Multiple adenomas of colon (polyposis)

MULTIPLE

ADENOMAS (PoLYPosIs)* HENRY F.

GRAHAM,

M.D.,

OF COLON

P.A.C.S.

BROOKLYN

T

The adolescent type usuaIIy commences in the first decade but frequentIy is not seen by the physician unti1 the second decade when a history of repeated hemorrhages for some years is obtained. It has frequentIy occurred in severa members of the same famiIy and the mucosa of the gut is thickIy and uniformIy studded with muItipIe adenomatous poIyps from the iIeoceca1 vaIve to the anus. Thediagnosis of this type may be definiteIy estabhshed or rejected by sigmoidoscopy. Of the second or adult type more than half occur in maIes from sixteen to thirty years oId and it is probabIy aIways the resuIt of infection in some form, aIthough Murphy in 1916 said: “The etioIogy of intestina1 poIypi, Iike that of the common wart, is shrouded in mystery: Whence they come and why they go is Iike the riddIe of the sphinx.” The poIypi are fewer in number and may be Iimited to certain regions of the coIon whiIe there may be none in the rectum, hence a negative sigmoidoscopy is of no diagnostic vaIue. UIcers may be present in this type.

HE determination of the cause of bIood in the stooIs is one of the most diffIcuIt probIems that ever confronts the physician. I shalI discuss only one phase of it namely, muItipIe adenomas, often incorrectIy caIIed poIyposis because many of the tumors are not peduncuIated. Adenomas constitute $3 per cent of the benign tumors and nearIy IOO per cent of the disseminated tumors of the gastrointestina1 tract. In 1721 MenzeI described a case in which there was a genera1 inflammation of the intestina1 tract and a number of wart-Iike excrescences in the coIon. Wagner in 18j2 and Rokitansky in 1839 described the method of formation of the poIypi from the mucosa surrounding the uIcers. In 1861 Luschka described a coIon containing thousands of poIyps from the iIeoceca1 vaIve to the anus and varying in size from a hempseed to a bean. They consisted of gIands resembIing those of Liberktihn. Virchow in 1863 described a case and caIIed it “CoIitis PoIyposa Cystica. ” Cripps in 1882 described three cases occurring in the same famiIy (brothers). He concIuded that the muItiple disseminated variety is extremely rare; that it seems most common in earIy youth; has a definite famiIia1 tendency and IS, probabIy, reIated to cancer. Niemack aIso reports the case of a girI aged tweIve and Pennant with familia1 tendency reported three cases in one family. The Iiterature of this subject bears such weIIknown names as: Murphy, LockhartMummery, Erdmann, Morris, Gant and Coffey. The simpIest classification is that of Erdmann and Morris,3 namely: I. AdoIescent (congenita1, disseminated) type. 2. AduIt

(acquired) *Read

PATHOLOGY

type.

As the result of irritation or infection in a susceptible individua1 there occurs a of the gIands and diffuse hyperpIasia stroma of the intestina1 mucosa with Iymphocytic infiItration and the formation of new bIood and lymph vesseIs. A IocaI thickening occurs which graduaIIy enIarges and in many places, as a resuIt of the mechanica traction and squeezing, first sessiIe and Iater peduncuIated tumors are formed. These are never confined to the smaI1 gut. They are usuaIIy limited to the coIon. They vary in size from a pin head to a pear and hundreds may be present. The we11 developed ones are true adenomas.

before Ex-Interne

Society,

Methodist-Episcopal

234

Hospital,

May

24 1928.

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The surface may be smooth, or rough and noduIar, like a cauhffower. In the acquired variety the intervening areas of mucosa may be ukerated. Within a period of from one to seven a majority of these adenomas years degenerate into adenocarcinomas. Coffey is probabIy no benign says : “There process in which there is a higher incidence of maIignancy than colonic poIyposis. ” Sopcr’s series shows an incidence of 43 per cent. ETIOLOGY

In the adoIescent type we must assume an unusuaIIy sensitive mucosa which is easiIy irritated and inffamed by even norma intestina1 contents. Tuberculosis, syphilis, amebic or baciIIary dysentery and other inflammations of the coIon, mechanica or medicina1 irritants and even parasites are recognized causes of poIyposis. SYMPTOMS

Hemorrhage into the Iumen of the bowe1 is the one symptom present in every case sooner or Iater. It may be microscopic in amount or profuse and dark or bright in color. “The adenomas secrete an abundthick, offensive, gIairy, irritating ant, discharge that incites tenesmus and frequent evacuations unreIieved by defecation. “4 Marked anemia and weakness eventuaIIy supervene. Pain due to enterospasm is present. Eberhard, before the Kings County MedicaI Society, reported the case of a man forty years of age who had sudden attacks of umbiIica1 pain. The diagnosis of muItipIe adenomas was finahy made by sigmoidoscope. Intussusception, procidentia recti or intestina1 obstruction may deveIop in the Iate cases, either previous to or associated with the adenocarcinoma that wiI1 IinaIIy appear in practically every case. TREATMENT

The treatment of muItipIe adenomas or poIyposis is coIectomy. Whether this

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shouId be done at one operation or in muItipIe stages must depend upon the condition of the patient and the ski11 of the operator. A two or three stage operation wouId seem advisabIe in most cases. This aIone can avert the onset of maIignant degeneration. How dangerous is a coIectomy? Here again the answer depends upon the condition of the patient and the ski11 of the operator. It is not an operation to be IightIy undertaken and wiI1 aIways have an appreciable mortaIity. CIark of PhiIadeIphia6 has reported 12 coIectomies for extreme constipation without any primary operative death. That there is danger of the formation of generaIized peritoneal adhesions as a seque1 of coIectomy must be admitted. In CIark’s 12 cases peritonea1 adhesions caused one death four months after operation and necessitated a second operation in two other cases. What are the end resuIts in poIypoid disease? LiIienthaIj presented a case to the New York SurgicaI Society tweIve years after a tota coIectomy for multiple papiIIomas of the coIon. His patient, a woman, was in exceIIent genera1 heaIth. In the case about to be reported many adhesions were found at the Iast operation. They may cause more troubIe Iater. In addition there is the danger of recurrence of the maIignancy in metastatic form. CASE

REPORT

The patient was a female, thirty-three years of age. Hospital number 122,423. FamiIy history, irreIevant. Past history: She had typhoid fever when she was a chiId. Patient took up nursing at the age of seventeen years. She was then in exceIIent heaIth and weighed about 125

pounds.

Present illness. In the spring of 1916 she weighed 125 pounds and was empIoyed as a graduate nurse at the Women’s HospitaI. The duty was arduous and she began to Iose weight, had gastrointestina1 upsets, fermentation of food and occasiona attacks of diarrhea. She was married October 3 I, I 9 I 8. Her weight at that time was 106 pounds. There were no

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other symptoms except a genera1 physica reduction and eructations. The Iiving condrtions were exceIIent but there was little or no gain in

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No nucIeated red ceIIs were found in the smear and the coIor index was not high. Injections of iron and arsenic were given. During the preg-

d FIG. I.

FIG. 2.

FIG

3

FIG. I. Condition before operation. FIG. z. Appendectomy;

first stage of Mikulicz

operation.

FIG. 3. IIeo proctostomy and iIeostomy; colon isolated.

weight. In August 1920, she suffered from an attack of dysentery with 6 or 7 bIoody stools a day. This iIIness Iasted six weeks. There was then a gradua1 improvement. During the earIy part of February 1922, she passed a IittIe bright bIood. A physician thought it was due to hemorrhoids or fissure. February 20, 1922, foIIowing a vagina1 examination, she compIained of severe headache, had a chiI1 and her temperature rose to 103~~. Two days later an abortion occurred at approximateIy one month. There was no infection. FolIowing this she again improved somewhat but her weight remained between 106 and I IO pounds. During the winter of 1923-I 924 she occasionaIIy passed a IittIe bright bIood with the stools. After an examination February I 2, 1924, by a surgeon, a tentative diagnosis of tuberculous coIitis was made and irrigations of siIver nitrate soIution were ordered. Later she was found to have a smaI1 interna hemorrhoid. During the summer of 1924 there was considerabIe improvement. She no Ionger passed any bIood. Her weight increased to I 12 pounds and she became pregnant. During the earIy months of her pregnancy she often compIained of intestina1 cramps and pain in the back. In December, 1924, a bIood count showed onIy 3,500,000 red ceIIs and 45 per cent hemogIobin. A diagnosis of secondary anemia was made.

nancy her weight increased from I 12 to I 24 pounds, but, on severa occasions, she suffered from severe chiIIs foIIowed by a temperature of 103~~. to 104’~. A smear made at this time was negative for maIaria. On May 22, 1924, she gave birth to a female child weighing six pounds and six ounces. This chiId has been we11 since birth. She was nursed for five months and then weaned because the mother was again becoming anemic. A specimen of stoo1 at this time showed B. coIi and streptococci at a pretty high IeveI and B. weIchii about 50 Iess than the average number. January 20, 1925, red ceIIs 3,300,000 and hemogIobrn 40 per cent. February 17, 1925, red ceIIs 3,300,000 and hemoglobin 40 per cent; Ieucocytes I 1,600 with 82 per cent poIynucIears. May 20, 1925, red cells_ 3,866,ooo; hemogIobin 62 per cent; Ieucocytes I 1,600; polynucIears 75 per cent. October 1925, again a diminution was present. Red ceIIs 3,000,000; hemoglobin 50 per cent; Ieucocytes 4,800 and 52 per cent polynucIears. These five counts were a11 made by Doctor Dexter. In some of the specimens poikiIocytosis was marked. No nucIeated ceIIs were found at any time, that is, no normobIasts. OccasionaIIy she wouId suffer from attacks of sore throat with various vague pains and aches.

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Such attacks Iasted onIy a day or two. The tons& were found to be diseased and during the Iatter part of June, 1926, were removed under a IocaI anesthetic. At infrequent intervaIs she wouId pass a IittIe bright red bIood from the rectum. The bIood count remained about the same; hemogIobin ~2 per cent and red ceIIs 3,500,000. In August she suffered from a chiI1 with a temperature of 103~~. and a pulse of 120. After a day or two she feIt quite we11 again. FoIIowing chiIdbirth her weight remained around I 12 pounds. During the winter of 1926-1927 she was fairIy weI1, but not particuIarIy strong. In the spring of 1927, foIIowing a period of worry over severe disappointment, she ate poorIy, became quite reduced and again suffered from a chiI1 and fever. Five weeks in the mountains produced considerabIe improvement, aIthough she had another chiI1 at the end of the first week. Two weeks after her return home she had another severe chiI1 with a temperature of I 02’~. and a puIse of 120. The foIIowing day she feIt quite we11 and was abIe to go out. Three weeks later there was a simiIar occurrence. 1927, the Ieucocytes were September I 7, 7,400; hemogIobin 48 per cent and the poIynucIears 66 per cent. She eats very we11 and is of an optimistic temperament, but is worrying a great deal about her condition, especiaIIy of Iate not having menstruated for two months. On October 3, she suffered from a sIight chiI1 foIIowed, as usual, by a temperature of 102”~. and a rapid puIse. FOJ severa years past she has, at intervaIs, compIained of intestina1 cramps, has passed a great dea1 of ffatus and has had eructations. For the past three or four years her stooIs have been more numerous than normai, usually 3 or 4 a day and, at times, even as many as 6 or 8 a day. The appearance was often Iike “sausage meat” and mucus and bIood were aImost invariabIy present. An examination of the feces on October IO, 1927, showed the folIowing: The appearance was brown and extremeIy gaseous. It was highIy acid to a11 indicators. Test for biIe was negative; hydrobiIirubin, positive; histamin, xxxx; bacteria producing suIphuretted hydrogen, oxaIic acid, acetone and formaIdehyde, Microscopic examination showed a negative. moderate amount of ceIIuIose; Iarge numbers of red ceIIs; no ova or parasites; bacteria, Gramnegative, 90 per cent. IndoI was present and a Iarge amount of mucus; bIood pigment was

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xxxx. Negative for pus. On the outside of the feca1 mass in the jar were many bIood ceIIs and mucus in Iarge amount.

FIG. 4. FIG. 4. Most of colon removed. FIG. 5. Colon entirely removed; rectum.

FIG. 5. ileum ending in the

On October 14, 1927 a roentgen-ray examination was made by Dr. Quimby at the N. Y. PoIycIinic HospitaI. It showed >io of the mea1 in the stomach at the end of six hours and the greater portion in the coiIs of jejunum, principaIIy in the right Iower quadrant. At fifty-six hours the barium was in the appendix and down to the rectum. The entire colon was moderateIy diIated. There was no evidence of a growth or of polypi. On November IO, 1927, Dr. Lynch removed a specimen through the sigmoidoscope and the report by Dr. Joseph FeIsen was as foIIows: “The specimen submitted for examination consists of a soft, soIid, pinkish, ova1 mass 1.8 cm. by I cm. in size, somewhat knobby but smooth on the surface. it appears to have been avulsed from it’s original attachment. Upon section the tissue is of a homogeneous, smooth, pinkish texture. Microscopic diagnosis: PapiIIary adenoma. The stroma is highIy vascuIarized with large and smaI1 thin-waIIed vesseIs and is infIItrated with ceIIs of the Iymphoid type. The genera1 microscopic appearance is very suggestive of a chronic irritative process.” Dr. Lynch at this time made a diagnosis of muItipIe poIyposis of the coIon. ShortIy after this the writer did a sigmoidoscopy which showed a few smaI1 interna hemorrhoids and a few areas of scar tissue in the rectum. No poIypi or uIcers were seen in the rectum or Iower sigmoid.

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November I I, 14, and 18 smaII injections of quinine and urea hydrochloride were given into the hemorrhoids but without cessation of the

FIG. 6. CoIon and sigmoid composite

photograph.

blood in the stooIs. On November 30, 1927, the patient was sent to the hospita1 for operation. Her red ceIIs, at this time, were 4, I 84,000, the white ceils were 6,800 and the hemogIobin was 30 per cent. During the course of her iIIness, up to this time, she had been in the care of 15 physicians, including the writer, and had received various diagnoses such as secondary anemia from an unknown foca1 infection,. hemorrhoids, fissure in ano, tubercuIous cohtrs, oId tuberculosis of the lungs, and poIyposis of the coIon. These facts are mentioned mereIy to show the diffIcuIty of diagnosis in these cases. PhysicaI examination at this time showed a we11 deveIoped and fairIy we11 nourished aduIt female. The skin was paIIid and the mucous membranes were bIanched. The heart and Iungs were normaI. The abdomen was soft and not distended and no masses were paIpabIe. For about two months prior to admission the patient had noticed a tendency to constipation instead of diarrhea. This assumed considerabIe importance later when considering the findings at the first operation. In view of the marked anemia and Iong standing disease a series of operations was found necessary. Operation No. I. December I, 1927. Appendectomy. First stage of MikuIicz operation on

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sigmoid. Through a McBurney incision on the right side with sufficient enIargement down the border of the rectus to admit a hand the appendix was removed and the abdomen expIored. A Iarge mass was found in the sigmoid. The origina incision was cIosed and a second simiIar muscIe spIitting incision made in the Ieft Iower quadrant near to the anterior superior spine. The sigmoid Ioop with the mass at its center was drawn out through the wound and the two Iimbs sutured together and the parieta1 peritoneum sutured to the base of the Ioop. Time of operation one hour and five minutes. The patient stood the operation weI1. Operation No. 2. December 2, 1927. Transfusion. BIood, 560 c.c., was given by the Unger method to hasten convalescence. The red ceIIs following this were 4,776,ooo and the hemogIobin 54 per cent. Operation No. 3. December I o, 1927. RemovaI of sigmoid. PathoIogicaI report: The specimen consists of a rough, irreguIar mass of tissue 14 X 13 X 8 cm. in size. It is IargeIy composed of firm, friabIe gray tissue. Here and there are poIypi adherent to the waI1 by a pedicIe. These vary from $5 to 2 inches in Iength. Microscopic examination by Dr. Smith: This is a coIitis poIyposa which has deveIoped into an adenocarcinoma. After the sigmoid Ioop had been removed severa polypi were seen dropping down into the upper opening of the coIostomy. One of these was tied off and removed. A microscopic examination of this showed a marked adenomatous hyperpIasia of the intestina1 glands. In a few pIaces there was a tendency toward carcinomatous growth. By December 23, 1927, the red ceIIs had risen to 4,496,ooo; the hemogIobin was 59 per cent; white ceIIs 7,050 and the poIynucIears 72 per cent. The urine, on a few ocasions whiIe in the hospital, showed a faint trace of aIbumen and several times showed pus ceIIs but neither of these was constant. We feIt that the improvement couId onI? be temporary as Iong as there were more po1yp1 present in the coIon so a coIectomy seemed imperative. The patient’s genera1 condition and moraIe were exceIIent throughout. AI1 her wounds heaIed niceIy without infection up to this time. Operation No. 4. December 31, 1927. Transfusion. IIeoproctostomy. BIood 160 c.c., was given by the Unger method. The termina1

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iIeum was then divided about 6 inches from the iIeoceca1 vaIve and the proxima1 end implanted into the side of the rectum about 6 inches beIow the abdomina1 wall by a Murphy button anastomosis because of the tension and fixation of the rectum. The dista1 end of the ileum was then fastened into the upper end of, the right rectus incision which had been used for this operation. The skin was IightIy attached to the peritoneum of the iIeum. Time of operation one hour and ten minutes. TechnicaIIy this was a diffIcuIt operation. Again the patient responded weI1. There was no shock and primary union resulted. On January z I, 1928, the patient had a norma red ceI1 count and hemogIobin of 77 per cent. She had an isolated coIon free from feca1 irritation with an iIeostomy on one end and a coIostomy on the other. Her feces were somewhat fluid and at night, especiaIIy, ran back and out through the coIostomy wound. She was permitted to Ieave the hospita1 and return home to recuperate before the coIectomy. She gained 15 pounds in weight and returned to the hospita1 in perfect heaIth. WhiIe at home she had severa hemorrhages from the coIon. One was quite profuse. Operation No. 3. March 3, 1928. CIosure of coIostomy and coIectomy. A circuIar incision was made around the coIostomy and deepened unti1 both Iimbs were thoroughIy Ioosened from the surrounding structures. Heavy siIk Iigatures were then tied around both ends and the mesenteric septum that united them was divided. Both ends were then steriIized by the actua1 cautery. The rectal end was then inverted by two purse string sutures of chromic gut and attached to the parieta1 peritoneum. The ora end was then pushed within the peritonea1 cavity and the intermuscuIar incision was cIosed in Iayers with a smaI1 tube to the cIosed end of the rectum. Next a 7 inch upper Ieft rectus incision was made. The outer Ieaf of the mesocoIon was spIit, the mesocoIon was cIamped, Iigated and cut and the entire descending coIon, transverse coIon, and nearly a11 of the ascending coIon removed to a point just above the cecum. The omentum was Ieft behind attached to the stomach. The wound was sutured in Iayers with chromic gut. Time of operation two hours. As usua1 the patient responded we11 and had no postoperative shock aIthough her puIse went up to 120 and took severa days to return

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again to norma and she appeared more tired out than after the previous operations. Primary union occurred in both wounds. PathoIogicaI report: The specimen consists of most of the colon commencing above the ceca1 pouch and ending at the sigmoid. When spIit IongitudinaIIy it shows scattered throughout it’s entire Iength numerous smaII projections. Some are minute and conica in shape, others are 3 cm. or more in Iength, are peduncuIated and have either a hard irreguIar adenomatous appearance and feeling or are smooth soft poIypi. At the ceca1 end of the specimen is a Iarge hard irreguIar mass about 4 cm. in diameter, raised up with a cauIiffower appearance and feeling, very evidentIy an adenocarcinoma. Microscopic examination: Adenocarcinoma. Operation No. 6. March 19, 1928. Removal of termina1 iIeum and cecum. An incision was made in the scar of the previous right rectus operation encircIing the ileum. This was deepened unti1 the peritoneum was opened and the entire end of the iIeum freed from the surrounding tissues. The cecum was then mobiIized at it’s Iower end and graduaIIy Ioosened up toward the upper end. At the site of the previous division the adhesions were very dense and the Iines of cIeavage were obliterated. There were omenta1 adhesions and adhesions of the smaI1 intestine to the abdomina1 wall and to adjacent coiIs. Good exposure was diffIcuIt to obtain and the anesthetist feared to push the ether for muscuIar reIaxation because the pupiIs quickIy dilated. As the fina separation was accompIished the coIon opened at the point of Iigation and a few drops of intestina1 contents were spiIIed. This was quickly wiped up and a sponge saturated with an aIcohoIic soIution of mercurochrome wiped over the spot. The raw area behind the cecum was covered with peritoneum and a tube pIaced down to the area that had been soiled. The abdomina1 waI1 was cIosed in Iayers with chromic gut and siIkworm gut. Time of operation one hour. There was no shock and 0nIy moderate acceIeration of the puIse. PathoIogicaI report: The specimen was opened and found to he smooth. OnIy one smaI1 poIyp was found in the cecum and there were none in the iIeum. After a few days of discomfort the patient again reacted we11 and except for an infection aIong the drainage tube and in the fat Iayer of

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the wound her convaIescence was as rapid as after her previous operations. The infection rapidIy subsided and the patient left the hospita1 on ApriI 17, 1928. The tota number of hospita1 days was ninety-seven. Her condition on discharge was exceIIent. Her coIor was normaI, she was strong and heaIthy and was waIking around and her bowels were moving normaIIy each day without cathartics. There was no blood in her stooIs.

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in the years that infection. As a direct resuIt of deveIop in the coIon these degenerate into A tot;1 coIectomy brings reIief.

foIIow

the

1928

origina

this, muItipIe poIypi and, in two pIaces, adenocarcinomas. in muItipIe stages

BIBLIOGRAPHY I. LEVIN, A. L. Extensive colonic poIyposis; report of 3 cases. New Orleans Med. EY Surg. J., 80: I 12,

SUMMARY

FoIIowing overwork a young woman has an acute infection of the coIon with dysentery Iasting six weeks. This inflammation never compIeteIy subsides, as shown by many attacks of chiIIs, fever, rapid puIse, Ieucocytosis and high poIynucIear count,

1927. 2. KEMP. Diseases of the Stomach and Intestines. Pp. 571, 1911. 2. ERDMANN.J. F.. and MORRIS. J. H. PoIvDosis of the CoIon. &rg. 6ynt-z. Ed Obst:, 40: 46o,“igz5. 4. GANT, S. G. Diseases of the Rectum, Anus and CoIon, W. B. Saunders Co. VoI. 2, p. 249. 5. LILIENTHAL, H. Case Report. Ann. Surg., 55: 883, 1912.

6. CLARK, J. G. Surg. Gynec. @ Obst.,

22:

533, rgr6.

CONCLUSION AI1 in a11 I feeI, after treating some thousands of patients with inoperable carcinoma, chiefly with roentgen rays, that the resuIts, even temporary and inconstant as they may be, are we11 worth the time and Iabor, and I Iook upon the method as one of the most usefu1 adjuvants to surgery which we have. Whether radiation wiI1 ever repIace surgery in the treatment of cancer

seems from our present knowIedge extremeIy doubtful, but the progress of the Iast ten vears warrants the hope that with the improvement in technique and better classification of patients into suitable and unsuitabIe groups, more and more wiI1 be gained in a palliative sense and possibIy a moderate number of cures may ultimately be obtained. No surgeon can afford to negIect the benefit which may accrue to his patients from postoperative radiation, whether he believes that his operation has been successfu1 or not. There are many operators who do not wish to confess that their surgerv cannot do everything, but the number is rapidly growing smaIIer and pubIic opinion is bringing resistant members of the surgical profession into Iine. I look forward to the day when every case of a maIignant growth wiI1 be studied by the surgeon and the radioIogist together and the advantages of both methods be simuItaneousIy offered to the patient. (From Radiation in Malignancy” Francis C. Wood, Practical Lectures, 1921-26. N. I'.,P. B. Hoeber, Inc., 1927.)