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Review of colorectal cancer in patients under age 40 years
Review of Colorectal Cancer in Patients Under Age 40 Years
Kathryn L. Safford, MD, Fort Sam Houston, Texas Michael J. Spebar, MD, Fort Sam Houston, Texas Daniel Rosenthal, MD, FACS, Fort Sam Houston, Texas
The incidence of neoplastic disease has been documented to increase with age. Colorectal cancers are no exception, with a mean age at occurrence of 65 years [l]. However, no age group is exempt, and adenocarcinoma of the colon has been reported in a 9 month old infant [2]. The prognosis for patients with colorectal cancer has generally been poor, but younger patients have reportedly had a worse prognosis [3-101. Several reviews have suggested multiple explanations, including a low index of suspicion, delay in diagnosis or uniquely virulent tumor cells [3-141. Several studies have been undertaken in an attempt to identify any distinctive characteristic of colorectal cancer in young patients. For this reason, a review of the experience with colorectal cancer in patients under age 40 years was undertaken at Brooke Army Medical Center. Material
and Methods
Records of the Tumor Registry for the 33 year period between 1947 and 1980 were reviewed. These charts were reviewed for.demographic data (age, sex and race) in addition to information on history, symptoms, tumor location, pathologic characteristics, surgical procedure, and additional chemotherapy or radiation therapy. Patients with carcinoid of the rectum or appendix were excluded. Results One hundred forty of 819 documented cases of colorectal malignancy occurred in patients age 40 years or under. Of these, 21 cases were diagnosed as From the General Surgery Service, Department of Surgery, Brooke Army Medical Center, Fort Sam Houston, Texas. The opinions or assertions contained herein are the private views of the authors and are not to be construed as official of as reflecting the views of tha Department of tha Army or the Department of Defense. Requests for reprints should be addressed to Kathryn L. Safford, MD, General Surgery Service, Damall Army Hospital, Fort Hood, Texas 76541. Presented at the 33rd Annual Meeting of the Southwestern Surgical Congress, Monterey, California, May 4-7, 1981.
Voiume 142, December 1991
carcinoid (16 appendiceal and 5 rectal). Twenty of these patients were excluded while 1 patient with carcinoid of the appendix and rectal carcinoma was included. The 120 cases reviewed represent an incidence of 14.6 percent. There were 86 male (72 percent) and 34 female patients (28 percent). Ninety-two patients were Caucasian, 16 were black, 11 were LatinAmerican and 1 was Oriental. The youngest patient in the series was 8 years old (lymphosarcoma of the cecum). A family history of colorectal cancer was found in six patients, while seven patients had ulcerative colitis and one had familial polyposis. Multiple symptoms were present in 70 percent of the patients, and 30 percent presented with a single symptom. The duration of symptoms was less than 1 month in 21 percent,~ 1 to 6 months in 41 percent, 6 to 12 months in 16 percent and greater than 1 year in 22 percent. The median duration of symptoms was 3 months. Comparison of the symptoms and duration is summarized in Table I. The pathologic diagnosis was adenocarcinoma in 118 patients, lymphosarcoma of the cecum in 1 and squamous cell carcinoma in 1. Following the Dukes’ classification system, 94 charts contained sufficient data to assign a Dukes’ classification: no class A; 19 class B, 33 class C and 42 class D. The anatomic locations of the tumors are illustrated in Figure 1. Chemotherapy was given to 29 patients and 11 received radiation therapy. Follow-up has been complete to this date on 98 percent of the patients (117). Two of the three patients lost to follow-up (at 14 and 24 years, respectively) were included in the 5 and 10 year survival rates. Overall, the 5 and 10 year survival rates were 34.6 and 32.2 percent, respectively. Patients with Dukes’ B lesions had 5 and 10 year survival rates of 66.6 percent. Patients with Dukes’ C lesions had 5
767
Safford et al
TABLE I
Symptoms and Duration
Symptoms Bleeding Pain Change in bowel habits Obstruction Weight loss Anemia Mass Fever
mo
Duration 1-6 mo 6-12 mo
>l
year
5 14 6
19
9
16 22
10 9
21 10 9
13 3 2 3 1
11 7 5 1
2 3 2 0 0
2 6 0 0 0
a
and 10 year survival rates of 36.6 and 30.0 percent, respectively. There were no 5 year survivors in the Dukes’ D group; those patients had a mean survival time of 10 months. The 5 year survival rate for the patients receiving chemotherapy and radiation therapy were 13.6 and 10 percent, respectively. Comments Colorectal carcinoma occurs infrequently in younger patients but is not uncommon. Our incidence of 14.6 percent was somewhat higher than in most previous reviews, but compatible with two similar reviews of military age populations [II]. The predominant presenting symptoms in the general population are abdominal pain, change in bowel habits, weight loss and bloody stool [1,15]. These are also the predominant symptoms in younger patients [2-7,11,12,16]. The duration of symptoms has been considered a measurement of delay in diagnosis and therefore indicative of ultimate prognosis [9,11]. Several investigators have suggested that if the duration of symptoms was reduced, prognosis might improve [17]. Support for this statement was offered by several studies of young patients that documented the average duration of symptoms as 5.5 to 8 months, [7-9,161, despite the fact that in the general population most patients present with a symptom duration of 6 months or less [l]. The median duration of symptoms in this series was 3 months, and did not correlate with Dukes’ classification or survival. A review of colon and rectal cancer by Welch at the Massachusetts General Hospital in 1974 documents a reduction in the duration of symptoms from 7 to 3 months but no significant change in the survival data [15]. A decline in survival rates with advancing Dukes’ classification parallels the trend seen in the general population [1,12,15]. This series agrees with previous reviews [11,16,18,19], which note a higher proportion of Dukes’ C and D lesions in younger patients. It has been suggested that this provides evidence of a more virulent tumor in younger patients [5]. However, this is not confirmed by analysis of the survival rates for each Dukes’ classification group. These individual
766
Figure 1, Anatomic distribution of tumors.
survival rates parallel those in the general population and do not support the likelihood of unique tumor behavior. In this series, as in the general population, the most accurate prognostic indicator of survival is the Dukes’ classification at the time of diagnosis. Tumor behavior as reflected by the duration of symptoms and survival rates appears to function independently of patient age. The results of this review do not offer evidence to support either a delay in diagnosis or unique tumor behavior in younger patients. Summary Brooke Army Medical Center Tumor Registry records from 1947 through 1980 were reviewed. One hundred forty of 819 patients with colorectal cancer were aged 40 or less. The 5 year survival rate was 35 percent and the 10 year survival rate 32 percent. The predominant presenting symptoms were bleeding, pain and change of bowel habits. The median duration of symptoms was 3 months. There were no Dukes’ A patients. The 5 and 10 year survival rates in 19 Dukes’ B patients were 67 percent. Thirty-three Dukes’ C patients had 5 and 10 year survival rates of 37 and 30 percent, respectively. In 42 Dukes’ D patients, however, there were no 5 year survivors, and the mean length of survival was only 10 months. Our findings support previous surveys which have concluded that stage at the time of diagnosis, rather than symptoms, duration or patient age, is the most accurate prognostic factor. References 1.
Falterman KW, Hill CB, Markey JC, Fox JW, Cohn I. Cancer Of the colon, rectum and anus: a review of 2,313 cases. Cancer 1974;34:951-9. 2. Sessions RT, Riddell DH. Kaplan HJ, Foster JH. Carcinoma of the colon in the first two decades of life. Ann Surg 1965; 162:279-84.
The American Journal of Surgery
Review of Colorectal Cancer
3. Ezzo JA. Sullivan JF, Mack RE. Carcinoma of the colon under the age of 40. Ann Intern Med 1958;49:321-5. 4. Recalde M, Holyoke EP, Elias EG. Carcinoma of the colon, rectum and anal canal in young patients. Surg Gynecol Obstet 1974;139:909-13. 5. Ferguson E. Obi LJ. Carcinoma of the colon and rectum in patients up to 25 years of age. Am Surg 1971;37:181-9. 6. Hall A, Coffey RJ. Cancer of the large bowel in the young adult. J Surg 1961;102:66-72. 7. Rosato FE, Frazier TG, Copeland EM, Miller LD. Carcinoma of the colon in young people. Surg Gynecol Obstet 1969; 129:29-32. 8. Coffey RJ, Cardenas F. Cancer of the bowel in the young adult. Dis Colon Rectum 1964;7:491-2. 9. Mayo CW, Pagtalunan RJG. Malignancy of the colon and rectum in patients under 30 years of age. Surgery 1963;53:71 l8. 10. Chabalko JJ, Fraumeni JF. Colorectal cancer in children: epidemiologic aspects. Dis Colon Rectum 1975;18:1-3. 11. Simstein NL, Kovalcik PJ, Cross GH. Colorectal carcinoma in patients less than forty years old. Dis Colon Rectum 1978; 21:169-71. 12. Howard EW, Cavallo C, Hovey LM. Nelson TG. Colon and rectal cancer in the young adult. Am Surg 1975;41:260-5. 13. Sanfelippo PM, Beahrs OH. Carcinoma of the colon in patients under forty years of age. Surg Gynecol Obstet 1974;138: 169-70. 14. Miller FE, Liechty RD. Adenocarcinoma of the colon and rectum in persons under 30 years of age. Am J Surg 1967;113: 507-10. 15. Welch JP, Donaldson GA. Recent experience in the management of the colon and rectum. Am J Surg 1974;127:25866. 16. Scarpa FJ, Hartmann WH, Sawyers JL. Adenocarcinoma of the colon and rectum in young adults. South Med J 1976;69: 24-7. 17. Gallagher EG, Zeigler MG. Rectal carcinoma in patients in the second and third decades of life. Am J Surg 1972;124: 655-9. 18. Roth-Mayo LA, Devitt JE, Brown FN. The effect of age on the
Volume 142, December 1981
behavior of carcinoma of the colon. Dis Colon Rectum 1968; 11:420-2. 19. Johnson JW, Judd ES, Dahlin DC. Malignant neoplasms of the colon and rectum in young persons. Arch Surg 1959;79: 21-8.
Discussion Anatolio B. Cruz, Jr. (San Antonio, TX): The symptoms in this series, as in others (bleeding, pain, change of bowel habits, obstruction, weight loss, anemia, mass) are not early symptoms. In the 94 patients who were staged, none were Dukes’ A, which is an indicting fact. Only 1 of 32 Dukes’ B, 14 of 32 Dukes’ C and 17 of 32 Dukes’ D patients were aged 30 years or less. We are not discovering the early lesions early enough. There are no data to suggest that they have more biologically aggressive tumors. Perhaps the development and increased use of biologic markers for screening will bring lesions to diagnosis and treatment earlier. William A. Albano (Omaha, NE): At Creighton University we have been interested in the hereditary cancer problem. In approximately 5 percent of all colon cancers seen at our institution, an autosomal dominant inheritance has been demonstrated. These may be classified as hereditary, nonpolyposis colon cancer. Such syndromes are characterized by a predominance of right-sided lesions and an increase in multiple primaries. A study just completed at Creighton Cancer Center involved survival assessment in 118 affected persons from 20 families manifesting hereditary colon cancer syndromes. Our study indicated that patients with hereditary colon cancer had a significantly better survival rate, stage for stage, than patients with sporadic colon cancer. The overall survival rate in the hereditary group at 5 years was approximately 67 percent.
769
Kathryn L. Safford, MD, Fort Sam Houston, Texas Michael J. Spebar, MD, Fort Sam Houston, Texas Daniel Rosenthal, MD, FACS, Fort Sam Houston, Texas
The incidence of neoplastic disease has been documented to increase with age. Colorectal cancers are no exception, with a mean age at occurrence of 65 years [l]. However, no age group is exempt, and adenocarcinoma of the colon has been reported in a 9 month old infant [2]. The prognosis for patients with colorectal cancer has generally been poor, but younger patients have reportedly had a worse prognosis [3-101. Several reviews have suggested multiple explanations, including a low index of suspicion, delay in diagnosis or uniquely virulent tumor cells [3-141. Several studies have been undertaken in an attempt to identify any distinctive characteristic of colorectal cancer in young patients. For this reason, a review of the experience with colorectal cancer in patients under age 40 years was undertaken at Brooke Army Medical Center. Material
and Methods
Records of the Tumor Registry for the 33 year period between 1947 and 1980 were reviewed. These charts were reviewed for.demographic data (age, sex and race) in addition to information on history, symptoms, tumor location, pathologic characteristics, surgical procedure, and additional chemotherapy or radiation therapy. Patients with carcinoid of the rectum or appendix were excluded. Results One hundred forty of 819 documented cases of colorectal malignancy occurred in patients age 40 years or under. Of these, 21 cases were diagnosed as From the General Surgery Service, Department of Surgery, Brooke Army Medical Center, Fort Sam Houston, Texas. The opinions or assertions contained herein are the private views of the authors and are not to be construed as official of as reflecting the views of tha Department of tha Army or the Department of Defense. Requests for reprints should be addressed to Kathryn L. Safford, MD, General Surgery Service, Damall Army Hospital, Fort Hood, Texas 76541. Presented at the 33rd Annual Meeting of the Southwestern Surgical Congress, Monterey, California, May 4-7, 1981.
Voiume 142, December 1991
carcinoid (16 appendiceal and 5 rectal). Twenty of these patients were excluded while 1 patient with carcinoid of the appendix and rectal carcinoma was included. The 120 cases reviewed represent an incidence of 14.6 percent. There were 86 male (72 percent) and 34 female patients (28 percent). Ninety-two patients were Caucasian, 16 were black, 11 were LatinAmerican and 1 was Oriental. The youngest patient in the series was 8 years old (lymphosarcoma of the cecum). A family history of colorectal cancer was found in six patients, while seven patients had ulcerative colitis and one had familial polyposis. Multiple symptoms were present in 70 percent of the patients, and 30 percent presented with a single symptom. The duration of symptoms was less than 1 month in 21 percent,~ 1 to 6 months in 41 percent, 6 to 12 months in 16 percent and greater than 1 year in 22 percent. The median duration of symptoms was 3 months. Comparison of the symptoms and duration is summarized in Table I. The pathologic diagnosis was adenocarcinoma in 118 patients, lymphosarcoma of the cecum in 1 and squamous cell carcinoma in 1. Following the Dukes’ classification system, 94 charts contained sufficient data to assign a Dukes’ classification: no class A; 19 class B, 33 class C and 42 class D. The anatomic locations of the tumors are illustrated in Figure 1. Chemotherapy was given to 29 patients and 11 received radiation therapy. Follow-up has been complete to this date on 98 percent of the patients (117). Two of the three patients lost to follow-up (at 14 and 24 years, respectively) were included in the 5 and 10 year survival rates. Overall, the 5 and 10 year survival rates were 34.6 and 32.2 percent, respectively. Patients with Dukes’ B lesions had 5 and 10 year survival rates of 66.6 percent. Patients with Dukes’ C lesions had 5
767
Safford et al
TABLE I
Symptoms and Duration
Symptoms Bleeding Pain Change in bowel habits Obstruction Weight loss Anemia Mass Fever
mo
Duration 1-6 mo 6-12 mo
>l
year
5 14 6
19
9
16 22
10 9
21 10 9
13 3 2 3 1
11 7 5 1
2 3 2 0 0
2 6 0 0 0
a
and 10 year survival rates of 36.6 and 30.0 percent, respectively. There were no 5 year survivors in the Dukes’ D group; those patients had a mean survival time of 10 months. The 5 year survival rate for the patients receiving chemotherapy and radiation therapy were 13.6 and 10 percent, respectively. Comments Colorectal carcinoma occurs infrequently in younger patients but is not uncommon. Our incidence of 14.6 percent was somewhat higher than in most previous reviews, but compatible with two similar reviews of military age populations [II]. The predominant presenting symptoms in the general population are abdominal pain, change in bowel habits, weight loss and bloody stool [1,15]. These are also the predominant symptoms in younger patients [2-7,11,12,16]. The duration of symptoms has been considered a measurement of delay in diagnosis and therefore indicative of ultimate prognosis [9,11]. Several investigators have suggested that if the duration of symptoms was reduced, prognosis might improve [17]. Support for this statement was offered by several studies of young patients that documented the average duration of symptoms as 5.5 to 8 months, [7-9,161, despite the fact that in the general population most patients present with a symptom duration of 6 months or less [l]. The median duration of symptoms in this series was 3 months, and did not correlate with Dukes’ classification or survival. A review of colon and rectal cancer by Welch at the Massachusetts General Hospital in 1974 documents a reduction in the duration of symptoms from 7 to 3 months but no significant change in the survival data [15]. A decline in survival rates with advancing Dukes’ classification parallels the trend seen in the general population [1,12,15]. This series agrees with previous reviews [11,16,18,19], which note a higher proportion of Dukes’ C and D lesions in younger patients. It has been suggested that this provides evidence of a more virulent tumor in younger patients [5]. However, this is not confirmed by analysis of the survival rates for each Dukes’ classification group. These individual
766
Figure 1, Anatomic distribution of tumors.
survival rates parallel those in the general population and do not support the likelihood of unique tumor behavior. In this series, as in the general population, the most accurate prognostic indicator of survival is the Dukes’ classification at the time of diagnosis. Tumor behavior as reflected by the duration of symptoms and survival rates appears to function independently of patient age. The results of this review do not offer evidence to support either a delay in diagnosis or unique tumor behavior in younger patients. Summary Brooke Army Medical Center Tumor Registry records from 1947 through 1980 were reviewed. One hundred forty of 819 patients with colorectal cancer were aged 40 or less. The 5 year survival rate was 35 percent and the 10 year survival rate 32 percent. The predominant presenting symptoms were bleeding, pain and change of bowel habits. The median duration of symptoms was 3 months. There were no Dukes’ A patients. The 5 and 10 year survival rates in 19 Dukes’ B patients were 67 percent. Thirty-three Dukes’ C patients had 5 and 10 year survival rates of 37 and 30 percent, respectively. In 42 Dukes’ D patients, however, there were no 5 year survivors, and the mean length of survival was only 10 months. Our findings support previous surveys which have concluded that stage at the time of diagnosis, rather than symptoms, duration or patient age, is the most accurate prognostic factor. References 1.
Falterman KW, Hill CB, Markey JC, Fox JW, Cohn I. Cancer Of the colon, rectum and anus: a review of 2,313 cases. Cancer 1974;34:951-9. 2. Sessions RT, Riddell DH. Kaplan HJ, Foster JH. Carcinoma of the colon in the first two decades of life. Ann Surg 1965; 162:279-84.
The American Journal of Surgery
Review of Colorectal Cancer
3. Ezzo JA. Sullivan JF, Mack RE. Carcinoma of the colon under the age of 40. Ann Intern Med 1958;49:321-5. 4. Recalde M, Holyoke EP, Elias EG. Carcinoma of the colon, rectum and anal canal in young patients. Surg Gynecol Obstet 1974;139:909-13. 5. Ferguson E. Obi LJ. Carcinoma of the colon and rectum in patients up to 25 years of age. Am Surg 1971;37:181-9. 6. Hall A, Coffey RJ. Cancer of the large bowel in the young adult. J Surg 1961;102:66-72. 7. Rosato FE, Frazier TG, Copeland EM, Miller LD. Carcinoma of the colon in young people. Surg Gynecol Obstet 1969; 129:29-32. 8. Coffey RJ, Cardenas F. Cancer of the bowel in the young adult. Dis Colon Rectum 1964;7:491-2. 9. Mayo CW, Pagtalunan RJG. Malignancy of the colon and rectum in patients under 30 years of age. Surgery 1963;53:71 l8. 10. Chabalko JJ, Fraumeni JF. Colorectal cancer in children: epidemiologic aspects. Dis Colon Rectum 1975;18:1-3. 11. Simstein NL, Kovalcik PJ, Cross GH. Colorectal carcinoma in patients less than forty years old. Dis Colon Rectum 1978; 21:169-71. 12. Howard EW, Cavallo C, Hovey LM. Nelson TG. Colon and rectal cancer in the young adult. Am Surg 1975;41:260-5. 13. Sanfelippo PM, Beahrs OH. Carcinoma of the colon in patients under forty years of age. Surg Gynecol Obstet 1974;138: 169-70. 14. Miller FE, Liechty RD. Adenocarcinoma of the colon and rectum in persons under 30 years of age. Am J Surg 1967;113: 507-10. 15. Welch JP, Donaldson GA. Recent experience in the management of the colon and rectum. Am J Surg 1974;127:25866. 16. Scarpa FJ, Hartmann WH, Sawyers JL. Adenocarcinoma of the colon and rectum in young adults. South Med J 1976;69: 24-7. 17. Gallagher EG, Zeigler MG. Rectal carcinoma in patients in the second and third decades of life. Am J Surg 1972;124: 655-9. 18. Roth-Mayo LA, Devitt JE, Brown FN. The effect of age on the
Volume 142, December 1981
behavior of carcinoma of the colon. Dis Colon Rectum 1968; 11:420-2. 19. Johnson JW, Judd ES, Dahlin DC. Malignant neoplasms of the colon and rectum in young persons. Arch Surg 1959;79: 21-8.
Discussion Anatolio B. Cruz, Jr. (San Antonio, TX): The symptoms in this series, as in others (bleeding, pain, change of bowel habits, obstruction, weight loss, anemia, mass) are not early symptoms. In the 94 patients who were staged, none were Dukes’ A, which is an indicting fact. Only 1 of 32 Dukes’ B, 14 of 32 Dukes’ C and 17 of 32 Dukes’ D patients were aged 30 years or less. We are not discovering the early lesions early enough. There are no data to suggest that they have more biologically aggressive tumors. Perhaps the development and increased use of biologic markers for screening will bring lesions to diagnosis and treatment earlier. William A. Albano (Omaha, NE): At Creighton University we have been interested in the hereditary cancer problem. In approximately 5 percent of all colon cancers seen at our institution, an autosomal dominant inheritance has been demonstrated. These may be classified as hereditary, nonpolyposis colon cancer. Such syndromes are characterized by a predominance of right-sided lesions and an increase in multiple primaries. A study just completed at Creighton Cancer Center involved survival assessment in 118 affected persons from 20 families manifesting hereditary colon cancer syndromes. Our study indicated that patients with hereditary colon cancer had a significantly better survival rate, stage for stage, than patients with sporadic colon cancer. The overall survival rate in the hereditary group at 5 years was approximately 67 percent.
769