Colorectal cancer in patients 20 years old or less in Taiwan.ABSTRACT Background. Colorectal cancer colorectal cancer Malignant tumour of the large intestine (colon) or rectum. Risk factors include age (after age 50), family history of colorectal cancer, chronic inflammatory bowel diseases, benign polyps, physical inactivity, and a diet high in fat. (CRC (Cyclical Redundancy Checking) An error checking technique used to ensure the accuracy of transmitting digital data. The transmitted messages are divided into predetermined lengths which, used as dividends, are divided by a fixed divisor. ) is predominantly a disease of the elderly population, but it sometimes occurs in young patients. The diagnosis of CRC in youngsters is often overlooked by physicians or presentation may be delayed. Methods. With assistance from the cancer registry A cancer registry is a systematic collection of data about cancer and tumor diseases. The data is collected by Cancer Registrars. Cancer Registrars capture a complete summary of patient history, diagnosis, treatment, and status for every cancer patient in the United States, and center of Taipei Veterans General Hospital, we collected data on all types of colorectal malignancy, including carcinoma, adenocarcinoma adenocarcinoma: see neoplasm. , or lymphoma in patients aged 20 or younger. All available medical charts and pathologic specimens were reviewed in detail. Results. A total of 28 cases were analyzed. The leading presenting symptom was abdominal pain Abdominal pain can be one of the symptoms associated with transient disorders or serious disease. Making a definitive diagnosis of the cause of abdominal pain can be difficult, because many diseases can result in this symptom. Abdominal pain is a common problem. (92%). The locations of the primary tumors were evenly distributed, and the major histologic type was predominantly adenocarcinoma. However, the proportion of mucinous mucinous /mu·ci·nous/ (mu´si-nus) resembling, or marked by formation of, mucin. mucinous relating to, resembling or containing mucin. adenocarcinoma was higher than that in the older population. Most of the cases were advanced (11 tumors were classified as Dukes stage C and another 11 as Dukes stage D). The overall 5-year survival rate was 21%. Conclusions. Despite the rarity of CRC during the first two decades of life, physicians need to be aware of the possibility and to evaluate suggestive signs and symptoms by colonoscopy or barium enema Barium Enema Definition A barium enema, also known as a lower GI (gastrointestinal) exam, is a test that uses x-ray examination to view the large intestine. . Family history of CRC, inflammatory bowel disease inflammatory bowel disease n. Abbr. IBD Any of several incurable and debilitating diseases of the gastrointestinal tract characterized by inflammation and obstruction of parts of the intestine. , previous polyps Polyps A tumor with a small flap that attaches itself to the wall of various vascular organs such as the nose, uterus and rectum. Polyps bleed easily, and if they are suspected to be cancerous they should be surgically removed. , or familial polyposis Familial Polyposis Definition Familial polyposis is an inherited condition which primarily affects the large intestine (colon and rectum). Large numbers of projecting masses of swollen and thickened or tumorous membrane (polyps) develop on the inner did not play a crucial role in this group of young patients. COLORECTAL CANCER (CRC) is predominantly a disease of the elderly, but it is not absolutely restricted to this population. In the US and European literature European literature refers to the literature of Europe. European literature includes literature in many languages; among the most important are English literature, Spanish literature, French literature, Polish literature, German literature, Italian literature, Greek , the incidence of CRC in subjects aged 40 or younger is reported to be between 2% and 8%.[1-3] It is even rarer during the first two decades of life, and there have been several reports of CRC in this subgroup from Western countries.[4-6] Yamamoto et [al.sup.7] reviewed CRC in Japan from 1937 to 1989 and found only 43 cases in children younger than 15. In other reports, the primary tumor sites have been evenly distributed throughout the colon, [8,9] and the prognosis has been diverse. [10-13] In the pathologic analysis, the percentage of mucin-producing carcinoma in patients less than age 15 is higher than that in ordinary adenocarcinoma, which is related to poor prognosis. [10-17] The aim of this study was to retrospectively analyze the clinical and pathologic characteristics of CRC in young Chinese patients. MATERIALS AND METHODS We collected data on cases of CRC diagnosed from July 1, 1970, to June 30, 2000, in patients no more than 20 years old. All histologic types of malignancy were collected. In the Cancer Therapy Center of Taipei Veterans General Hospital, all important data on every cancer patient are abstracted and registered. The conditions and survival of the patients are regularly followed by the Center's staff through telephone interview. A total of 28 cases of CRC in patients aged [less than or equal to]20 were found from the file. The total number of cases of CRC at Taipei Veterans General Hospital during the study period was 7,634. RESULTS Twenty-eight cases (only 26 cases with available complete medical charts) were enrolled and analyzed. The male-female ratio was 16/12. The mean age at the time of diagnosis was 17.9 [+ or -] 2.14 years (range, 12 to 20 years). The major presenting symptoms were abdominal pain, palpable abdominal mass An abdominal mass is any localized enlargement or swelling in the human abdomen. Depending on its location, the abdominal mass may be caused by an enlarged liver (hepatomegaly), enlarged spleen (splenomegaly), protruding kidney, a pancreatic mass, a retroperitoneal mass (a mass in , abdominal fullness, unexplained weight loss, and bleeding (Table 1). No specific predisposing factor was found. One patient had a family history of CRC (his grandmother had died of CRC at the age of 72), and one had had adenomatous polyps of the colon previously. None of these patients had a history of inflammatory bowel disease or familial polyposis. The primary tumor sites were evenly distributed throughout the colon, and 6 patients had synchronous tumors (Table 2). The distribution of histologic types was adenocarcinoma in 13 (46%), mucinous adenocarcinoma in 5 (18%), signet ring cell signet ring cell A usually malignant cell containing copious clear cytoplasm that flattens a hyperchromatic nucleus to one side, having an appearance fancifully likened to a signet ring; CAs composed predominantly of SRCs often carry a worse prognosis; the carcinoma in 2 (7%), lymphoma in 2 (7%), and unspecified carcinoma in 1 (4%). There was no pathologic diagnosis in 6 patients either because the diagnosis was based on clinical course or they were referred from another hospital without definite pathologic reports. Dukes staging of disease (not including the two cases of lymphoma) showed only 1 Dukes B cancer; 11 cases were Dukes C, and another 11 were Dukes D. None of these lesions was Dukes A, and three could not be staged because of the shortage of detail in the pathologic description (Table 3). Surgery remained the mainstay of treatment of the disease. Eighteen patients were treated by surgical resection, 3 by chemotherapy, and 2 by radiotherapy. The remaining 5 patients received palliative care palliative care (paˑ·lē·ā·tiv kerˑ), n an approach to health care that is concerned primarily with attending to physical and emotional comfort rather only because of advanced disease or poor general condition. The overall 5-year survival rate was 21.4% and the median survival was 44 months. In patients with Dukes C disease, the 5-year survival rate was 27.3% (3/11), and in those with Dukes D disease, it was 9.1% (1/11). The only patient with Dukes B disease is still alive more than 25 years later. DISCUSSION The incidence of CRC in patients less than 40 years old is estimated to be 2% to 8% in the United States and Europe, (1-3) and about 1.3 cases per million children occur in Japan. (7) In our series, CRC in patients (aged no more than 20 years) represented only 0.37% of all cases of CRC over the past three decades at Taipei Veterans General Hospital. In other reports of CRC in young patients, the tumors have been evenly distributed throughout the colon. (7-9) Our results showed a similar pattern except for the higher rate of synchronous lesions. Adenocarcinoma was the major pathologic diagnosis in previous series and in ours. The reported percentage of mucinous carcinoma mucinous carcinoma n. An adenocarcinoma in which the neoplastic cells secrete significant amounts of mucin. Also called colloid carcinoma. has ranged from 22% to 43% of cases in young patients with CRC (9,15) and significantly higher than in older patients. (16,17) In our series, careful review of the pathologic specimens showed that the percentage of mucinous adenocarcinoma was 17.9% among all patients, but it would be 19.2% if the 2 patients with lymphoma were excluded. The presenting signs and symptoms in our study were similar to those described in previous reports, but there was a higher incidence of abdominal fullness and even intestinal obstruction intestinal obstruction Blockage of the small intestine or large intestine, resulting from either lack of peristalsis or mechanical obstruction (e.g., by narrowing, foreign objects, or hernia). Obstruction near the start of the small intestine often causes vomiting. . This might be due to the delayed diagnosis. The median survival was 44 months, which is similar to that in other series. (9) We analyzed the relationship between survival, histologic type, and Duke's stage, but no statistical significance was obtained due to a small number in each subgroup. The prognosis in CRC in the young patient is usually poor. (8) The 5-year survival in the present series is 21.4%, which is similar to that in some previous series. (8-11) The high percentage of mucinous carcinoma and late stage at presentation may explain the poor prognosis. (10-13,15) Our results were consistent with previous reports in that neither family history, previous colonic polyps, nor familial polyposis syndrome had any correlation with early age at diagnosis of CRC. (1-11) The predisposing factors for CRC in the young should be assumed to be different from the conventional ones in elderly patients with CRC. The relationship of CRC and age is believed to be the result of constant exposure to carcinogens Carcinogens Substances in the environment that cause cancer, presumably by inducing mutations, with prolonged exposure. Mentioned in: Colon Cancer, Rectal Cancer , such as diet factors, starting in early life. (18) Nevertheless, such a relationship cannot explain those cases with extraordinarily early onset of disease. Epidemiologic data suggest that some genetic factor might play a role in the carcinogenesis car·ci·no·gen·e·sis n. The production of cancer. carcinogenesis production of cancer. biological carcinogenesis viruses and some parasites are capable of initiating neoplasia. of CRC in young patients in China, (14) but no co nclusive pattern has been confirmed. Hereditary nonpolyposis colorectal cancer Hereditary nonpolyposis colorectal cancer (HNPCC), also known as Lynch syndrome, is characterized by an increased risk of colorectal cancer and other cancers of the endometrium, ovary, stomach, small intestine, hepatobiliary tract, upper urinary tract, brain, and skin. (HNPCC HNPCC Hereditary Nonpolyposis Colorectal Cancer HNPCC Hereditary non-polyposis colon cancer ) is a syndrome of inherited bowel cancer with or without other cancers. It has been reported that 5% to 15% of all CRC might be accounted for by HNPCC. (19) According to the "Amsterdam criteria Amsterdam criteria Oncology Criteria for diagnosing hereditary nonpolyposis colorectal cancer, see there Amsterdam criteria
References (1.) Pitluk H, Poticha SM: Carcinoma of colon and rectum in patients less than 40 years of age. Surg Gynecol Obstet 1991; 172:1-7 (2.) Jarvinen HJ, Turunen MJ: colorectal carcinoma before 40 years of age: prognosis and predisposing conditions. Scand J Gastroenterol 1984; 19:634-638 (3.) Safford KL, Spebar MJ, Rosenthal D: Review of colorectal cancer in patients under age 40 years. Am J Surg 1981; 142:767-769 (4.) Anderson A, Bergdahi L: Carcinoma of the colon in children: a report of six new cases and a review of the literature. J Pediatr Surg l976; 11:967-971 (5.) Middelkamp JN, Haffner H: Carcinoma of the colon in children. Pediatrics 1963; 32:558-571 (6.) Cain AS, Longino LA: Carcinoma of the colon in children. J Pediatr Surg 1970; 5:527-532 (7.) Yamamoto K, Tanaka T, Kuno K, et al: Carcinoma of the colon in children: case report and review of the Japanese literature. J Gastroenterol 1994; 29:647-652 (8.) Minardi AJ Jr, Sittig KM, Zibari GB, et al: Colorectal cancer in the young patients. Am Surg 1998; 64:849-853 (9.) Lee PY, Fletcher WS, Sullivan ES, et al: Colorectal cancer in young patients: characteristics and outcome, Am Surg 1994; 60:607-612 (10.) LaQuaglia MP, Heller G, Filippa DA, et al: Prognostic factors and outcome in patients 21 years and under with colorectal carcinoma. J Pediatr Surg 1992; 27:1085-1090 (11.) Odone V. Chang L, Caces J, et al: The natural history of colorectal carcinoma in adolescents. Cancer 1982; 49:1716-1720 (12.) Petrek J, Sandberg W, Bean P: The role of gender and other factors in the prognosis of young patients with colorectal cancer. Cancer 1985; 56:952-955 (13.) Umpleby HC, Williamson RCN RCN n abbr (= Royal Canadian Navy) → kanadische Marine : Carcinoma of the large bowel large bowel n. See large intestine. in the first four decades. Br J Surg 1984; 71:272-277 (14.) Yang G: Comparison between large bowel cancer in young people and that in middle-aged and old-aged people. Chin J Epidemiol 1993; 14:341-345 (15.) Shahrudin MD, Noori SM: Cancer of the colon and rectum in the first three decades of life. Hepatogastroenterology 1997; 44:441-444 (16.) Symonds DA, Vickery AL: Mucinous carcinoma of the colon and rectum. Cancer 1976; 37:1891-1900 (17.) Hulten L, Kewenter J, Ahren C, et al: Clinical and morphological characteristics of colitis carcinoma and colorectal carcinoma of young people. Scand J Gastroenterol 1979; 14:673-678 (18.) Fishel R, Lescoe MK, Rao MR, et al: The human mutator A mutator may refer to:
n. Variant of homologue. MSH MSH melanocyte-stimulating hormone. MSH abbr. melanocyte-stimulating hormone MSH, n See hormone, melanocyte-stimulating. MSH melanocyte-stimulating hormone. 2 and its association with hereditary nonpolyposis colon cancer hereditary nonpolyposis colon cancer Lynch I syndrome Oncology A relatively distinct AD form of cancer that may account for 5-10% of all colorectal CA Clinical Family Hx of colorectal CA at relatively young age, primarily of proximal colon, tendency toward multiple . Cell 1993; 75:1027-1038 (19.) Leach FS, Nicolaides NC, Papadopoulos N, et al: Mutations of a mutS homolog in hereditary nonpolyposis colon cancer. Cell 1993; 75:1215-1225 (20.) Nicolaides NC, Papadopoulos N, Liu B, et al: Mutations of two PMS (Pantone Matching System) A color matching system that has a unique number assigned to more than 500 different colors and shades. This standard for the printing industry has been built into many graphics and desktop publishing programs to ensure color accuracy. homologues in hereditary nonpolyposis colon cancer. Nature 1994; 371:75-80 (21.) Papadopoulos N, Nicolaides NC, Wei YF, et al: Mutation of a mut L homolog in hereditary colon cancer colon cancer, cancer of any part of the colon (often called the large intestine). Colon cancer is the second most common cancer diagnosed in the United States. . Science 1994; 263: 1625-1629 (22.) Liu B, Farrington SM, Petersen GM, et al: Genetic instability occurs in the majority of young patients with colorectal cancer. Natl Med 1995; 1:348-352 (23.) Dunlop MG, Farrington SM, Carothers AD, et al: Cancer risk associated with germline DNA mismatch repair gene mutations. Hum Mol Genet genet: see civet. 1997; 6:105-110 (24.) Farrington SM, Lin-Goerke J, Ling J, et al: Systematic analysis of hMSH2 and hMLH1 in young colon cancer patients and controls. Am J Hum Genet 1998; 63:749-759 (25.) Newell GR, Spitz spitz Any of several northern dogs, including the chow chow, Pomeranian, and Samoyed, characterized by a dense, long coat, erect pointed ears, and a tail that curves over the back. In the U.S. MR, Sider JG: Cancer and age. Semin Oncol 1989; 16:3-9 (26.) Dunlop MG: Molecular genetics molecular genetics n. The branch of genetics that deals with hereditary transmission and variation on the molecular level. of colon cancer. The Molecular Genetics of Cancer. Cowell JK (ed). Oxford, BIOS Scientific Publications, 1995, pp 113-134
TABLE 1
Clinical Manifestations of Colorectal Cancer in Patients Aged 12 to 20
Years
Presenting No. of Patients (%)
Features (n = 26)
Abdominal pain 24 (92.3)
Palpable mass 10 (38.5)
Abdominal distention 10 (38.5)
Unexplained body 6 (23.1)
weight loss (*)
Bleeding 4 (15.4)
(*)Defined as reduction of more than 5% of baseline body weight in 1
month without any known cause.
TABLE 2
Primary Tumor Distribution
Primary No. of Patients (%)
Tumor Site (n = 28)
Cecum 5 (17.9)
Ascending colon 3 (10.7)
Hepatic flexure 3 (10.7)
Transverse colon 5 (17.9)
Descending colon 1 (3.6)
Sigmoid colon 5 (17.9)
Synchronous tumor 6 (21.4)
TABLE 3
Histopathology and Dukes Staging of Colorectal Cancers
No. of
Patients Dukes Stage
Histopathology (n = 28) A B C
Adenocarcinoma 13 7
Mucinous adenocarcinoma 5 1 3
Signet-ring cell carcinoma 2 1
Lymphoma 2
Unspecified carcinoma 1
No pathologic diagnosis 5
Dukes No
Stage
Histopathology D Staging
Adenocarcinoma 4 2
Mucinous adenocarcinoma 1
Signet-ring cell carcinoma 1
Lymphoma
Unspecified carcinoma 1
No pathologic diagnosis 4 1
RELATED ARTICLE: KEY POINTS * Colorectal cancer can be easily overlooked in youngsters. * Among the 28 cases analyzed, the presenting symptom was abdominal pain in 92%. * Most cases were advanced, and the overall survival rate was only 21% at 5 years. * Physicians should use colonoscopy or barium enema examination to evaluate suggestive symptoms in young patients. |
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