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Intestinal polyps and associated carcinoma in childhood
Intestinal
Polyps and Associated
Carcinoma
in Childhood* PETER K. KOTTMEIER,
M.D., Brooklyn, New York, AND II. WILLIAM CLATWORTHY, JR., ~I.D.,
Columbus, Ohio
From the Department of Surgery, The Ohio State University College of Medicine and The Children’s Hospital, Columbus, Ohio.
intestinal polyps. .4t sigmoidoscopy three polyps were seen in the sigmoid colon between 15 and 20 cm. above the dentate line. On October 9, 1959 these polyps were removed and their bases fulgurated. The patient was readmitted March 3, 1960 because one month prior to admission, she again noted bloody streaks in her stools. There were periods of constipation but no pain or weight loss. At sigmoidoscopy five polyps measuring up to 8 mm. in diameter were found between 4 to 8 cm. above the dentate line. (Fig. 1.) These polyps were removed and the base fulgurated. Microscopic examination showed focal ulceration with acute and chronic inflammation and mild epithelial atypia. The atypia noted was interpreted as the result of chronic inflammation. On June 20, 1960, two months after the second removal of polyps, she returned for the fourth time. She had been asymptomatic until three weeks before when blood streaked stools again occurred. So other symptoms were present. Physical examination again was within normal limits, including rectal examination. The barium enema showed a filling defect in the lower descending colon, opposite the left sacroiliac joint, with a second small defect adjacent to it. Sigmoidoscopy demonstrated two polyps 7 and 10 cm. above the dentate line with broad bases. These polyps were removed by snare and the bases fulgurated. The areas where previous polyps had been removed failed to show any recur-rence. Microscopic examination showed polyps with ulceration and granulation tissue. On October 19, 1960, three months after the third removal of intestinal polyps, the patient was readmitted for the fifth time because of continued bright bloody streaked stools. At sigmoidoscopy, one polyp 7 cm. above the dentate line on the posterior wall of the rectum was found. This polyp had a broad base. It was removed by snare and the base was cauterized. Microscopic examination showed superficial ulceration and chronic inflammatory
carcinoma of the colon has been in children [l-5], but the association of intestinal carcinoma with intestinal polyps is rare in childhood [6,7]. Attention has been called to the fact that there is a significant difference in the pathologic and clinical behavior of polyps of rectum and colon in children as compared with those in adults [a]. It is generally assumed that intestinal polyps in children almost never undergo malignant transformation, or that carcinoma of the colon rarely exists simultaneously with intestinal polyps in young people. This has also been our experience, until recently carcinoma of the descending colon developed in one of our patients while under observation and treatment for multiple intestinal polyps.
I SOLATED reported
CASE REPORT K. L. (CH No. 94822), a twelve year old white girl, was first admitted to Children’s Hospital at the age of three years. The child was reported to have been anemic since birth, in spite of continuous iron therapy for two years. Congenital hemolytic anemia was diagnosed. Splenectomy was performed with complete recovery. The child was readmitted at the age of ten years. At this time the patient entered with the chief complaint of bright red blood in her stools for forty days prior to admission. There was no history of familial polyposis, and no known relatives had died of colonic carcinoma. Physical examination was within normal limits. Barium enema failed to show any evidence of * Presented
at the Seventeenth
Vol. 110. .‘Vovembev 1065
Annual Meeting
of the Southwestern May 10-13, 1965. 709
Surgical Congress, Hot Springs, i\rkansas,
Kottmeier and Clatworthy
FIG. 1. Summary of distribution and therapy of multiple adenomatous polyps and coexistent carcinoma of the colon in a twelve year old girl. infiltration. The glandular structures showed pseudostratification, and moderate pleomorphism with hyperchromatism. In most areas the basement membrane appeared to be intact; in a few areas, however, a breakthrough could not be ruled out. The polyp was considered to show atypical changes and a close follow-up of the patient was suggested. The patient was then readmitted, after four months, on February 1, 1961 with some diarrhea and rectal bleeding for three weeks. Barium enema showed a filling defect in the mid-transverse colon as well as sigmoid colon. At sigmoidoscopy there were no recurrent polyps up to 20 cm. above the dentate
A
line. .4n exploratory laparotomy was performed and the entire colon mobilized. Transillumination as well as palpation disclosed four polyps, the largest in the lower sigmoid colon. Proximal to this polyp, which measured approximately 4 cm. in diameter, was a smaller polyp approximately 2 cm. in diameter. A 2 to 3 cm. polyp was found in the transverse colon and another in the ascending colon. Examination of the remaining intestine up to the ligament of Treitz showed no other polyps. A colotomy was performed in the ascending colon, and a pedunculated polyp, which measured 2 by 3 cm., was removed with a base of normal-appearing mucosa. A second cototomy was performed in the transverse colon, where a larger polyp was excised in a similar fashion. A third colotomy was then performed in the sigmoid colon and an attempt was made to remove both polyps. The most distal polyp, which measured 4 cm. in diameter, showed what appeared to be atypical changes in the surrounding mucosa with slight puckering and adherence to the underlying muscularis. A segmental sleeve resection, which included both polyps, was therefore performed, leaving at least 4 cm. of normal bowel proximal and distal to both polyps. An incidental appendectomy was also performed. Microscopic section of the largest polyp, situated in the sigmoid colon, showed an adenocarcinoma with infiltration of the submucosa and lymphatic channels by glandular cells. (Fig. 2A and B.) These cells showed hyperchromic nuclei with loss of polarity. The underlying muscularis was not invaded. Resected lymph nodes showed no metastases. The patient had an uneventful postoperative recovery and was readmitted after four weeks on
B
FIG. 2. A and B, photomicrographs of adenocarcinoma of colon in patient with multiple adenomatous polyps; magnification X 200 and 400, respectively, before reduction. American
Journal of
Surgery
Intestinal
Polyps
March 3, 1’31iI l’or an elective subtotal colectomy and ileoljr:“tostc,nl~. 1+1xploration of the abdomen at this time again showed no evidence of metastases. A sul)tri;al colectomy, including M cm. of terminal ileum was pt!riormed. .\pproxintatcly I1 cm. of rectum remained Microscopic examination of tifty mesenteric Iyml)h nodes failed to show any signs of metastasis. Tlic resected colon showed four more polyps in the transverse colon and three polyps in the left descending colon, all benign on microscopic examination. ‘The patient had an uneventful postoperative reco\-cry and was discharged after six days. Fi\le months postcolectomy the patient h-as readmitted for routine sigmoidoscopy. The patient had been asymptomatic since the subtotal colectomy and had two to three stools daily. She had gained 13 pounds since the time of colectomy. Physical examination again was within normal limits. Sigmoidoscopy was performed which showed a normal terminal ileum with a patent anastomosis at the site of the ileoproctostomy. Just below the anastomosis at :! : 00, there was a single small polyp present which was removed and the base cauterized. Xl pol?;ps removed in this patient were of the adenomatous type except for a few which showed extensive inflammation without identifiable glandular elements. SUMMARY
OF CASE MATERIAL
Forty-nine additional infants and children with intestinal polyps, admitted between 1950 and 1960 to the Columbus Children’s Hospital were also studied. In these fifty patients a total of 144 polyps were found. Incidence ilccording to Age. The youngest patient in our group was four days of age, the oldest patient had his first admission for intestinal polyps at the age of thirteen years. The
age distribution (Fig. 3) shows that a considerable number of polyps were found between birth and the age of one year (eight polyps). However. the largest number is found in children between the age of three and six years (twenty-one polyps). Subsequently, there appears to be a steady decline in number during the prepuberal years. This decline suggests that there is a natural tendency for polyps in children to disappear during puberty. Comparing our findings with other reported cases, a similar age incidence is found, with the exception of the first year of life, wherein fewer patients are usually reported. Ravitch [9] reported polypoid adenomas of the entire gastrointestinal tract in an eighteen month old child. Cabrera [lo] found in autopsy specimens of Vol. 110. November 1965
and Carcinoma
;‘I1
thirty-two children, that one patient was in the one to two year group, nine in the three to four year group, and nine in the five to six year group with a decline in the older ages. Horrilleno [ll] gave 4.7 years as the average age for intestinal polyps in children. IMauro [12] reported twenty-five cases between two to eleven years, with the average age of 5.4 years. Helwig [13] found six polyps in 449 autopsies of children under twenty-one years of age; none, however, under the age of three years. Family History. It is often impossible to decide in the individual case, whether one is dealing with “multiple scattered polyps” or true “polyposis.” A positive family history of either “familial polyposis” or carcinoma of the lower intestine may, therefore, be of help in differentiating the two, probably unrelated, entities. Gordon [C;] used the term “diffuse polyposis” for patients with myriads of polyps, whereas patients with ten to one hundred polyps were included under the term “polyposis.” Gordon found a positive family history in children with polyposis in one of six patients with diffuse polyposis, three of eight patients with ten to one hundred polyps and three of ninety patients with one to nine polyps. Four of the latter had relatives with carcinoma of the colon. Flotte [14] reported sixty-three patients with intestinal polyposis, 34 per cent with a positive family history. In our group, a family history of either polyposis or intestinal carcinoma was not helpful. In patients with solitary polyps two had a positive family history; one patient’s
712
FIG. 4. Symptom tinal polyps.
Kottmeier
and Clatworthy
incidence in fifty children with intes-
grandfather was reported as having had a colonic carcinoma. The mother of a four months’ old patient with a solitary adenomatous polyp had a history of multiple intestinal polyps, In patients with two to ten polyps the family history was entirely negative. In patients with more than ten polyps the father of one patient had a single polyp removed as a child, followed by negative barium enemas. One other patient in the same group had a great grandfather with colonic carcinoma. The family history in our group, therefore, did not show a significant increased incidence of intestinal polyps or carcinoma in the patient’s relatives. Symptoms. The most frequent finding was mild melena which occurred in 90 per cent of all cases. (Fig. 4.) Marked melena, however, was present in but 6 per cent. The second most common finding was prolapse of rectal polyps, which was observed in 22 per cent. Abdominal pain was present in 16 per cent and appeared to be more common in patients with multiple polyps. Self-amputation or sloughing of polyps was reported in 12 per cent. In patients in whom the location of these polyps could not be determined, these polyps were arbitrarily listed as rectal polyps. Constipation was reported in 8 per cent of all children and diarrhea in 2 per cent. Anemia (hemoglobin below 10 gm.), was present in only 4 per cent of our patients. Vomiting occurred in 4 per cent of all patients, and had no apparent relation to the number of intestinal polyps. Cabrera [IO] reported thirty-two patients, nine of whom showed mild anemia and three severe anemia. In Gordon’s [6] group, ninety-
FIG. 5. Distribution of 144 countable intestinal polyps observed in fifty children. Fifty-eight per cent of the individual polyps are within reach of the sigmoidoscope.
nine patients of 104 had bright blood in the rectum. “Hemorrhage,” however, was found in only four patients. Excessive mucous material was found in thirty patients, prolapse of intestinal polyps in nineteen, vomiting in six, abdominal pain in twenty-nine, and diarrhea in twenty-six. Diarrhea was commonest in patients with multiple polyps. Kennedy [7] and Horrilleno [II] also described diarrhea as one of the symptoms of intestinal polyps. Intussusception as a complication of intestinal polyps in children is rarely reported. Distribution of Polyps. Figure 5 shows the distribution of 144 countable polyps in fifty children under the age of thirteen years. Fifty polyps were found in the lower rectum, thirtyfour polyps in the lower sigmoid. Fifty-eight per cent of all polyps were, therefore, within the reach of the sigmoidoscope. Twenty-nine polyps were found in the left colon above the region of the sigmoidoscope, twenty in the transverse colon, eight in the ascending colon, one polyp in the ileum, and two polyps in the jejunum. Comparison of the distribution of solitary and multiple polyps (Fig. 6) shows that 93 per cent of the solitary polyps were present in the lower rectum and in the sigmoid colon. Only two polyps were found in the left upper colon and one polyp in the ascending colon. There were eight patients in our group with two to nine polyps, (a total of twenty-three polyps), in whom 87 per cent could have been seen by American Journal
of Surgery
Intestinal NO. PATIENT:
Polyps
7 I3
and Carcinoma
36 PATIENTS
8 PATIENTS
SOLITARY
2-Y POLYPS
_____ TYPE POLYP:
io+ POLYPS
TOTAL: 144
REClUM
I 122
62%
15
15%
50
35%
SIGMOID
Ill
31%
5
21%
34
24%
2
4%
3
1
2%
36% ' 84
56%
DESC.
COLON
TRANSV.
"
ASCDG.
"
ILEUM JWUNUM TOTAL NUMBER POLYPS/GROUP
36
23
WITHIN REACH OF SIGMOIDOSCOPE: b
33
92%
20
0
FIG. 6. Comparison of the distribution polyps in fifty children.
Fifteen polyps were present in the lower rectum, five polyps in the lower sigmoid, and three polyps in the left upper colon. The distribution in patients with multiple polyps differs, however. In patients with over ten polyps (six patients with a total of eighty-five polyps), the number of polyps which could be found within the rectum and sigmoid colon was considerably smaller. Only 36 per cent of all polyps in patients with more than ten polyps were lvithin the reach of the sigmoidoscope. Twenty-four polyps were found in the left descending colon, twenty in the transverse colon, and seven in the ascending colon. One polyp ~vaas in the ileum and two polyps in the jejunum. Horrilleno [II] found 76.7 per cent of all polyps were in the rectum, 14.6 per cent in the sigmoid colon, and less than 9 per cent above the sigmoid colon; 27.3 per cent had multiple polyps, 10 per cent had two polyps, and 3 per cent three polyps: 2 per cent had three or more polyps. Mauro [12] found in twenty-five patients with intestinal polyps, nineteen solitary polyps: thirteen in the rectum, six in the sigmoid colon. Gordon [6] found that 51 per cent of all polyps (one hundred cases of children with polyps) could be reached with a sigmoidoscope. Even though 58 per cent of all polyps in our group are located within the region of the sigmoidoscope. there remain 42 per cent which are sigmoidoscopy.
Vol. 110. Novembtv 1965
85 I 87% / 31
0
of 144 solitary and multiple intestinal
above the sigmoid colon. This finding would indicate that all patients with rectal polyps should have a diagnostic air contrast barium enema study. Treatment: Pathology and Recurrence. The treatment of polyps in the rectum and sigmoid colon within the reach of the sigmoidoscope consisted of either excision or excision and fulguration. In children proctoscopy was carried out under general anesthesia. For polyps above the reach of the sigmoidoscope one or more colotomies were generally performed in symptomatic patients. Histologic examination of the excised polyps showed that there were three patients with adenomatous polyps and the remaining were classified as juvenile or inflammatory polyps. Two of the three patients with adenomatous polyps had more than ten polyps, in one of whom carcinoma developed. The third patient had a solitary adenomatous lesion. Of thirty-three patients with solitary polyps located within the rectum or lower sigmoid, five patients were followed and only one showed a recurrence within the rectum. Eight patients who had between two to ten polyps underwent excision and fulguration of rectal or sigmoid polyps. Three patients were followed, all of whom showed either recurrent or new polyps within the rectum. Patients with more than ten polyps had local recurrences after every excision and fulguration of rectal and sigmoidal
Kottmeier and Clatworthy
714
8 PATIENTS
FIG. 7. Postoperative recurrence of intestinal polyps.
polyps. It should be understood that “recurrence” could be either a true recurrence or new formation of polyps. For obvious reasons it is impossible in most cases to differentiate between the two forms. Figure 7 indicates that patients with multiple polyps showed a higher incidence of local recurrence. The low number of recurrence in patients with solitary polyps, however, that is only one patient, does not necessarily indicate that this was the only patient with recurrent polyps, since repeated studies are not generally carried out on asymptomatic patients. One may safely assume that patients with multiple polyps were more symptomatic and recurrent polyps could, therefore, more readily be suspected and found. Colotomies were performed in three patients with solitary polyps, two of whom were followed. In these two patients no recurrence was noted. In two of the group with two to ten polyps, colotomies were performed. Both patients were followed up and one showed a rectal recurrence. There was no apparent colonic recurrence. By contrast, in the group with multiple polyps, that is ‘more than ten polyps, recurrences of colonic polyps were found after every single colotomy. COMMENTS
The therapy of intestinal polyps in children should depend on the “natural history” of these polyps. This “natural history” will depend .upon whether the polyp is of the “adenomatous” or the “juvenile” type. In recent years several authors [9,11,13,15] have called attention to the histologic and clinical differ-
ence of the juvenile and adenomatous polyps. The juvenile polyp, which histologically differs from the adenomatous polyp by the absence of true neoplastic features and the presence of cystically dilated glands and inflammatory stroma, does not become malignant nor is it associated with carcinoma of the colon or rectum [16]. The majority of intestinal polyps in children are of this juvenile type, which apparently has a limited life span and usually disappears spontaneously. Although more common in children, such polyps have also been observed in adults. The adenomatous polyp, on the other hand, is rarely seen in children. These polyps are considered to be true tumors [13], and carcinomas have been found in association with such lesions in children [7,11]. The histologic separation of juvenile and adenomatous polyps and the clinical implication has not been accepted by all pathologists. Gelb, and co-workers [17] described coexistent adenomatous and juvenile polyps in some patients and believes that the i‘juvenile” polyp represents one stage in the reaction of the adenomatous polyp to the inflammatory changes that follow surface erosion. In our group of fifty children it is possible to differentiate juvenile and adenomatous polyps in most cases. All patients had either one or the other type. The juvenile and adenomatous polyp did not occur simultaneously in the same patient. In some cases the polyps will be found which grossly have the appearance of an intestinal polyp and on histologic examination shows a inflammatory granuloma without polypoid, epithelial or glandular elements. These “inAmerican
Journal of Surgery
Intestinal
flammatory polyps” were seen, for instance, in the reported case of the twelve year old girl with the adenomatous polyps and the associated carcinoma. At the present time we believe the treatment of intestinal polyps in children should be based on two factors: histologic appearance of the polyp and the symptomatology produced by the lesion. Excisional biopsy is obviously necessary to differentiate between the juvenile and adenomatous polyp. In most cases, with the exception of “inflammatory polyps” without glandular elements, such a differentiation will be histologically possible. In patients with single polyps excisional biopsy is indicated for diagnosis and treatment. If a juvenile polyp is found, we do not believe that a close follow-up with repeated barium enemas is warranted unless symptoms recur. If an adenomatous polyp is found, the patient should be followed up closely with diagnostic air contrast studies and sigmoidoscopy. In patients with asymptomatic multiple polyps, the finding of a juvenile polyp indicates that the other polyps are also of the juvenile type and that no further surgery is indicated. Since only an occasional patient will have melena severe enough to produce anemia, the usual finding of occasional mild melena does not present an indication for repeated surgery. However, in the patient with multiple adenomatous polyps or familial polyposis the question is not whether surgery is indicated, but the extent of the surgery to be performed [18]. Subtotal colectomy with fulguration of the rectal polyps or total colectomy and an ileostomy should be performed [9]. The most difficult therapeutic dilemma arises in patients with multiple symptomatic juvenile polyps located beyond the reach of the sigmoidoscope. In such cases the surgeon must decide between multiple colotomies or subtotal colectomy. In six children with more than ten intestinal polyps, two were found to have adenomatous polyps and four juvenile polyps. All of the patients with more than ten polyps who underwent colotomies had recurrence and eventually required subtotal colectomy to control symptoms. In retrospect, an elective primary subtotal colotomy would appear to have been a reasonable procedure initially since the morbidity would be less. Previous reports [18] have shown that such subtotal colectomies do not impair growth in children. Vol. 110, November
1965
7 I.5
Polyps and Carcinoma CONCLUSIONS
A study
of fifty children
with 1-M intestinal
polyps, including one case of coexistent carcinoma, is presented. The clinical and pathologic findings and a review of the literature have led us to the following conclusions : 1. Excision biopsy is indicated to determine the histologic diagnosis. In more than half of the patients, this is readily accomplished by sigmoidoscopy alone. 2. Most intestinal polyps in children are of the “juvenile” type and the indications for their removal depend on the severity of the symptoms they produce. We do not consider that occasional mild melena warrants major surgery in patients in whom the histologic diagnosis of juvenile polyp has been previously made. 3. Scattered symptomatic “juvenile” polyps are best treated by subtotal colectomy rather than repeated colotomies. 4. Adenomatous polyps should routinely be removed by local excision or, if multiple, by subtotal colectomy even in an asymptomatic patient. REFERENCES
1. BONELLI, W. Malignant tumors of the small and large intestines in infants and children. Pr~c. Children’s Hospital, 3: 151, 1947. 2. CARRINGTON, W. Carcinoma of the colon in childhood. Ann. Surg., 39: 816, 1954. 3. JOHNSON, J. W., JUDD, E. W., and DAHLIX, D. C. Malignant neoplasms of the colon and rectum in young persons. Arch. Surg., 79 : 321, 1959. 4. NELSON, C. E., BARR, R. M., and DEEB, P. H. Carcinoma of the colon in children. _4m. J. .%7g., 7x: 531, 1949. 5. OGILVIE, R. F. Mucoid carcinoma of the caecum in a boy of thirteen years. Brit. J. Szq., 23: 601, 1936. 6. GORDON, D. L., HALLENBECK, G. E., DOCKERTY, M. B., KENNEDY, R. L. J., and JACKXAN, R. J. Polyps of the colon in children. Arch. Surg., 75: 90, 1957. 7. KENNEDY, R. L. J. Polyps of the rectum and colon in infants and children. ilm. J. Dis. Child., 62: 181, 1941. 8. CHURCH, R. E. and SCHWARTZ, D. Polyps of the rectum and colon in children. J. Pediat., 44: 104, 1954. 9. RA~ITCH, M. M. Polypoid adenomatosis of the entire gastrointestinal tract. Ann. Surg., 128: 233, 1948. 10. CABRERA, A. and LEGA, J. Polyps of the colon and rectum in children. Am. J. Surg., 100: 551, 1960. 11. HORRILLENO, E. G., ECKERT, C., and ACKERMAX., L. V. Polyps of the rectum and colon in chiIdren. Cancer, 10: 1, 1957. 12. MAURO, J. and PRIOR, J. T. Gastrointestinal poly-
Kottmeier and Clatworthy poid lesions in childhood. Cancer, 10: 131, 1957. 13. HELWIG, E. B. Adenomas of the large intestines in children. Am. J. Dis. Child., 72: 289, 1946. 14. FLOTTE, C. T., O’DELL, F. C., JR., and COLLER, F. A. Polyposis of the colon. Ann. Surg., 144: 165, 1956. 15. ANDRE’N, L. and FRIEDBERG, S. Spontaneous regression of polyps of the colon in children. Acta radial., 46: 507, 1956.
16. TURRELL, R. and MAYNARD, A. DE L. Adenomas of the rectum and colon in juvenile patients. J.A.M.A., 161:57, 1956. 17. GELB, A. M., MINKOWITZ, S., and TRESSER, M. Rectal and colonic polyps occurring in young people. New York J. Med., 62: 513, 1962. 18. COLEMAN, S. T. and ECKERT, C. Preservation of rectum in familial polyposis of the colon and rectum. Arch. Szrrg., 73: 635, 1956.
Polyps and Associated
Carcinoma
in Childhood* PETER K. KOTTMEIER,
M.D., Brooklyn, New York, AND II. WILLIAM CLATWORTHY, JR., ~I.D.,
Columbus, Ohio
From the Department of Surgery, The Ohio State University College of Medicine and The Children’s Hospital, Columbus, Ohio.
intestinal polyps. .4t sigmoidoscopy three polyps were seen in the sigmoid colon between 15 and 20 cm. above the dentate line. On October 9, 1959 these polyps were removed and their bases fulgurated. The patient was readmitted March 3, 1960 because one month prior to admission, she again noted bloody streaks in her stools. There were periods of constipation but no pain or weight loss. At sigmoidoscopy five polyps measuring up to 8 mm. in diameter were found between 4 to 8 cm. above the dentate line. (Fig. 1.) These polyps were removed and the base fulgurated. Microscopic examination showed focal ulceration with acute and chronic inflammation and mild epithelial atypia. The atypia noted was interpreted as the result of chronic inflammation. On June 20, 1960, two months after the second removal of polyps, she returned for the fourth time. She had been asymptomatic until three weeks before when blood streaked stools again occurred. So other symptoms were present. Physical examination again was within normal limits, including rectal examination. The barium enema showed a filling defect in the lower descending colon, opposite the left sacroiliac joint, with a second small defect adjacent to it. Sigmoidoscopy demonstrated two polyps 7 and 10 cm. above the dentate line with broad bases. These polyps were removed by snare and the bases fulgurated. The areas where previous polyps had been removed failed to show any recur-rence. Microscopic examination showed polyps with ulceration and granulation tissue. On October 19, 1960, three months after the third removal of intestinal polyps, the patient was readmitted for the fifth time because of continued bright bloody streaked stools. At sigmoidoscopy, one polyp 7 cm. above the dentate line on the posterior wall of the rectum was found. This polyp had a broad base. It was removed by snare and the base was cauterized. Microscopic examination showed superficial ulceration and chronic inflammatory
carcinoma of the colon has been in children [l-5], but the association of intestinal carcinoma with intestinal polyps is rare in childhood [6,7]. Attention has been called to the fact that there is a significant difference in the pathologic and clinical behavior of polyps of rectum and colon in children as compared with those in adults [a]. It is generally assumed that intestinal polyps in children almost never undergo malignant transformation, or that carcinoma of the colon rarely exists simultaneously with intestinal polyps in young people. This has also been our experience, until recently carcinoma of the descending colon developed in one of our patients while under observation and treatment for multiple intestinal polyps.
I SOLATED reported
CASE REPORT K. L. (CH No. 94822), a twelve year old white girl, was first admitted to Children’s Hospital at the age of three years. The child was reported to have been anemic since birth, in spite of continuous iron therapy for two years. Congenital hemolytic anemia was diagnosed. Splenectomy was performed with complete recovery. The child was readmitted at the age of ten years. At this time the patient entered with the chief complaint of bright red blood in her stools for forty days prior to admission. There was no history of familial polyposis, and no known relatives had died of colonic carcinoma. Physical examination was within normal limits. Barium enema failed to show any evidence of * Presented
at the Seventeenth
Vol. 110. .‘Vovembev 1065
Annual Meeting
of the Southwestern May 10-13, 1965. 709
Surgical Congress, Hot Springs, i\rkansas,
Kottmeier and Clatworthy
FIG. 1. Summary of distribution and therapy of multiple adenomatous polyps and coexistent carcinoma of the colon in a twelve year old girl. infiltration. The glandular structures showed pseudostratification, and moderate pleomorphism with hyperchromatism. In most areas the basement membrane appeared to be intact; in a few areas, however, a breakthrough could not be ruled out. The polyp was considered to show atypical changes and a close follow-up of the patient was suggested. The patient was then readmitted, after four months, on February 1, 1961 with some diarrhea and rectal bleeding for three weeks. Barium enema showed a filling defect in the mid-transverse colon as well as sigmoid colon. At sigmoidoscopy there were no recurrent polyps up to 20 cm. above the dentate
A
line. .4n exploratory laparotomy was performed and the entire colon mobilized. Transillumination as well as palpation disclosed four polyps, the largest in the lower sigmoid colon. Proximal to this polyp, which measured approximately 4 cm. in diameter, was a smaller polyp approximately 2 cm. in diameter. A 2 to 3 cm. polyp was found in the transverse colon and another in the ascending colon. Examination of the remaining intestine up to the ligament of Treitz showed no other polyps. A colotomy was performed in the ascending colon, and a pedunculated polyp, which measured 2 by 3 cm., was removed with a base of normal-appearing mucosa. A second cototomy was performed in the transverse colon, where a larger polyp was excised in a similar fashion. A third colotomy was then performed in the sigmoid colon and an attempt was made to remove both polyps. The most distal polyp, which measured 4 cm. in diameter, showed what appeared to be atypical changes in the surrounding mucosa with slight puckering and adherence to the underlying muscularis. A segmental sleeve resection, which included both polyps, was therefore performed, leaving at least 4 cm. of normal bowel proximal and distal to both polyps. An incidental appendectomy was also performed. Microscopic section of the largest polyp, situated in the sigmoid colon, showed an adenocarcinoma with infiltration of the submucosa and lymphatic channels by glandular cells. (Fig. 2A and B.) These cells showed hyperchromic nuclei with loss of polarity. The underlying muscularis was not invaded. Resected lymph nodes showed no metastases. The patient had an uneventful postoperative recovery and was readmitted after four weeks on
B
FIG. 2. A and B, photomicrographs of adenocarcinoma of colon in patient with multiple adenomatous polyps; magnification X 200 and 400, respectively, before reduction. American
Journal of
Surgery
Intestinal
Polyps
March 3, 1’31iI l’or an elective subtotal colectomy and ileoljr:“tostc,nl~. 1+1xploration of the abdomen at this time again showed no evidence of metastases. A sul)tri;al colectomy, including M cm. of terminal ileum was pt!riormed. .\pproxintatcly I1 cm. of rectum remained Microscopic examination of tifty mesenteric Iyml)h nodes failed to show any signs of metastasis. Tlic resected colon showed four more polyps in the transverse colon and three polyps in the left descending colon, all benign on microscopic examination. ‘The patient had an uneventful postoperative reco\-cry and was discharged after six days. Fi\le months postcolectomy the patient h-as readmitted for routine sigmoidoscopy. The patient had been asymptomatic since the subtotal colectomy and had two to three stools daily. She had gained 13 pounds since the time of colectomy. Physical examination again was within normal limits. Sigmoidoscopy was performed which showed a normal terminal ileum with a patent anastomosis at the site of the ileoproctostomy. Just below the anastomosis at :! : 00, there was a single small polyp present which was removed and the base cauterized. Xl pol?;ps removed in this patient were of the adenomatous type except for a few which showed extensive inflammation without identifiable glandular elements. SUMMARY
OF CASE MATERIAL
Forty-nine additional infants and children with intestinal polyps, admitted between 1950 and 1960 to the Columbus Children’s Hospital were also studied. In these fifty patients a total of 144 polyps were found. Incidence ilccording to Age. The youngest patient in our group was four days of age, the oldest patient had his first admission for intestinal polyps at the age of thirteen years. The
age distribution (Fig. 3) shows that a considerable number of polyps were found between birth and the age of one year (eight polyps). However. the largest number is found in children between the age of three and six years (twenty-one polyps). Subsequently, there appears to be a steady decline in number during the prepuberal years. This decline suggests that there is a natural tendency for polyps in children to disappear during puberty. Comparing our findings with other reported cases, a similar age incidence is found, with the exception of the first year of life, wherein fewer patients are usually reported. Ravitch [9] reported polypoid adenomas of the entire gastrointestinal tract in an eighteen month old child. Cabrera [lo] found in autopsy specimens of Vol. 110. November 1965
and Carcinoma
;‘I1
thirty-two children, that one patient was in the one to two year group, nine in the three to four year group, and nine in the five to six year group with a decline in the older ages. Horrilleno [ll] gave 4.7 years as the average age for intestinal polyps in children. IMauro [12] reported twenty-five cases between two to eleven years, with the average age of 5.4 years. Helwig [13] found six polyps in 449 autopsies of children under twenty-one years of age; none, however, under the age of three years. Family History. It is often impossible to decide in the individual case, whether one is dealing with “multiple scattered polyps” or true “polyposis.” A positive family history of either “familial polyposis” or carcinoma of the lower intestine may, therefore, be of help in differentiating the two, probably unrelated, entities. Gordon [C;] used the term “diffuse polyposis” for patients with myriads of polyps, whereas patients with ten to one hundred polyps were included under the term “polyposis.” Gordon found a positive family history in children with polyposis in one of six patients with diffuse polyposis, three of eight patients with ten to one hundred polyps and three of ninety patients with one to nine polyps. Four of the latter had relatives with carcinoma of the colon. Flotte [14] reported sixty-three patients with intestinal polyposis, 34 per cent with a positive family history. In our group, a family history of either polyposis or intestinal carcinoma was not helpful. In patients with solitary polyps two had a positive family history; one patient’s
712
FIG. 4. Symptom tinal polyps.
Kottmeier
and Clatworthy
incidence in fifty children with intes-
grandfather was reported as having had a colonic carcinoma. The mother of a four months’ old patient with a solitary adenomatous polyp had a history of multiple intestinal polyps, In patients with two to ten polyps the family history was entirely negative. In patients with more than ten polyps the father of one patient had a single polyp removed as a child, followed by negative barium enemas. One other patient in the same group had a great grandfather with colonic carcinoma. The family history in our group, therefore, did not show a significant increased incidence of intestinal polyps or carcinoma in the patient’s relatives. Symptoms. The most frequent finding was mild melena which occurred in 90 per cent of all cases. (Fig. 4.) Marked melena, however, was present in but 6 per cent. The second most common finding was prolapse of rectal polyps, which was observed in 22 per cent. Abdominal pain was present in 16 per cent and appeared to be more common in patients with multiple polyps. Self-amputation or sloughing of polyps was reported in 12 per cent. In patients in whom the location of these polyps could not be determined, these polyps were arbitrarily listed as rectal polyps. Constipation was reported in 8 per cent of all children and diarrhea in 2 per cent. Anemia (hemoglobin below 10 gm.), was present in only 4 per cent of our patients. Vomiting occurred in 4 per cent of all patients, and had no apparent relation to the number of intestinal polyps. Cabrera [IO] reported thirty-two patients, nine of whom showed mild anemia and three severe anemia. In Gordon’s [6] group, ninety-
FIG. 5. Distribution of 144 countable intestinal polyps observed in fifty children. Fifty-eight per cent of the individual polyps are within reach of the sigmoidoscope.
nine patients of 104 had bright blood in the rectum. “Hemorrhage,” however, was found in only four patients. Excessive mucous material was found in thirty patients, prolapse of intestinal polyps in nineteen, vomiting in six, abdominal pain in twenty-nine, and diarrhea in twenty-six. Diarrhea was commonest in patients with multiple polyps. Kennedy [7] and Horrilleno [II] also described diarrhea as one of the symptoms of intestinal polyps. Intussusception as a complication of intestinal polyps in children is rarely reported. Distribution of Polyps. Figure 5 shows the distribution of 144 countable polyps in fifty children under the age of thirteen years. Fifty polyps were found in the lower rectum, thirtyfour polyps in the lower sigmoid. Fifty-eight per cent of all polyps were, therefore, within the reach of the sigmoidoscope. Twenty-nine polyps were found in the left colon above the region of the sigmoidoscope, twenty in the transverse colon, eight in the ascending colon, one polyp in the ileum, and two polyps in the jejunum. Comparison of the distribution of solitary and multiple polyps (Fig. 6) shows that 93 per cent of the solitary polyps were present in the lower rectum and in the sigmoid colon. Only two polyps were found in the left upper colon and one polyp in the ascending colon. There were eight patients in our group with two to nine polyps, (a total of twenty-three polyps), in whom 87 per cent could have been seen by American Journal
of Surgery
Intestinal NO. PATIENT:
Polyps
7 I3
and Carcinoma
36 PATIENTS
8 PATIENTS
SOLITARY
2-Y POLYPS
_____ TYPE POLYP:
io+ POLYPS
TOTAL: 144
REClUM
I 122
62%
15
15%
50
35%
SIGMOID
Ill
31%
5
21%
34
24%
2
4%
3
1
2%
36% ' 84
56%
DESC.
COLON
TRANSV.
"
ASCDG.
"
ILEUM JWUNUM TOTAL NUMBER POLYPS/GROUP
36
23
WITHIN REACH OF SIGMOIDOSCOPE: b
33
92%
20
0
FIG. 6. Comparison of the distribution polyps in fifty children.
Fifteen polyps were present in the lower rectum, five polyps in the lower sigmoid, and three polyps in the left upper colon. The distribution in patients with multiple polyps differs, however. In patients with over ten polyps (six patients with a total of eighty-five polyps), the number of polyps which could be found within the rectum and sigmoid colon was considerably smaller. Only 36 per cent of all polyps in patients with more than ten polyps were lvithin the reach of the sigmoidoscope. Twenty-four polyps were found in the left descending colon, twenty in the transverse colon, and seven in the ascending colon. One polyp ~vaas in the ileum and two polyps in the jejunum. Horrilleno [II] found 76.7 per cent of all polyps were in the rectum, 14.6 per cent in the sigmoid colon, and less than 9 per cent above the sigmoid colon; 27.3 per cent had multiple polyps, 10 per cent had two polyps, and 3 per cent three polyps: 2 per cent had three or more polyps. Mauro [12] found in twenty-five patients with intestinal polyps, nineteen solitary polyps: thirteen in the rectum, six in the sigmoid colon. Gordon [6] found that 51 per cent of all polyps (one hundred cases of children with polyps) could be reached with a sigmoidoscope. Even though 58 per cent of all polyps in our group are located within the region of the sigmoidoscope. there remain 42 per cent which are sigmoidoscopy.
Vol. 110. Novembtv 1965
85 I 87% / 31
0
of 144 solitary and multiple intestinal
above the sigmoid colon. This finding would indicate that all patients with rectal polyps should have a diagnostic air contrast barium enema study. Treatment: Pathology and Recurrence. The treatment of polyps in the rectum and sigmoid colon within the reach of the sigmoidoscope consisted of either excision or excision and fulguration. In children proctoscopy was carried out under general anesthesia. For polyps above the reach of the sigmoidoscope one or more colotomies were generally performed in symptomatic patients. Histologic examination of the excised polyps showed that there were three patients with adenomatous polyps and the remaining were classified as juvenile or inflammatory polyps. Two of the three patients with adenomatous polyps had more than ten polyps, in one of whom carcinoma developed. The third patient had a solitary adenomatous lesion. Of thirty-three patients with solitary polyps located within the rectum or lower sigmoid, five patients were followed and only one showed a recurrence within the rectum. Eight patients who had between two to ten polyps underwent excision and fulguration of rectal or sigmoid polyps. Three patients were followed, all of whom showed either recurrent or new polyps within the rectum. Patients with more than ten polyps had local recurrences after every excision and fulguration of rectal and sigmoidal
Kottmeier and Clatworthy
714
8 PATIENTS
FIG. 7. Postoperative recurrence of intestinal polyps.
polyps. It should be understood that “recurrence” could be either a true recurrence or new formation of polyps. For obvious reasons it is impossible in most cases to differentiate between the two forms. Figure 7 indicates that patients with multiple polyps showed a higher incidence of local recurrence. The low number of recurrence in patients with solitary polyps, however, that is only one patient, does not necessarily indicate that this was the only patient with recurrent polyps, since repeated studies are not generally carried out on asymptomatic patients. One may safely assume that patients with multiple polyps were more symptomatic and recurrent polyps could, therefore, more readily be suspected and found. Colotomies were performed in three patients with solitary polyps, two of whom were followed. In these two patients no recurrence was noted. In two of the group with two to ten polyps, colotomies were performed. Both patients were followed up and one showed a rectal recurrence. There was no apparent colonic recurrence. By contrast, in the group with multiple polyps, that is ‘more than ten polyps, recurrences of colonic polyps were found after every single colotomy. COMMENTS
The therapy of intestinal polyps in children should depend on the “natural history” of these polyps. This “natural history” will depend .upon whether the polyp is of the “adenomatous” or the “juvenile” type. In recent years several authors [9,11,13,15] have called attention to the histologic and clinical differ-
ence of the juvenile and adenomatous polyps. The juvenile polyp, which histologically differs from the adenomatous polyp by the absence of true neoplastic features and the presence of cystically dilated glands and inflammatory stroma, does not become malignant nor is it associated with carcinoma of the colon or rectum [16]. The majority of intestinal polyps in children are of this juvenile type, which apparently has a limited life span and usually disappears spontaneously. Although more common in children, such polyps have also been observed in adults. The adenomatous polyp, on the other hand, is rarely seen in children. These polyps are considered to be true tumors [13], and carcinomas have been found in association with such lesions in children [7,11]. The histologic separation of juvenile and adenomatous polyps and the clinical implication has not been accepted by all pathologists. Gelb, and co-workers [17] described coexistent adenomatous and juvenile polyps in some patients and believes that the i‘juvenile” polyp represents one stage in the reaction of the adenomatous polyp to the inflammatory changes that follow surface erosion. In our group of fifty children it is possible to differentiate juvenile and adenomatous polyps in most cases. All patients had either one or the other type. The juvenile and adenomatous polyp did not occur simultaneously in the same patient. In some cases the polyps will be found which grossly have the appearance of an intestinal polyp and on histologic examination shows a inflammatory granuloma without polypoid, epithelial or glandular elements. These “inAmerican
Journal of Surgery
Intestinal
flammatory polyps” were seen, for instance, in the reported case of the twelve year old girl with the adenomatous polyps and the associated carcinoma. At the present time we believe the treatment of intestinal polyps in children should be based on two factors: histologic appearance of the polyp and the symptomatology produced by the lesion. Excisional biopsy is obviously necessary to differentiate between the juvenile and adenomatous polyp. In most cases, with the exception of “inflammatory polyps” without glandular elements, such a differentiation will be histologically possible. In patients with single polyps excisional biopsy is indicated for diagnosis and treatment. If a juvenile polyp is found, we do not believe that a close follow-up with repeated barium enemas is warranted unless symptoms recur. If an adenomatous polyp is found, the patient should be followed up closely with diagnostic air contrast studies and sigmoidoscopy. In patients with asymptomatic multiple polyps, the finding of a juvenile polyp indicates that the other polyps are also of the juvenile type and that no further surgery is indicated. Since only an occasional patient will have melena severe enough to produce anemia, the usual finding of occasional mild melena does not present an indication for repeated surgery. However, in the patient with multiple adenomatous polyps or familial polyposis the question is not whether surgery is indicated, but the extent of the surgery to be performed [18]. Subtotal colectomy with fulguration of the rectal polyps or total colectomy and an ileostomy should be performed [9]. The most difficult therapeutic dilemma arises in patients with multiple symptomatic juvenile polyps located beyond the reach of the sigmoidoscope. In such cases the surgeon must decide between multiple colotomies or subtotal colectomy. In six children with more than ten intestinal polyps, two were found to have adenomatous polyps and four juvenile polyps. All of the patients with more than ten polyps who underwent colotomies had recurrence and eventually required subtotal colectomy to control symptoms. In retrospect, an elective primary subtotal colotomy would appear to have been a reasonable procedure initially since the morbidity would be less. Previous reports [18] have shown that such subtotal colectomies do not impair growth in children. Vol. 110, November
1965
7 I.5
Polyps and Carcinoma CONCLUSIONS
A study
of fifty children
with 1-M intestinal
polyps, including one case of coexistent carcinoma, is presented. The clinical and pathologic findings and a review of the literature have led us to the following conclusions : 1. Excision biopsy is indicated to determine the histologic diagnosis. In more than half of the patients, this is readily accomplished by sigmoidoscopy alone. 2. Most intestinal polyps in children are of the “juvenile” type and the indications for their removal depend on the severity of the symptoms they produce. We do not consider that occasional mild melena warrants major surgery in patients in whom the histologic diagnosis of juvenile polyp has been previously made. 3. Scattered symptomatic “juvenile” polyps are best treated by subtotal colectomy rather than repeated colotomies. 4. Adenomatous polyps should routinely be removed by local excision or, if multiple, by subtotal colectomy even in an asymptomatic patient. REFERENCES
1. BONELLI, W. Malignant tumors of the small and large intestines in infants and children. Pr~c. Children’s Hospital, 3: 151, 1947. 2. CARRINGTON, W. Carcinoma of the colon in childhood. Ann. Surg., 39: 816, 1954. 3. JOHNSON, J. W., JUDD, E. W., and DAHLIX, D. C. Malignant neoplasms of the colon and rectum in young persons. Arch. Surg., 79 : 321, 1959. 4. NELSON, C. E., BARR, R. M., and DEEB, P. H. Carcinoma of the colon in children. _4m. J. .%7g., 7x: 531, 1949. 5. OGILVIE, R. F. Mucoid carcinoma of the caecum in a boy of thirteen years. Brit. J. Szq., 23: 601, 1936. 6. GORDON, D. L., HALLENBECK, G. E., DOCKERTY, M. B., KENNEDY, R. L. J., and JACKXAN, R. J. Polyps of the colon in children. Arch. Surg., 75: 90, 1957. 7. KENNEDY, R. L. J. Polyps of the rectum and colon in infants and children. ilm. J. Dis. Child., 62: 181, 1941. 8. CHURCH, R. E. and SCHWARTZ, D. Polyps of the rectum and colon in children. J. Pediat., 44: 104, 1954. 9. RA~ITCH, M. M. Polypoid adenomatosis of the entire gastrointestinal tract. Ann. Surg., 128: 233, 1948. 10. CABRERA, A. and LEGA, J. Polyps of the colon and rectum in children. Am. J. Surg., 100: 551, 1960. 11. HORRILLENO, E. G., ECKERT, C., and ACKERMAX., L. V. Polyps of the rectum and colon in chiIdren. Cancer, 10: 1, 1957. 12. MAURO, J. and PRIOR, J. T. Gastrointestinal poly-
Kottmeier and Clatworthy poid lesions in childhood. Cancer, 10: 131, 1957. 13. HELWIG, E. B. Adenomas of the large intestines in children. Am. J. Dis. Child., 72: 289, 1946. 14. FLOTTE, C. T., O’DELL, F. C., JR., and COLLER, F. A. Polyposis of the colon. Ann. Surg., 144: 165, 1956. 15. ANDRE’N, L. and FRIEDBERG, S. Spontaneous regression of polyps of the colon in children. Acta radial., 46: 507, 1956.
16. TURRELL, R. and MAYNARD, A. DE L. Adenomas of the rectum and colon in juvenile patients. J.A.M.A., 161:57, 1956. 17. GELB, A. M., MINKOWITZ, S., and TRESSER, M. Rectal and colonic polyps occurring in young people. New York J. Med., 62: 513, 1962. 18. COLEMAN, S. T. and ECKERT, C. Preservation of rectum in familial polyposis of the colon and rectum. Arch. Szrrg., 73: 635, 1956.