FaCD Online Syndrome Fact Sheet

Last updated: 15 Sep 2008

Name: Colorectal Cancer, Multiple Primary Malignancies in Patients with

Tumor features

breast cancer
colorectal cancer
endometrial cancer
ovarian cancer (i.e. epithelial origin)
prostate cancer
small intestinal cancer

Tumor features (possible)

cervical cancer
melanoma, cutaneous
renal cell cancer
skin cancer, basal cell
skin cancer, squamous cell
thyroid cancer


Approximately 5% (3.8%-7.8%) of patients with colon cancer (will) have (had) primary cancer outside the colon[1]. Enblad et al.[2] analyzed the occurrence of a second primary malignant disease in 38,166 patients with cancer of the colon and 23,603 patients with rectal cancer reported to the Swedish Cancer Registry. Their study suggested that cancer of the small intestine, breast, endometrium, and possibly of the ovary and prostate may have etiologic factors in common with cancer of the large bowel, particularly those located in the colon.
Evans et al.[3] studied 127,281 colorectal cancer patients from the Thames Cancer Registry database for the occurrence of second primary cancers. Small intestinal cancer was significantly increased in men diagnosed with colorectal cancer before the age of 60 years and in women diagnosed with colorectal cancer after the age of 65 years. Colorectal cancer was also significantly increased after a first diagnosis of cancer of the small intestine. Other cancer sites with a significant increase after colorectal cancer included the cervix uteri, corpus uteri, and ovary.
McCredie et al.[4] examined data from the New South Wales Central Cancer Registry to determine the risk of second primary cancers after an initial invasive colorectal cancer. After colon cancer, there was an excess of cancers of the small intestine in both sexes (Relative Risk (RR)s of 4.5 and 4.4); prostate (1.4) and kidney (1.8) in men; and breast (1.3), body of uterus (1.9), ovary (2.8), and thyroid (2.7)(see also this ref[8]) in women. After rectal cancer, men had increased risks of cancers of the colon (1.5) and prostate (1.3).
Malmer et al.[5] examined the associations between colorectal cancer as first primary cancer and the risk of developing astrocytoma and meningioma as a second primary cancer in Swedish colorectal cancer patients (572, 422 person-years). A significant increased risk for developing meningioma was seen, standardized incidence ratio 1.60 (95% CI 1.32-1.94). An increased risk to develop colorectal cancer has been suggested in patients with malignant skin tumors[6], although a decrease in risk has been suggested by others[7].

General aspects to consider with respect to multiple primary tumors:
- Shared genetic (immune response, metabolic/hormonal/DNA-repair pathways) or non-genetic (chemical carcinogens, radiation, viruses, life-style) risk factors
- Therapy (radiation, chemotherapy, hormonal,...) related effects
- Possible bias because of increased surveillance and autopsy findings


[1] Lee TK, Barringer M, Myers RT, Sterchi JM. Multiple primary carcinomas of the colon and associated extracolonic primary malignant tumors. Ann Surg 1982; 195(4):501-507.
[2] Enblad P, Adami HO, Glimelius B, Krusemo U, Pahlman L. The risk of subsequent primary malignant diseases after cancers of the colon and rectum. A nationwide cohort study. Cancer 1990; 65(9):2091-2100.
[3] Evans HS, Moller H, Robinson D, Lewis CM, Bell CM, Hodgson SV. The risk of subsequent primary cancers after colorectal cancer in southeast England. Gut 2002; 50(5):647-652.
[4] Mccredie M, Macfarlane GJ, Bell J, Coates M. Second primary cancers after cancers of the colon and rectum in New South Wales, Australia, 1972-1991. Cancer Epidemiol Biomarkers Prev 1997; 6(3):155-160.
[5] Malmer B, Tavelin B, Henriksson R, Gronberg H. Primary brain tumours as second primary: a novel association between meningioma and colorectal cancer. Int J Cancer 2000; 85(1):78-81.
[6] Levi F, Randimbison L, Te VC, Conconi MM, La Vecchia C. Risk of prostate, breast and colorectal cancer after skin cancer diagnosis. International journal of cancer 2008; epub ahead of print.
[7] Soerjomataram I, Louwman WJ, Lemmens VE, Coebergh JW, de Vries E. Are patients with skin cancer at lower risk of developing colorectal or breast cancer?. American journal of epidemiology 2008; 167(12):1421-9.
[8] Subramanian S, Goldstein DP, Parlea L, Thabane L, Ezzat S, Ibrahim-Zada I, Straus S, Brierley JD, Tsang RW, Gafni A, Rotstein L, Sawka AM. Second primary malignancy risk in thyroid cancer survivors: a systematic review and meta-analysis. Thyroid 2007; 17(12):1277-88.