Colorectal Carcinoma in Young Persons: Experience at Howard University Hospital 1 955.1 977 Edward L. Mosley, Jr, MD, Ed B. Chung, MD, PhD, Edward E. Cornwell, MD, FACS, Javan Anderson, MD, and LaSalle D. Leffall, Jr, MD, FACS Washington, DC
Case records of all patients 30 years of age and under with a proven pathological diagnosis of colorectal cancer at Howard University Hospital between January 1955 and December 1977 were reviewed. Over this 23-year period, 14 cases were documented. All patients were black. This study reaffirms the poor prognosis which accompanies colorectal carcinoma in the young, particularly in those patients with mucinous carcinoma.
Introduction Colorectal carcinoma is a disease classically thought to be confined to the aged. In recent years it has become clear that this disease is not restricted to the aged. It is the most frequently diagnosed gastrointestinal malignancy regardless of age. ' Although it has been described in a stillborn, the youngest documented case is a nine-month-old girl. Recent publications suggest an unfavorable prognosis in young patients. Although there are many similarities to colorectal cancer in adults, the rare childhood mortality is greater in the black population than in the white, particularly in boys, reflecting the rising incidence of this tumor in young blacks.2 This paper reviews 23 years of our experience at Howard University Hospital (formerly Freedmen's Hospi-
Read at the 83rd Annual Convention and Scientific Assembly of the National Medical Association, Washington, DC, July 30-August 3, 1978. Won First place in Resident's CompetitionCharles R. Drew Forum. From the Departments Qf Surgery, Pathology, and Radiology at Howard University Hospital, Washington, DC. Requests for reprints should be addressed to Dr. Edward L. Mosley, Jr, Department of Surgery, Howard University Hospital, Washington, DC 20060
tal) with black patients 30 years and younger with carcinoma of the colorectum.
known dead, three patients are alive, and two patients were lost to follow-up. The survival after surgery is summarized in Table 7.
Material and Methods
Discussion
The case reports of all patients 30 years of age and under with a proven pathological diagnosis of colorectal cancer at Howard University Hospital, between January 1955 and December 1977, were reviewed.* Over this 23year period, 14 cases were documented. All patients in this study were black. Of the 14 patients nine were females and five were males. The average age was 26 years with the range from 15 to 30 years. The location of the carcinoma, presenting symptoms, and physical findings are summarized in Tables 1, 2, and 3, respectively. The types of cancers found, surgery performed, and stage of the disease are summarized in Tables 4, 5, and 6, respectively. Nine of the 14 patients are
Colorectal cancer is an extremely rare form of cancer in children and adolescents, with fewer than 300 cases having been reported in the literature.3 The 14 young patients in this series constitute 4.7 percent of all patients treated during the period under review. Our incidence of 4.7 percent is slightly higher than most series reported from predominatly white populations.4 Study of death rates for colorectal cancer in children in the United States showed a predominance in black children. Mortality was very low in children less than 15 years old and much higher at 15 to 19 years-the start of a rising trend through adult life. The incidence and mortality rates for colorectal cancer show excesses in blacks from childhood until middle life when the rates for blacks plateau and are surpassed for the continuously rising trend for whites.2 The most common presenting symptoms were rectal bleeding and change
*Two of the patients were seen at Greater Southeast Community Hospital on the Howard Surgical Service.
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 71, NO. 5, 1979
449
Table 1. Location of Carcinoma
Location
Number
Rectosigmoid Cecum Ascending colon Hepatic flexure Transverse colon Descending colon
8 2 1 1 1 1
Table 2. Presenting Symptoms
Number of Patients
Symptoms* Rectal bleeding Change of bowel habits Incomplete evacuation Constipation Decreased size of stool Diarrhea Weakness Anorexia
Percentage
9
64
9 3 3 2 1
64 21.4 21.4 14.2 7
3
21.4
3
21.4
Epigastric pain
3
21.4
Cachexia Rectal pain Right lower quadrant pain Anemia Vomiting Periumbilical pain
3
21.4
2
14.2
Lethargy Nausea Low back pain
2
14.2
2
14.2
2
14.2
2
14.2
2
14.2
1
7
1
7
*The duration of symptoms ranged from one week to two years with an average duration of three to four weeks.
Table 3. Physical Findings
Physical Findings
Patients
Rectal mass Abdominal distention Left lower quadrant tenderness Right lower quadrant tenderness Hepatomegaly Pelvic mass
3 2 2 1
1 1
*Positive physical findings were not found in 36% of the patients presenting.
450
of bowel habits (Table 2). Colorectal symptoms in the young should never automatically be attributed to benign disease. As with the older population, these complaints in the young merit full work-up to rule out carcinoma. Most of our patients had a relatively short duration of symptoms, yet were subsequently found to have far advanced lesions. Seventy-three percent of our patients had Dukes C or D lesions at diagnosis (Table 6). This supports the conclusion made by those who claim that symptoms become manifest late in young patients.; Six (42.8 percent) patients had mucinous adenocarcinoma (Table 4). Seventy-five percent of the patients that died less than four months after diagnosis had mucinous adenocarcinoma. This variety seems to be more lethal than the non-mucinous lesions. Sixty-four percent of our patients are dead (Table 7). Two of the patients were lost to follow-up and are presumed dead, judging from their poor condition when last seen. It is apparent that, regardless of type, carcinoma in the young black carries a very poor prognosis. The longest survival was 15 years post-surgery in a patient with non-mucinous adenocarcinoma. Since survival from carcinoma of the colon and rectum is directly related to the stage of the disease at the time of treatment, it is no wonder that young patients have an extremely poor prognosis with their disease.6 Four (26 percent) of our patients had a family history of intestinal polyps. In most instances a strong family history of colorectal cancer or associated premalignant conditions is lacking. However, a close search and follow-up of the concerned families should be undertaken, especially in those with diseases implicated as precursors of colon and rectal carcinoma. The majority of cases comprise ulcerative colitis, familial polyposis coli, and non-familial multiple polyps. Hall and Coffey have reported that 16 percent of 50 patients less than 40 years old had either ulcerative colitis, familial polyposis, or multiple polyposis prior to diagnosis.6 One of our patients under 30 had familial polyposis and carcinoma in situ.7 Dietary and epidemiologic data suggest that carcinoma of the colon and rectum is a carcinogen-induced disease which is strongly influenced by both exogenous and endogenous factors. In-
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 71, NO. 5, 1979
Table 4. Histological Types
Patients
Type
Adenocarcinoma* Mucinous adenocarcinoma
Table 6. Stage Using Dukes Modified Classification
Table 5. Surgery Performed
8 6
*Familial polyposis was present in one patient.
Operation
Patients
Abdomino-perineal resection Right hemicolectomy Palliative resection of left colon Anterior resection Sigmoid colostomy
5
Stage
Patients
A B C D
1 2 6 5
5 2 1
1
Table 7. Survival Post-Surgery
Time Range
Adenocarcinoma Surviving Deceased
Less than 4 months 1 to 3 years 4 to 6 years 6 to 9 years 10 to 15 years
1 0
1 2 1
1
Mucinous Adenocarcinoma* Surviving Deceased 3 1 1 1 0
1 -year mortality=41 % (both types) 5-year mortality=66% (both types) *Two patients were lost to follow-up.
creased fecal-bile acid concentrations are present in the stool of patients with carcinoma of the colon. In addition to these high levels of bile acids, clostridia, which are capable of dehydrogenating the bile acid steroid nucleus, are present. These factors could result in the presentation of endogenously produced carcinogens to the colonic mucosa. It has been known for decades that degradation products of bile salts are carcinogenic to animals. The inhabitants of the United States eat a diet high in refined carbohydrates (with low residue). Thus, any carcinogen in the bowel lumen would be present in much higher concentration, particularly if there is bowel stasis (which appears to be prevalent in the United States).' Although none of these factors has been investigated in the young population in particular, patients developing this disease at an earlier age may represent one extreme of the spectrum of increased susceptibility to these changes." Pratt
et a13 documented a history of exposure to farm or agricultural chemicals for eight of nine patients (adolescents) of rural backgrounds with colorectal carcinoma, and for one of four from urban backgrounds. These findings must be considered highly suggestive of additional causes.
Conclusion In conclusion, this study reaffirms the poor prognosis which accompanies colorectal carcinoma in the young (30 and under). Errors in diagnosis, probably due to young age and failure to recognize and treat the disease aggressively, may contribute to the poor prognosis. A high index of suspicion and complete work-up, including stools for blood, sigmoidoscopy, barium enema with air contrast, and, if indi-
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 71, NO. 5, 1979
cated, colonoscopy, are mandatory for earlier diagnosis and treatment of colorectal cancers in the young.
Literature Cited 1. Walton WW: Colorectal adenocarcinoma in patients less than 40 years old. Dis Colon Rectum 19:529-534, 1976 2. Chabalko JJ, Draumeni F: Colorectal cancer in children. Dis Colon Rectum 18:1-3, 1975 3. Pratt CB, Rivera G, Shanks E: Colorectal carcinoma in adolescents. Cancer 40:24642472, 1977 4. Sessions RTA, Riddell PH, Kaplan HJ: Carcinoma of the colon in the first two decades of life. Ann Surg 174:162-279, 1965 5. Miller FE, Richard D, Liechty RD: Adenocarcinoma of the colon and rectum in persons under 30 years of age. Am J Surg 113:500-507, 1967 6. Warren EE, Paloyan E, Kirsner J: Carcinoma of the colon in the adolescent. Am J Surg 133:737-742, 1977 7. Leffall LD Jr, Chung EB, DeWitty R: Familial polyposis coli in black patients. Ann Surg 186(3):324-333, 1977 8. Cohn Jr, Nance FC: The colon and rectum. In Sabiston DC Jr (ed): Textbook of Surgery, ed 10. Philadelphia, WB Saunders Co, 1972, p 965
451
Case records of all patients 30 years of age and under with a proven pathological diagnosis of colorectal cancer at Howard University Hospital between January 1955 and December 1977 were reviewed. Over this 23-year period, 14 cases were documented. All patients were black. This study reaffirms the poor prognosis which accompanies colorectal carcinoma in the young, particularly in those patients with mucinous carcinoma.
Introduction Colorectal carcinoma is a disease classically thought to be confined to the aged. In recent years it has become clear that this disease is not restricted to the aged. It is the most frequently diagnosed gastrointestinal malignancy regardless of age. ' Although it has been described in a stillborn, the youngest documented case is a nine-month-old girl. Recent publications suggest an unfavorable prognosis in young patients. Although there are many similarities to colorectal cancer in adults, the rare childhood mortality is greater in the black population than in the white, particularly in boys, reflecting the rising incidence of this tumor in young blacks.2 This paper reviews 23 years of our experience at Howard University Hospital (formerly Freedmen's Hospi-
Read at the 83rd Annual Convention and Scientific Assembly of the National Medical Association, Washington, DC, July 30-August 3, 1978. Won First place in Resident's CompetitionCharles R. Drew Forum. From the Departments Qf Surgery, Pathology, and Radiology at Howard University Hospital, Washington, DC. Requests for reprints should be addressed to Dr. Edward L. Mosley, Jr, Department of Surgery, Howard University Hospital, Washington, DC 20060
tal) with black patients 30 years and younger with carcinoma of the colorectum.
known dead, three patients are alive, and two patients were lost to follow-up. The survival after surgery is summarized in Table 7.
Material and Methods
Discussion
The case reports of all patients 30 years of age and under with a proven pathological diagnosis of colorectal cancer at Howard University Hospital, between January 1955 and December 1977, were reviewed.* Over this 23year period, 14 cases were documented. All patients in this study were black. Of the 14 patients nine were females and five were males. The average age was 26 years with the range from 15 to 30 years. The location of the carcinoma, presenting symptoms, and physical findings are summarized in Tables 1, 2, and 3, respectively. The types of cancers found, surgery performed, and stage of the disease are summarized in Tables 4, 5, and 6, respectively. Nine of the 14 patients are
Colorectal cancer is an extremely rare form of cancer in children and adolescents, with fewer than 300 cases having been reported in the literature.3 The 14 young patients in this series constitute 4.7 percent of all patients treated during the period under review. Our incidence of 4.7 percent is slightly higher than most series reported from predominatly white populations.4 Study of death rates for colorectal cancer in children in the United States showed a predominance in black children. Mortality was very low in children less than 15 years old and much higher at 15 to 19 years-the start of a rising trend through adult life. The incidence and mortality rates for colorectal cancer show excesses in blacks from childhood until middle life when the rates for blacks plateau and are surpassed for the continuously rising trend for whites.2 The most common presenting symptoms were rectal bleeding and change
*Two of the patients were seen at Greater Southeast Community Hospital on the Howard Surgical Service.
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 71, NO. 5, 1979
449
Table 1. Location of Carcinoma
Location
Number
Rectosigmoid Cecum Ascending colon Hepatic flexure Transverse colon Descending colon
8 2 1 1 1 1
Table 2. Presenting Symptoms
Number of Patients
Symptoms* Rectal bleeding Change of bowel habits Incomplete evacuation Constipation Decreased size of stool Diarrhea Weakness Anorexia
Percentage
9
64
9 3 3 2 1
64 21.4 21.4 14.2 7
3
21.4
3
21.4
Epigastric pain
3
21.4
Cachexia Rectal pain Right lower quadrant pain Anemia Vomiting Periumbilical pain
3
21.4
2
14.2
Lethargy Nausea Low back pain
2
14.2
2
14.2
2
14.2
2
14.2
2
14.2
1
7
1
7
*The duration of symptoms ranged from one week to two years with an average duration of three to four weeks.
Table 3. Physical Findings
Physical Findings
Patients
Rectal mass Abdominal distention Left lower quadrant tenderness Right lower quadrant tenderness Hepatomegaly Pelvic mass
3 2 2 1
1 1
*Positive physical findings were not found in 36% of the patients presenting.
450
of bowel habits (Table 2). Colorectal symptoms in the young should never automatically be attributed to benign disease. As with the older population, these complaints in the young merit full work-up to rule out carcinoma. Most of our patients had a relatively short duration of symptoms, yet were subsequently found to have far advanced lesions. Seventy-three percent of our patients had Dukes C or D lesions at diagnosis (Table 6). This supports the conclusion made by those who claim that symptoms become manifest late in young patients.; Six (42.8 percent) patients had mucinous adenocarcinoma (Table 4). Seventy-five percent of the patients that died less than four months after diagnosis had mucinous adenocarcinoma. This variety seems to be more lethal than the non-mucinous lesions. Sixty-four percent of our patients are dead (Table 7). Two of the patients were lost to follow-up and are presumed dead, judging from their poor condition when last seen. It is apparent that, regardless of type, carcinoma in the young black carries a very poor prognosis. The longest survival was 15 years post-surgery in a patient with non-mucinous adenocarcinoma. Since survival from carcinoma of the colon and rectum is directly related to the stage of the disease at the time of treatment, it is no wonder that young patients have an extremely poor prognosis with their disease.6 Four (26 percent) of our patients had a family history of intestinal polyps. In most instances a strong family history of colorectal cancer or associated premalignant conditions is lacking. However, a close search and follow-up of the concerned families should be undertaken, especially in those with diseases implicated as precursors of colon and rectal carcinoma. The majority of cases comprise ulcerative colitis, familial polyposis coli, and non-familial multiple polyps. Hall and Coffey have reported that 16 percent of 50 patients less than 40 years old had either ulcerative colitis, familial polyposis, or multiple polyposis prior to diagnosis.6 One of our patients under 30 had familial polyposis and carcinoma in situ.7 Dietary and epidemiologic data suggest that carcinoma of the colon and rectum is a carcinogen-induced disease which is strongly influenced by both exogenous and endogenous factors. In-
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 71, NO. 5, 1979
Table 4. Histological Types
Patients
Type
Adenocarcinoma* Mucinous adenocarcinoma
Table 6. Stage Using Dukes Modified Classification
Table 5. Surgery Performed
8 6
*Familial polyposis was present in one patient.
Operation
Patients
Abdomino-perineal resection Right hemicolectomy Palliative resection of left colon Anterior resection Sigmoid colostomy
5
Stage
Patients
A B C D
1 2 6 5
5 2 1
1
Table 7. Survival Post-Surgery
Time Range
Adenocarcinoma Surviving Deceased
Less than 4 months 1 to 3 years 4 to 6 years 6 to 9 years 10 to 15 years
1 0
1 2 1
1
Mucinous Adenocarcinoma* Surviving Deceased 3 1 1 1 0
1 -year mortality=41 % (both types) 5-year mortality=66% (both types) *Two patients were lost to follow-up.
creased fecal-bile acid concentrations are present in the stool of patients with carcinoma of the colon. In addition to these high levels of bile acids, clostridia, which are capable of dehydrogenating the bile acid steroid nucleus, are present. These factors could result in the presentation of endogenously produced carcinogens to the colonic mucosa. It has been known for decades that degradation products of bile salts are carcinogenic to animals. The inhabitants of the United States eat a diet high in refined carbohydrates (with low residue). Thus, any carcinogen in the bowel lumen would be present in much higher concentration, particularly if there is bowel stasis (which appears to be prevalent in the United States).' Although none of these factors has been investigated in the young population in particular, patients developing this disease at an earlier age may represent one extreme of the spectrum of increased susceptibility to these changes." Pratt
et a13 documented a history of exposure to farm or agricultural chemicals for eight of nine patients (adolescents) of rural backgrounds with colorectal carcinoma, and for one of four from urban backgrounds. These findings must be considered highly suggestive of additional causes.
Conclusion In conclusion, this study reaffirms the poor prognosis which accompanies colorectal carcinoma in the young (30 and under). Errors in diagnosis, probably due to young age and failure to recognize and treat the disease aggressively, may contribute to the poor prognosis. A high index of suspicion and complete work-up, including stools for blood, sigmoidoscopy, barium enema with air contrast, and, if indi-
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 71, NO. 5, 1979
cated, colonoscopy, are mandatory for earlier diagnosis and treatment of colorectal cancers in the young.
Literature Cited 1. Walton WW: Colorectal adenocarcinoma in patients less than 40 years old. Dis Colon Rectum 19:529-534, 1976 2. Chabalko JJ, Draumeni F: Colorectal cancer in children. Dis Colon Rectum 18:1-3, 1975 3. Pratt CB, Rivera G, Shanks E: Colorectal carcinoma in adolescents. Cancer 40:24642472, 1977 4. Sessions RTA, Riddell PH, Kaplan HJ: Carcinoma of the colon in the first two decades of life. Ann Surg 174:162-279, 1965 5. Miller FE, Richard D, Liechty RD: Adenocarcinoma of the colon and rectum in persons under 30 years of age. Am J Surg 113:500-507, 1967 6. Warren EE, Paloyan E, Kirsner J: Carcinoma of the colon in the adolescent. Am J Surg 133:737-742, 1977 7. Leffall LD Jr, Chung EB, DeWitty R: Familial polyposis coli in black patients. Ann Surg 186(3):324-333, 1977 8. Cohn Jr, Nance FC: The colon and rectum. In Sabiston DC Jr (ed): Textbook of Surgery, ed 10. Philadelphia, WB Saunders Co, 1972, p 965
451
