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AYA Monograph Colon and Rectal Cancer

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The web site itself may have changed. You can check the current page or check for previous versions at the Internet Archive.Yahoo! is not affiliated with the authors of this page or responsible for its content.AYA Monograph Colon and Rectal CancerSheri Spunt, MDWayne Furman, MDMichael La Quaglia, MDMelissa Bondy, PhDRichard Goldberg, MDChapter 10Colon and Rectal CancerCancer in 15- to 29-Year-Olds in the United StatesCHAPTER 10COLON & RECTAL CANCERSEER AYA Monograph124National Cancer InstituteHIGHLIGHTSIncidence Colorectal carcinoma occcurs in adolescents and young adults at an incidence that increases exponentially between10 and 35 years of age. During 1975 to 2000, colorectal cancer accounted for 2.1% of all neoplasms diagnosed in 15- to 29-year-olds. In the year 2000, an estimated 432 individuals 15 to 29 years of age were diagnosed with cancer of the colon. The incidence of colorectal carcinoma relative to other cancers rose from 1% in the 15- to 19-year age group, to 1.7%in the 20- to 24-year age group, to 2.7% in the 25- to 29-year age group. Males had a higher incidence of colorectal carcinoma than females at all ages, except in individuals 15 to 19 years of age. Although the incidence of colorectal carcinoma in individuals over 45 years declined during the period 1975 to 2000,the incidence in 15- to 29-year-olds increased. The incidence of colorectal carcinoma was approximately equal in white non-Hispanics, Hispanics, AfricanAmericans/blacks, and Asians/Paci c Islanders who were 15 to 29 years of age when diagnosed. American Indians/Alaska Natives between 20 and 35 years of age tended to have a lower incidence of colorectalcarcinoma than other racial/ethnic groups.Mortality & Survival Five-year survival for individuals 15 to 29 years of age was similar to that of older individuals. Colorectal carcinoma survival improved over time, although it remained relatively stable for individuals in the 15- to29-year age group. A 54% 5-year survival rate for colorectal carcinoma for the age group was achieved in the era 1975 to 1980; the5-year survival rate increased to 58% in the era 1993 to 1999. Females had higher 5-year survival rates than males at virtually all ages; the disparity was particularly marked inindividuals 15 to 29 years of age. African Americans/blacks in the 15- to 29-year age group had the worst survival, approximately 20% worse thanwhites, non-Hispanics, and Asians/Paci c Islanders. Whites tended to have the best prognosis.Risk Factors Predisposing factors for colorectal carcinoma in children and young adults include hereditary conditions (polyposisand non-polyposis syndromes), in ammatory bowel disease, and prior radiation exposure. Hamartomatous polyposis syndromes carry a lower risk of colorectal carcinoma than adenomatous polyposissyndromes.INTRODUCTIONAlthough colorectal carcinoma is among the mostcommon malignancies of adulthood, the disease is
uncommon in adolescents and young adults. Between1975 and 2000 in the U.S., colorectal carcinomaaccounted for 2.1% of all neoplasms in adolescents andyoung adults 15 to 29 years of age. In the year 2000,
432 individuals in this age group were diagnosed withcolorectal carcinoma in the U.S.METHODS, CLASSIFICATION SYSTEM, AND
BIOLOGICAL IMPLICATIONSThe International Classi cation of Childhood Cancer(ICCC) has no speci c category for colorectal cancer.These cancers are contained with category XI(f), Otherand Unspeci ed Carcinomas, as one of the Carcinomas
and Other Epithelial Neoplasms (category XI). Hence,
the SEER site recode based on the International
Classi cation of Diseases for Oncology (ICD-O) was
used exclusively for this chapter.National Cancer Institute125SEER AYA MonographCHAPTER 10COLON & RECTAL CANCERFor colorectal cancer, the ICD-O Topographicalcategories are C18.0-C20.9, C26.0 (colon, rectum, andintestine NOS) and the ICD-O Morphologic categories
include general carcinomas and adenocarcinomas
(8010-8041, 8140, many others) and speci c cancers of
the colon/rectum. The latter include carcinoid tumors
(8240-8245). No attempt was made to separate cancer of
the colon from rectal cancer. Cancer of the anus is not
included in this chapter.As explained in the Methods chapter, data are presented for
15- to 29-year-olds with comparisons to the age groups 0 to 15years and 30 to 44+ years, as appropriate. For some analysesthe entire age range from birth to 85+ years is included. The
absence of data in any gure or table within this chapter
means that too few cases were available for analysis; it does
not mean that the rate or change in rate was zero.Since the ICCC was set up as a classification forchildhood cancer, it does not have a separate category for
colorectal cancer. Topography and histology from ICD-O
can be used to examine differences among very young
colorectal cancer patients compared to older patients, butit is clear that this method needs to be complementedwith other biologic determinants such as microsatelliteinstability, which is far more common in the colorectalcarcinomas that occur in adolescents and young adults,
as opposed to the sequential gene mutationsincluding
p53 mutationsthat occur in older adults.INCIDENCEAccording to SEER data, colorectal carcinoma accountedfor 2.1% of all malignancies diagnosed in individuals 15to 29 years of age between 1975 and 2000, and occurred
at a rate of 7.21 per million, age adjusted to the 2000census and to 5-year age intervals. It was the 11th mostcommon cancer in this age bracket. By using the data inTrends in Incidence (section below), a total of 432 newcases of colorectal cancer were estimated to have been
diagnosed in the U.S. in the year 2000 (Table 10.1).Age-Speci c IncidenceThe striking dependence of the incidence of colorectalcarcinoma on age is shown in Figure 10.1. During 1975
to 2000, it increased exponentially between ages 10 and35, as highlighted by the red line in the semilog plot inthe inset to gure 10.1.Figure 10.2 shows the incidence of colorectal carcinomarelative to that of all cancers in 5-year age groups from
0 to 44 years. The incidence of colorectal carcinoma
relative to all cancers increased steadily with advancing
ageagain, even within the 15- to 29-year age group.Gender-Speci c IncidenceThe incidence of colorectal carcinoma in males andfemales was similar before age 20, and higher in malesthan females older than 20 years. The predilection for male
gender increases with age, such that by 50 years of age,
males had a nearly 50% greater incidence (Figure 10.3).Racial/Ethnic Differences in IncidenceThe incidence of colorectal carcinoma was approximatelyequal in white non-Hispanics, Hispanics, AfricanAmericans/blacks, and Asians/Paci c Islanders who were
15 to 29 years of age when diagnosed (Figures 10.4 and
10.5 [the log version of 10.4]). American Indians/AlaskaNatives between 20 and 35 years of age tended to havea lower incidence of colorectal carcinoma than other
racial/ethnic groups. African Americans/blacks had alower incidence from 0 to 20 years of age (Figure 10.5).Table 10.1: Incidence of Colorectal Carcinoma in Persons Younger Than 30 Years of Age, U.S., 1975-2000AGEATDIAGNOSIS (YEARS)<55-910-1415-1920-2425-29U.S. population, year 2000 census (in millions)19.17620.55020.52820.22018.96419.381Average incidence per million, 1975-2000, SEER^^0.62.05.614.6Average annual % change in incidence, 1975-2000, SEER^^0000Estimated incidence per million, year 2000, U.S.^^0.62.05.614.6Estimated number of persons diagnosed, year 2000, U.S.^^1341107284^ Too few for a reliable estimateCHAPTER 10COLON & RECTAL CANCERSEER AYA Monograph126National Cancer InstituteTrends in IncidenceFigure 10.6 displays the incidence of colorectal carci-noma in 5-year age groups over 4 time periods: 1975
to 1980, 1981 to 1986, 1987 to 1992, and 1993 to 2000.For individuals over 45 years of age, the incidence inthe most recent time period (1993 to 2000) was lower
than during earlier years.As shown in Figure 10.7, a decline in colorectal carci-noma incidence in younger individuals was not readily
apparent. The rarity of colorectal carcinoma in patients
under 45 years of age may explain the dif culty in iden-
tifying a trend in incidence in this age group.Figure 10.8 displays the average annual percent change
in incidence of colorectal carcinoma by age group dur-
ing the years 1975 to 2000. The incidence of colorectalcarcinoma in patients in the 15- to 29-year age groupincreased during this period, whereas the incidence in
patients over 30 years of age declined.OUTCOMEMortalityAs with incidence (Figure 10.7), mortality of colorectalcancer was directly proportional to age and was higherin males than females (Figure 10.9). In the U.S., AfricanAmericans/blacks had higher mortality from colorectalcarcinoma than any other racial/ethnic group. This was
true not only for older adults but also for young adults25 years of age and older (Figure 10.10).Figure 10.1: Incidence of Colorectal Carcinoma, SEER 1975-2000Figure 10.2: Incidence of Colorectal Cancer Relative to All
Cancer, SEER, 1975-2000Figure 10.3: Incidence of Colorectal Carcinoma by Gender,
SEER 1975-2000Figure 10.4: Incidence of Colorectal Carcinoma by Race/
Ethnicity, SEER 1992-2000National Cancer Institute127SEER AYA MonographCHAPTER 10COLON & RECTAL CANCERSince 1975, colorectal carcinoma mortality has declinedamong 15- to 29-year-olds (Figure 10.11), despite the in-
creased incidence in this age group (Figure 10.8). The re-
duction in national colorectal cancer mortality during the
past quarter century averaged 1.4% per year in patientsyounger than age 45 (p < 0.05), and the decline was alsostatistically signi cant in each 5-year age group under
age 45 (Figure 10.12). There is a suggestion that the trendin national colorectal cancer mortality reduction is di-
rectly proportional to age, with patients younger than 35years of age experiencing less of a reduction in mortalitythan those 35 to 44 years of age (Figure 10.12).SurvivalFive-year survival of individuals with colorectal carci-noma 15 years of age and older was between 50% and61% for all ve-year age groups (Figure 10.13). Five-yearsurvival rates improved in successive eras, though ratesfor individuals 15 to 29 years of age remain ed relativelystable (Figure 10.14).Gender-Speci c SurvivalFemales had a consistently higher 5-year survival ratecompared to males; this was most apparent in patientsyounger than 40 years of age (Figures 10.15 and 10.16)Racial/Ethnic Differences in SurvivalSurvivalas a function of race/ethnicityfor 15- to29-year-olds diagnosed with colorectal cancer dur-ing the period 1992 to 1999 is shown in Figure 10.17.African Americans/blacks had the worst survival inthis age group. White non-Hispanics, Hispanics, andFigure 10.7: Change in Incidence of Colorectal Carcinoma by
Era, SEER 1975-2000Figure 10.8: Average Annual Percent Change (A APC) in
Incidence of Colorectal Carcinoma, SEER 1975-2000Figure 10.5: Incidence of Colorectal Carcinoma by Race/
Ethnicity, SEER 1992-2000Figure 10.6: Change in Incidence of Colorectal Carcinoma by
Era, SEER 1975-2000CHAPTER 10COLON & RECTAL CANCERSEER AYA Monograph128National Cancer InstituteAsians/Pac i c Islanders had comparable survival, whichwas from 15% to 20% better than for African Americans/blacks as early as 1 year after diagnosis and persistingfor at least ve years. Whites (including Hispanic whites)tended to have the best prognosis.Survival According Extent of DiseaseFigure 10.18 displays 5-year survival rates for individuals
with colorectal carcinoma according to disease extent.There were no apparent differences in survival in indivi-duals 15 to 29 years of age compared to older individuals.RISK FACTORSPredisposing factors for colorectal carcinoma in childrenand young adults include hereditary conditions affecting
the bowel (polyposis and nonpolyposis syndromes), in-
ammatory bowel disease, and radiation exposure. Ap-
proximately 15 to 20% of colorectal cancer patients havefamilial colon cancer without a de ned genetic pattern,1about 5% have hereditary nonpolyposis colon cancer,2and 1% have hereditary polyposis syndromes.3Hereditary Nonpolyposis Colon Cancer (HNPCC)HNPCC was de ned by Lynch,4 who observed a numberof families with an increased risk of colon cancer in the
absence of polyposis. HNPCC accounts for approximate -
ly 5% of all colorectal cancer cases and is associated with
an early age at diagnosis, proximal colonic site predomi-
nance, mucinous phenotype and multiple synchronous
and metachronous tumors.5,6 Families with HNPCCalso have a higher incidence of other tumors, including
stomach, small intestine, hepatobiliary system, ovary,Figure 10.9: National Mortality of Colorectal Carcinoma by
Gender, U.S., 1975-2000Figure 10.10: National Mortality of Colorectal Carcinoma by
Race/Ethnicity, U.S., 1975-2000Figure 10.11: National Mortality of Colorectal Carcinoma by Era,
U.SFigure 10.12: Average Annual Percent Change (AAPC) in
National Mortality, Colorectal Carcinoma, 1975-2000National Cancer Institute129SEER AYA MonographCHAPTER 10COLON & RECTAL CANCERendometrium, and upper urinary tract cancers.7 HNPCCis associated with a lower stage at diagnosis, a lower
incidence of metastases, and a better prognosis than spor-adic colorectal carcinoma.8Germline mutations of DNA mismatch repair genes are
responsible for the predisposition to colorectal carcinomaamong patients with HNPCC. Absence of normal mis-
match repair function in colonic epithelial cells leads to
microsatellite instability and malignant transformation.9The Amsterdam criteria for de ning HNPCC10 includecolorectal cancer in at least three individuals spanning
two generations, at least one of whom is a rst-degree rela-
tive of the other two. In a small series of colorectal cancer
patients who were 21 years of age or less at diagnosis,
microsatellite instability was observed in about half,
though few ful lled the diagnostic criteria for HNPCC.11Polypoid Disease of the Gastrointestinal TractColonic polyps can be divided by histology into adenoma-
tous and hamartomatous categories. Adenomatous polyps
represent a growth alteration in the colonic mucosa result-
ing in neoplastic proliferation and substantial malignant
potential. Hamartomatous polyps, though less proliferative
in nature, are also associated with a signi cant cancer risk.Hamartomatous Polyposis SyndromesOnly two hamartomatous polyposis syndromes have beenclearly associated with an increased risk of colorectal
carcino ma. Juvenile polyposis, which encompassesjuven ile polyposis coli and diffuse juvenile polyposis,Figure 10.15: 5-Year Survival Rate for Colorectal
Carcinoma by Gender, SEER 1975-19992040600Age at Diagnosis (Years)15-1920-2425-2930-3435-3940-4445-4950-5455-5960-6465-6970-7475-795-YearSurvival(%)80-84FemaleMaleFigure 10.13: 5-Year Survival Rate for Colorectal Carcinoma,
SEER 1975-1999Figure 10.14: 5-Year Survival Rate for Colorectal Carcinoma
by Era, SEERFigure 10.15: 5-Year Survival Rate for Colorectal Carcinoma by
Gender, SEER 1975-1999Figure 10.16: 5-Year Survival Rate for Colorectal Carcinoma by
Gender, SEER 1975-1999CHAPTER 10COLON & RECTAL CANCERSEER AYA Monograph130National Cancer InstitutePeutz-Jeghers syndrome,17 characterized by variablemucocutaneous pigmentation abnormalities and gastro-
intestinal hamartomas, is also associated with an
increased risk of colorectal malignancy. These patients
typ ically present during childhood with recurrent intussus-
ception/bowel obstruction, rectal bleeding, anemia, and
rectal prolapse, in some cases before the pigmentary
changes classically associated with the disorder are pre-
sent.18 Males become symptomatic at an earlier age (peak5-10 years) than females (peak 10-15 years). Hamartoma-t ous polyps of the small intestine are most common;
however, about one-third of patients also have colorectal
involvement. Most cases of Peutz-Jeghers syndrome are
due to germline mutation of the STK11 gene, which
encodes a serine threonine kinase.19 The transformationfrom hamartoma to adenocarcinoma in patients with
germ line STK11 mutations depends on additional somaticmutations.20 In an analysis of 33 patients with Peutz-Jeghers syndrome, the standardized mortality ratio forgastrointestinal cancer was 24.8.21Adenomatous Polyposis SyndromesThe familial adenomatous polyposis (FAP) syndromesare characterized by the early development of multiple
adenomatous colonic polyps. FAP affects about 1 in7,000 individuals.9 Virtually all patients with FAP willdevelop colorectal carcinoma unless a total colectomyis performed prior to the onset of malignancy. FAP isan autosomal dominant trait with high but variable
penetrance; 10 to 20% of cases are de novo muta-
tions without any apparent family history. Mutationsin the APC gene at 5q21 are responsible for the FAPsyndrome.22 The clinical phenotype, including the pres-ence or absence of extracolonic abnormalities, appears tovary according to the exact site of APC gene mutation andthe presence of modifying genes.23,24 Two types of FAPseem to exist, and a relationship between the location
of mutations in the gene and the phenotypic expression
of FAP has been established:25 the sparse type, whichis characterized by hundreds of polyps, and the profuse
type, which presents with thousands of polyps. Patientswith the profuse type tend to develop adenocarcinoma atan earlier age. FAP is associated with the development of
extracolonic malignancies, including periampullary and
thyroid carcinomas and hepatoblastoma.26,27typically presents with rectal bleeding and anemia in
patients between 4 and 30 years of age.12,13 Other symptomsinclude intussusception or bowel obstruction, rectal or polyp
prolapse, abdominal pain, and protein-losing enteropathy.The polyps may occur throughout the gastrointestinaltract, but most often affect the stomach, distal colon, and
rectum. Juvenile polyposis is transmitted as an autosomal
dominant trait; SMAD4 and BMPR1A gene mutations
have been implicated in the etiology of this syndrome.14 Ina review of cases reported in the English literature, Coburn
et al. found that 17% of patients developed gastrointestinal
malignancies, at a mean age of 35.5 years (range, 4-60years).15 The cumulative risk of colorectal malignancy hasbeen reported to be 68% by 60 years of age.16Figure 10.17: Relative Survival for Colorectal Carcinoma by
Race/Ethnicity, SEER 1992-1999Figure 10.18: 5-Year Survival Rate for Colorectal Cancer by
Extent of Disease, SEER 1975-2000National Cancer Institute131SEER AYA MonographCHAPTER 10COLON & RECTAL CANCERA proportion of FAP patients have Gardners syndrome,28which includes desmoid tumors, cysts of the mandible, bromas, osteomas, and congenital hypertrophy of the
retinal pigment epithelium. Desmoid tumors of the
abdominal wall and mesentery occur in a signi cant
proportion of these patients, and are a leading cause of
death in post-colectomy patients.Other syndromes associated with FAP include Turcots
syndrome29 and Old elds syndrome.30 Patients withTurcots syndrome manifest multiple pediatric brain tumors(medulloblastoma, gliomas, and others) in conjunction
with FAP. Hamilton et al. found that two distinct germ-
line defects, mutation of the APC gene and mutation of amismatch-repair gene may each give rise to Turcots syn-
drome.31 The type of brain tumor correlates with the muta-tion, with medulloblastomas characteristic of APC-related
mutations and glioblastoma multiforme seen in patientswith mismatch-repair gene mutations. Old elds syndromeincludes FAP in association with multiple sebaceous cysts.In ammatory Bowel DiseaseUlcerative colitis is clearly associated with the devel-opment of colorectal carcinoma.32 The age at initialpresen tation and extent of colonic involvement are strong
independent risk factors for subsequent development of
colorectal cancer.33 Patients less than 15 years of ageat diagnosis and those with involvement of the entire
colon are at the highest risk. The cumulative risk of colo-
rectal carcinoma in individ uals less than 40 years of
age with pancolitis was 13% at 25 years from diagno-
sis. Synchronous colorectal tumors are more common in
patients with ulcerative colitis than in the remainder of
the population with colorectal carcinoma.34 In a reportby Lashner et al.,35 11 of 15 ulcerative colitis patientswith strictures were found to have carcinomas on biop-sy. Thus, patients who develop colonic strictures should
be considered to have carcinomas until proven other-wise, and stricture formation is an indication for surgery.Crohns disease, when it involves the colon or rectum, isassociated with an increased risk of colorectal carcinoma.The relative risk is quite high (20.9 odds ratio) in those inwhom the diagnosis is made before the age of 30 years.36About one-third of the colorectal carcinomas in patientswith Crohns disease are mucinous adenocarcinoma.37Other Factors Predisposing to Colorectal CarcinomaColorectal carcinoma has been reported in patients with
Bloom syndrome, a rare autosomal recessive disordercaused by germline mutations of the BLM gene.38 Gruberet al. showed recently that carriers of BLM mutations are
also at increased risk of colorectal carcinoma.39 About5% of patients undergoing urinary diversion with ureter-osigmoidostomy develop colon cancer, probably due to
chronic in ammation caused by the mixture of feces and
urine at the implant site.40Colorectal Carcinoma in Childhood Cancer SurvivorsChildren who receive abdominal or pelvic radiation
therapy for the treatment of a malignancy are at increased
risk for early development of colorectal cancer in the ra-diation eld.41,42 This may be particularly problematic inany child who has one of the above genetic predisposi-
tions. Overall, however, colorectal cancer is one of theleast common second cancers in long-term survivors ofchildhood cancer.43-46SUMMARYColorectal carcinoma accounted for 2.1% of all malig-
nancies in individuals between 15 and 29 years of age,and was the 11th leading cause of cancer in this age group.The average annual incidence of colorectal carcinomawas 7.2 per million in 15- to 29-year-old individuals. Theincidence of colorectal carcinoma rose with advancingage, even within the 15- to 29-year age group. Colo rectal
carcinoma accounted for an increasing proportion of all
malignancies with advancing age. While it accounted for
only 1% of malignancies in the 15- to 19-year age group,it was responsible for 2.7% of cancers in the 25- to 29-year age group. Colorectal carcinoma was more commonin males at all ages over 20 years of age. Although the
incidence of colorectal carcinoma has declined over time
in individuals over 45 years of age, a similar decline has
not been observed in individuals in the 15- to 29-yearage group. The small number of patients diagnosed with
colorectal carcinoma in this age group makes it dif cult
to draw conclusions about incidence and outcome data.The average percent annual change in the incidence ofcolorectal carcinoma increased between 1975 and 2000
among individuals 15 to 29 years of age, and declined inindividuals over 30 years of age.CHAPTER 10COLON & RECTAL CANCERSEER AYA Monograph132National Cancer InstituteFive-year survival rates for individuals with colorectalcarcinoma in the 15- to 29-year age group were similar to
those for older individuals. The survival improvements
over time observed in individuals over 45 years of age
have also been noted in individuals 15 to 29 years of
age. Females with colorectal carcinoma had a superior 5-year survival compared to males at virtually all ages; thedispar ity was particularly marked in individuals younger
than 40 years of age.Risk factors for the development of colorectal carcinoma
in childhood and young adulthood include hereditaryconditions (polyposis and non-polyposis syndromes), in-
ammatory bowel disease, and radiation exposure. Hamar-
tomatous polyposis syndromes carry a lower risk of colo-
rectal carcinoma than adenomatous polyposis syndromes.Colorectal carcinoma has also been reported in individualswith Bloom syndrome and in those who have undergoneurinary diversion via ureterosigmoidostomy.REFERENCES 1. Boutron M-C, Faivre J, Quipourt V, et al.: Family history of colorectal tumours and implications for the adenoma-carcinoma sequence: a case control study. Gut 1995;37:830-4. 2. Marra G, Boland CR: Hereditary nonpolyposis colorectal cancer: the syndrome, the genes, and historical perspectives.J Natl Cancer Inst 1995;87:1114-25. 3. Vogelstein B: Genetic testings for cancer: the surgeons critical role. Familial colon cancer. J Am Coll Surg 1999;188:74-9. 4. Lynch HT, de la Chapelle A: Genetic susceptibility to non-polyposis colorectal cancer. J Med Genet 1999;36:801-18. 5. Lynch HT, Smyrk T, Lynch J: An update of HNPCC (Lynch syndrome). Cancer Genet Cytogenet 1997;93:84-99. 6. 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