in a 68-year-old man. When proctoscopic examination


502 MORTON, STABINS AND MORTON Annals 1fSrg956showed no mucosal ulceration, the tumorwas excised through the anus along with amargin of normal tissue. The microscopicsections showed a leiomyoma. No recur-rence was noted on his last examinationover four years postoperatively.The other patient was a 62-year-old

woman admitted in January 1956 with thehistory of having noted for four or fivemonths the sensation of a mass in the rec-tum with associated rectal pain and thefeeling of incomplete defecation. Melenawas denied, and a stool was guaiac nega-tive. Examination revealed a firm but cys-tic tumor mass between the vagina andthe rectum encroaching on the anteriorrectal wall. Sigmoidoscopy and barium en-ema were negative. The mass was excisedthrough the floor of the vagina. It provedto be an encapsulated eight centimeter tu-more apparently arising from the outerrectal wall. The tumor was removed locallywithout entering the rectal lumen and, onhistological examination, proved to be aleiomyosarcoma. Her course since opera-tion has been uneventful, and her bowelsare moving normally.

REMARKS

In 1925, Eliason and Wright 28 concludedthat benign leiomyomas of the stomachwere far from being as benign in their endresults as their benign classification sug-gested. As Ripstein and Flint pointed out,whether the tumors are benign or malig-nant histologically, in most cases the great-est danger they represent is not their malig-nant potentialities but the gravity of theclinical syndromes which they produce. Anattempt has been made in this review toassess the prognosis of the frankly malig-nant lesions and to point out the symptomsand signs associated with this group of tu-mors throughout the gastro-intestinal tract.If the surgeon is familiar with these ratherunusual lesions, therapy can be better ap-plied. If careful follow-up observations aremade, it should be possible in the future

to assess the prognosis more accuratelythan at present.

CONCLUSIONS

A review of the smooth muscle tumorsof the alimentary canal, including illustra-tive cases from Strong Memorial, RochesterMunicipal and Genesee Hospitals, has beenpresented. The gross pathologic features,the type of metastatic spread and the slowevolution of the tumors have been pre-sented with particular reference to theireffects on proper surgical management.The importance of considering these lesionsin evaluating patients showing some of thecommon symptoms associated with diges-tive tract disease has been stressed.

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63. Marshall, S. F. and W. A. Meissner: Leio-myoma of the Stomach. S. Clin. NorthAmerica, 31: 735, 1951.

64. Marvin, C. P. and W. Walters: Leiomyo-sarcoma of the Stomach. Arch. Surg., 57:62, 1948.

65. Mass, M. and J. D. Kirshbaum: Leiomyo-sarcoma of the Fundus of the Stomach withPerforation. Am. J. Roentgenol., 44: 716,1940.

66. Matas, R.: Pediculated Polypoid Fibro-Adenoma of the Stomach of Ball-Valve Type,Causing an Unusual and Complex Syndrome.Surg., Gynec. & Obst., 37: 723, 1923.

67. Mathews, F. S.: Meckel's Diverticulum: AReport of Twelve Cases, Including OneTumor. Arch. Surg., 10: 720, 1925.

68. McCullough, K.: Leiomyosarcoma of theDuodenum. New York J. Med., 44: 1248,1944.

69. McEachem, C. G. and J. E. Arata: Leiomyo-sarcoma of the Rectum. Arch. Surg., 66:388, 1953.

70. Meissner, W. A.: Leiomyoma of the Stomach.Arch. Path., 38: 207, 1944.

71. Melnick, P. J.: Metastasizing Leiomyoma ofthe Stomach. Am. J. Cancer, 16: 890, 1932.

72. Minnes, J. F. and C. F. Geschickter: BenignTumors of the Stomach. Am. J. Cancer, 28:136, 1936.

73. Moses, W. R.: Meckel's Diverticulum. NewEngland J. Med., 237: 118, 1947.

74. Muir, J. B. G.: Notes on a Case of Leio-myoma of the Stomach. M. J. Australia, 2:87, 1943.

75. Neuman, Z.: Leiomyosarcoma of the Rectum,Developing from Benign Leiomyoma. Ann.Surg., 135: 426, 1952.

76. Nygaard, K. K. and W. Walters: MalignantTumors of Meckel's Diverticulum. Arch.Surg., 35: 1159, 1937.

77. Oberhelman, H. A., J. B. Condon and A. C.Guzauskas: Leiomyoma of the Gastrointes-tinal Tract. S. Clin. North America, 32: 111,1952.

78. Olson, J. D., MI. B. Dockerty and H. K.Gray: Benign Tumors of the Small Bowel.Ann. Surg., 134: 195, 1951.

79. Ovens, J. M. and W. 0. Russell: ConcurrentLeiomyosarcoma and Squamous Carcinomaof the Esophagus. Arch. Path., 51: 560, 1951.

80. Palmer, E. D.: Benign Intramural Tumors ofthe Stomach: A Review with Special Refer-ence to Gross Pathology. Medicine, 30: 81,1951.

81. Piacentini, L.: Leiomyoma of the Esophagus.J. Thoracic Surg., 29: 296, 1955.

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83. Rabinovitch, J., D. M. Grayzel, A. J. Swyerand B. Pines: Sarcomas of the Small andLarge Intestine. Surg., Gynec. & Obst., 85:333, 1947.

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86. Rieniets, J. H.: The Frequency and Patho-logic Aspects of Gastric Leiomyoma. Proc.Staff Meet. Mayo Clin., 5: 364, 1930.

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DISCUSSION.-DR. SAMUEL J. STABINS, Roch-ester, New York: I would like to comment on onephase of this subject, namely, the massive hema-temesis in the presence of gastric smooth muscletumors, and the urgency of immediate surgery insuch patients.

Unfortunately the diagnosis is usually made ofa peptic ulcer; and although there is a good dealof controversy as to how bleeding peptic ulcershould be treated, it is more or less agreed that itshould be given a conservative trial. Such trialin a patient with a gastric smooth muscle tumormight be disastrous.

In several cases I have seen, they were diag-nosed as peptic ulcer. In the first case the patientwas seen on the medical service for three days,then suddenly went into shock and died. Atautopsy a small 4 cm tumor was found in the

stomach. Unquestionably, if the patient had beenoperated on when it was found the patient wasnot stabilizing, he would be alive today.

The other instance I saw about 14 months ago.This patient had a similar massive hematemesis,and diagnosis clinically was made of a peptic ulcer.He reacted to transfusion and subsequently had agastro-intestinal series which showed a large lesionin the stomach that was thought to be an advancedcarcinoma. The patient was rather reluctant tohave surgery, and so was the physician, because itwas felt it was inoperable. However, the patienthad no choice, because within 24 hours after thex-ray pictures were taken he went into shock andwas operated on. He had a benign leiomyoma,with normal convalescence following the subtotalgastric resection. He is now perfectly well.

smooth muscle tumors of the alimentary canal *

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