Colorectal Carcinoma in Children

By ibrahim Karnak, Arbay 0. Ciftci, Mehmet Emin Senocak, and Nebil Bijyiikpamukqzu
Ankara, Turkey

Bac&ground/Purpose:Colorectal carcinoma is extremely rare patients. Predominant histological type was mutinous adeno-
in children and presents with a poor prognosis. Surgical carcinoma (80%). All patients but one received adjuvant
management and long-term follow-up of this entity are still chemotherapy, and 2 received palliative radiotherapy. Thir-
obscure because of lack of data. Therefore, a retrospective teen patients died of disease in a period ranging from 1 day to
clinical trial was performed to evaluate the clinical character- 1 year after initial surgery. The fate of 4 patients who were
istics of childhood colorectal carcinoma and to determine the discharged in a very ill status was unknown. Three patients
predictors of poor outcome. were alive for 2 years to 4 years postoperatively, and one of
them presented with end stage disease.
Methods: Records of children who had colorectal carcinoma and
were treated at our unit between 1972 and 1997, inclusive, were Conclusions: Delayed diagnosis, advanced stages of disease
reviewed retrospectively. Information recorded for each patient at presentation, and, most importantly, mutinous type of
included age, sex, clinical characteristics, diagnostic procedures, histology are the major determinants of poor outcome in
extent of disease, treatment methods, histological types, and childhood colorectal carcinoma. We emphasize that possibil-
outcome. A modified Dukes staging scheme was used. ity of a malignant colorectal tumor should be considered for
Resu/ts:There were 12 boys and 8 girls who were treated for any childhood case with signs and symptoms of intestinal
colorectal carcinoma (range, 7 to 16 years). Predisposing obstruction, intractable abdominal pain, alteration in bowel
conditions were encountered in 2 patients (IO%), one with habits and gastrointestinal bleeding. Colorectal malignancy
Blooms syndrome and another with familial occurrence of should not be excluded only on the basis of the patients age.
colonic carcinomas. Predominant symptoms were abdomi- Because of the steadily increasing incidence of precancerous
nal pain, vomiting, and rectal bleeding. Barium enema, bowel diseases and poor prognosis of colorectal carcinoma,
ultrasonography, computerized tomography, and endo- childhood cases of bowel disorders should receive the same
scopic prbcedures were used for the diagnosis. Rectosig- detailed and vigorous diagnostic evaluation and appropriate
moid region was the most common site for the primary treatment as given to adult cases. Contrast studies, ultraso-
tumor (65%). All patients presented with advanced stages of nography, computed tomography, and endoscopy are essen-
disease (stage C, 7; stage D, 13). Surgical procedures were tial procedures for both confirming the diagnosis and detect-
incisional biopsy (n = 4), palliative permanent colostomy ing the extent of the disease.
(n = 4), segmental resection (n = 5), complete resection J Pediatr Surg 34:1499-7504. Copyright o 1999 by W.B.
(n = 6), and rectal biopsy (n = 1). Peritoneum was the most Saunders Company.
common site of extensive intraabdominal disease followed
by omentum majus and liver. The lung also was involved in 2 INDEX WORDS: Colon, rectum, adenocarcinoma, mutinous.

P RIMARY GASTROINTESTINAL system malignan-

cies constitute approximately 1% of pediatric neo-
plasms, and of these, colorectal carcinoma is the second
retrospectively.
clinical
Information
characteristics,
treatment methods, histological
recorded for each patient included age, sex,
diagnostic procedures, stage of the disease,
type, and outcome. Extent of disease
was determined by using modified Dukes staging scheme.3 According
most common malignancy after primary liver tumors to this classification, in stage A, only the mucosa and submucosa are affected,
with an incidence of one per million.2,3 It may locate at in stage B, the disease. is limited to the bowel walk in stage C, the disease is
any site in the colon and usually occurs sporadically with- limited to the lymph nodes; and in stage D, them are distant metastases or
peritoneal implants or direct invasion of other viscera.
out an associated predisposing factor or family history.3,4
Unlike adult colorectal carcinoma, the overall progno- RESULTS
sis is poor.5 Additionally, the surgical management and
follow-up of children with colorectal carcinoma are still There were 20 children treated for colorectal carci-
obscure because of lack of data. Therefore, a retrospec- noma in the study period. The mean age of the patients at
tive clinical trial was performed to evaluate the clinical presentation was 13.2 t- 2 years (range, 7 to 16 years)
characteristicsof childhood colorectal carcinoma and to deter-
mine the predictors of almost uniformly bad outcome. From the Department of Pediatric Surgery, Hacettepe University
Medical Faculty, Ankara, Turkey.
Address reprint requests to Arbay 0. Cifci, MD, Associate Professor
MATERIALS AND METHODS
of Pediatric Surgery Hacettepe University Medical Faculty, 06100
Records of children who had colorectal carcinoma diagnosed and Ankara, Turkey.
treated at the Department of Pediatric Surgery of Hacettepe University Copyright 8 1999 by WB. Saunders Company
Medical Faculty between 1972 and 1997, inclusive, were reviewed 0022-3468/99/3410-0012$03.00/O

Journal of Pediatric Surgery, Vol34, No 10 (October), 1999: pp 1499-1504 1499

1500 KARNAK ET AL

Table 1. Clinical Characteristics

0% Age (~4 Presenting Symptoms Duration of

NO. and Sex and Signs symptoms Primary Site

1 13, M Abdominal pain, vomiting, mass 15d Cecum

2 16, M Intestinal obstruction 2d Hepatic flexura
3 13, M Abdominal pain, rectal bleeding 6mo Rectum
4 13, M Intestinal obstruction 2d Rectum
5 14, F Abdominal pain, rectal bleeding 1 mo Rectum
6 15, F Abdominal pain, constipation 1 mo Rectum
7 11, F Abdominal pain, constipation 1 mo Rectum
8 14, F Abdominal pain, rectal bleeding 3mo Rectum
9 13, F Abdominal pain, vomiting 4mo Rectosigmoid
10 11, M Intestinal obstruction 10 d Transverse colon
11 12, M Abdominal pain, rectal bleeding 2mo Sigmoid colon
12 16, M Abdominal pain, vomiting 4mo Sigmoid colon
13 14, F Abdominal pain, rectal bleeding 2 vr Rectum
14 13, M Intestinal obstruction 15d Sigmoid colon
15 14, M Abdominal pain, distension 6mo Hepatic flexura
16 13, F Abdominal pain, diarrhea 3mo Transverse colon
17 7. F Abdominal pain, rectal bleeding 4mo Sigmoid colon
18 15, M Abdominal pain, vomiting 2mo Descending colon
19 14, M Abdominal pam, vomiting 5mo Sigmoid colon
20* 14, M Abdominal pain, vomiting, mass 3mo Transverse colon

*With Blooms syndrome.

with a male to female ratio of 3:2. In regard to predispos- was noted in 4 patients and only incisional biopsies could
ing factors, there was 1 patient who had Blooms be taken. In another group of 4 patients, palliative
syndrome diagnosed previously. Father and 2 uncles of permanent colostomies were performed. Segmental resec-
another patient had died of colonic cancer. tion was defined as resection of the involved segment
The predominant symptom was abdominal pain (80%) with margins less than 5 cm with incomplete resection of
followed by rectal bleeding, vomiting, and alteration of the mesentery. Two patients underwent segmental resec-
bowel habits. Weight loss, anemia, and anorexia were
noted in later stages of the disease. Duration of symptoms
before admission in patients with intestinal obstruction
ranged from 2 days to 15 days, whereas in the remaining
patients it ranged from 15 days to 2 years (mean, 4.3
months). Palpable abdominal mass (n = 7) and acute
intestinal obstruction picture (n = 4) were the most
significant physical examination findings.
Primary site of the tumor was rectosigmoid in 12
patients, transverse colon in 3, and hepatic flexure in 2
patients. The cecum, descending colon, and rectosigmoid
junction were the other sites in the remaining 3 patients
(Table 1).
Plain abdominal x-rays showed multiple air-fluid lev-
els (n = 4) and abnormal gas pattern (n = 12). Abdomi-
nal ultrasonography was performed in 10 patients and
detected mass in 7 of them. Barium enema was per-
formed in 11 patients and showed the obstructed segment
or colonic mass in all cases (Figs 1 and 2). Computerized
tomography was performed in 7 patients and showed
intraperitoneal disseminated disease. Rectosigmoidos-
copy (n = 5) and colonoscopy (n = 7) were performed in
association with contrast studies.
Laparotomy findings showed that 7 patients had stage
C and 13 patients had stage D disease. An unresectable Fig 1. Severe obstruction of the sigmoid colon caused by carci-
tumor associated with widespread intraabdominal disease noma (arrows).
COLORECTAL CARCINOMA 1501

and distal ends with its mesentery. Among these patients,

complete resection of the rectum was performed in 3
patients by using Miles operation. Complete resection
also was performed in 3 patients for tumors involving sig-
moid, transverse, and descending colon. One patient with
rectal tumor refused major surgery after rectal biopsy.
Adjacent invasion, tumor seeding, and metastases to
intraabdominal organs were found in all stage D patients.
Peritoneum was the most commonly involved site (n = 10,
83%) followed by omentum (n = 4, 33%) in stage D
patients. The omentum was purely involved in 2 patients
and associated with peritoneal disease in the other 2
patients. Liver and lung were involved in 2 patients and 1
patient, respectively. Additionally, one of stage C patients
progressed to stage D disease with extensive lung and
liver metastases.
Histopathologic examination showed mutinous type
of adenocarcinoma in 16 patients (80%) and adult form of
nonmucinous adenocarcinoma in 4 patients (20%).
All but one patient received adjuvant chemotherapy
consisting of 5-fluorouracil (5-FU; n = 6), 5-PU and
cyclohexylnitrosourea (CCNU; n = 6), 5-PU, Adriamy-
tin and mitomycine (n = 3), and 5-FU and levamisole
Fig 2. Contrast enema shows obstruction at the middle of the
transverse colon (oblique projection, arrows). (n = 4).
There were 3 known long-term survivors in our series
tion and colostomy, and another group of 3 patients had (Table 2). One of them (case 1) had a primary cecal
segmental resection and colocolic anastomosis. Complete tumor, was treated with ileocecal resection and primary
resection was performed in 6 patients by resection of the anastomosis, and received 5-FU plus CCNU for 14
colon with margins of more than 5 cm in both proximal months. He underwent follow-up in continuous remission

Table 2. Surgical Features and Outcome

Case No Surgical Procedure stage* Histology Outcome

1 lleocecal resection & anastomosis C Mutinous Alive for 4 yearst

2 lleocecal resection & anastomosis C Mutinous DOD in 1 yr
3 Miles operation D Nonmucinous DOD in 6 mo
4 Miles operation D Mutinous DOD in 6 mo
5 Colostomy D Mutinous Unknown
6 Colostomy D Mucrnous DOD in 6 mo
7 Resection & colostomy D Mutinous DOD in 8 mo
8 Refused operation D Nonmucinous DOD in 1 mo
9 Miles operation C Nonmucinous DOD in 6 mo
10 Biopsy only D Nonmucinous DODinld
11 Biopsy only D Mutinous Unknown
12 Biopsy only D Mutinous Unknown
13 Colostomy D Mutinous DOD in 1 yr
14 Resection &colostomy C Mutinous Alive for 2 yr
15 lleocecal resection D Mutinous DOD in 12 d
16 Biopsy only D Mutinous DOD in 1 mo
17 Resection & anastomosis C Mutinous Alive for 3 yrS
18 Resection & colostomy C Mutinous DOD in 6 mo
19 Colostomy D Mutinous DODin7d
20 Resection & anestomosis C Mutinous Unknown

Abbreviation: DOD, died of disease.

*Modified Dukes Classification.
tNo further follow-up.
*Readmitted with stage D drsease and then no further follow-up.
SProgressed to stage D and then no further follow-up.
1502
for 4 years. No information concerning him was received
after that time. The second (case 14) had a sigmoid colon
tumor, was treated with resection and colostomy, and
received 5-FU. He is still alive with end-stage disease.
The third (case 17) had a sigmoid colon tumor, was
treated with subtotal colectomy and primary anastomosis
without chemotherapy. However, the patient was lost to
follow-up for 3 years and then she was readmitted with
extensive intraabdominal disease. She received 5-FU
plus levamisole therapy without improvement in the
course of disease.
DISCUSSION
Colorectal carcinoma is extremely rare in children1 and
the current study represents one of the largest series of
colorectal carcinoma in children. Although it has been
reported in newborn and infants,6~7the youngest patient
was a 7-year-old girl, and the remaining patients were
older than 10 years in our series.
Epidemiological studies indicate that environmental
factors are likely to cause bowel cancer, and there is
strong evidence that diet is the predominant environmen-
tal influence.s Familial polyposis of colon and other
polyposis syndromes (Gardners syndrome, Turcots syn-
drome,9 Peutz-Jeghers syndrome, juvenile polyposis
coliO); ulcerative colitis; ureterosigmoidostomy; the fa-
milial occurrence of colorectal cancer (bowel cancer in a
close relative); familial multiple cancer syndromes4**; and
Blooms syndrome are the predisposing pathologies
that increase the risk of development of colorectal
carcinoma. Predisposing factors are noted in 10% of the
childhood colorectal carcinomas,4 which is far higher
than that in adult patients. In the current series, we have
encountered the predisposing factors only in 2 patients,
which represents a 90% spontaneous occurrence similar
to that of previous reports.
Abdominal pain and vomiting were the most common
symptoms in previous reports.1~2,5~8 Abdominal disten-
sion, palpable mass, and change in bowel habits were
observed less frequently as well as weight loss and
anemia. Nonspecific symptoms were considered to be
suggestive of more common pediatric disorders such as
appendicitis, intussusception, and gastroenteritis, thus,
colorectal carcinoma could be easily overlooked.5g13
Sometimes, after a long period of vague gastrointestinal
complaints, typical signs of acute intestinal obstruction
had occurred as noted in our 4 patients. An important
observation was that adolescents generally did not wish
to relate rectal bleeding with malignancy, and were
unlikely to volunteer this information.2 Screening meth-
ods used in adults were not feasible in children.12,14
Therefore, it is very difficult to make an early diagnosis
of a colorectal malignancy during childhood because
benign polyps or functional problems are considered
KARNAK ET AL
more likely in the light of patients age, as was true in our
series. Thus, early diagnosis must depend on a high index
of suspicion. But, unfortunately, delayed diagnosis is still
one of the determinants of poor prognosis.
Striking differences between adult and children colorec-
tal carcinomas have been reported with regard to duration
of symptoms, primary site, pathological findings, stage,
and prognosis.2,8 There is notable preponderance of boys
with colorectal carcinoma as noted in our series, whereas
sex distribution is equal for adults.2*12In adult patients,
60% of the colonic cancer is located within 25 cm of the
anus. The rectum and sigmoid are also common sites for
mutinous adenocarcinoma in adults, occurring in 33%
and 27% of cases, respectively.15 In children, the sites of
tumors were located equally in all parts of the colon.* In
another series reported by Andersson and Bergdahl,16 the
most common area was the transverse colon (39%). In
our series, we have encountered rectosigmoid area as the
most frequent site of location of the primary tumor (65%)
as in adults. There was no correlation between site of
tumor and presenting symptoms except rectal bleeding
associated with rectal tumor. We stress that rectal bleed-
ing prompts careful search of the colon and rectum to
exclude malignancy.
Mutinous or colloidal adenocarcinoma has been noted
in only 5% of adult colorectal adenocarcinomas.17 Inci-
dence of mutinous carcinoma in children has been
reported as more than 50% of colonic carcinomas.l* The
mucin absorbs water, swells and invades tissues, thereby
promoting spread of malignant cells. This tumor may
grow to huge sizes because of the pooling mucin. It also
interferes with the immune recognition of carcinoma
cells caused by mucopolysaccharide coating. The signet
ring subtype has the worst prognosis. It grows so rapidly
that regional lymph node involvement and diffuse perito-
neal seeding is a common finding at the time of presenta-
tion, which leads to poor prognosis.2 In our series, the
most frequent histological type has been mutinous adeno-
carcinoma (80%), which is a higher figure compared with
the previous reports. Mutinous type of colorectal carci-
noma seems to be the major determinant of poor outcome
when compared with early presentation.
During childhood, most patients (60% to 80%) with
colorectal carcinoma have stage C or D disease at
presentation.1,8s13We have encountered advanced stages
of disease at presentation in all patients (65% stage D,
35% stage C). Vague symptoms, decreased awareness,
and increased frequency of mutinous type results in
advanced stages at presentation.
Results of many series confirm that surgery is the most
effective treatment for colorectal carcinoma.2,13s17Al-
though this is true for 90% to 95% of adult cases, the rate
of complete resection has been less than optimal in
children. In our series, this was possible in only 6 patients
COLORECTAL CARCINOMA 1503

(30%). Studies comparing the results of conservative and value of benefit in most of the cases3sUas seen in our
aggressive surgery showed not only. an increase in series, they are still used as adjuvant therapy modes. It
resectability rate (72% to 92%), but also increased has been reported that adjuvant chemotherapy extends
survival rates by aggressive surgical procedures2J9J0 the life of some patients during childhood.2 Although
Radical resection of tumoral mass including lymphat- very rare, some patients with metastatic disease had been
its associated with primary anastomosis should be the reported to be cured with chemotherapy.24 Therefore, we
procedure of choice. If the patient is operated on for acute believe that adjuvant therapy should be tried in children
intestinal obstruction without bowel preparation, tempo- with colorectal carcinoma.
rary ileostomy or colostomy after resection may be added The survival pattern of our patients is similar to those
to the procedure. Palliative procedures should be done in of the previous series in which only 2 of 38 patients and 3
the presence of peritoneal spread or distant metastasis or of 200 patients survived 5 years or more after curative
to prevent bleeding and obstruction. As our approach to surgery.15J7 Delayed diagnosis, advanced stages of dis-
solid tumors had been more aggressive, we performed ease at presentation, and, most importantly, mutinous
wider excisions like Miles operation even in patients of type of histology are the major determinants in poor
stage D in early years of our study period. After 1985, we outcome.
preferred more conservative surgical procedures such as
Our experience and literature findings emphasize that
only performing biopsy on the tumor, palliative colosto-
possibility of a malignant colorectal tumor should be
mies, or segmental resection in stage D patients. How-
considered for any childhood case with signs and symp-
ever, we have not noticed increased survival rates by any
of the surgical procedures. toms of intestinal obstruction, intractable abdominal
Second-look surgery was advocated by some investiga- pain, alteration in bowel habits, and gastrointestinal
tors.21.22The major problem of second-look surgery was bleeding. Colorectal malignancy should not be excluded
optimal timing and operative morbidity. This concept was only on the basis of the patients age. Because of the
modified by advocating chemotherapy before second- steadily increasing incidence of precancerous bowel
look procedures. A small number of patients benefited diseases and poor prognosis of colorectal carcinoma,
from these operations and survived 5 years or more.23 No childhood cases of bowel disorders should receive the
planned second-look operations had been done in our same detailed and vigorous diagnostic evaluation and
series. However, second-look operations had been per- appropriate treatment as given to adult cases. Contrast
formed in 2 cases for relief of intestinal obstruction and studies, ultrasonography, computed tomography, and en-
reexploration for late onset of disease. doscopy are essential procedures for both confirming the
Although chemotherapy and radiotherapy has little diagnosis and detecting the extent of the disease.
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