Hereditary nonpolyposis colorectal cancer natural history
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]
Overview
If left untreated, hereditary nonpolyposis colorectal cancer progression occurs rapidly and is then followed by sentinel organ cancer or metastasis. Hereditary nonpolyposis colorectal cancer is an aggressive syndrome characterized by early onset of cancer. Affected organs include endometrium (second most common after colon), ovary, stomach, small intestine, hepatobiliary tract, upper urinary tract, brain, and skin. Complications of hereditary nonpolyposis colorectal cancer are usually related to the surgery. The 5-year relative survival of patients with hereditary nonpolyposis colorectal cancer is approximately 79.3%. A feature associated with worse prognosis is late stage diagnosis.
Natural History, Complications, and Prognosis
Natural History
- If left untreated, hereditary nonpolyposis colorectal cancer progression occurs rapidly and is then followed by sentinel organ cancers or metastasis.
- Other organ cancers include:[1]
- Genitourinary tract malignancies
- Endometrial carcinoma: 30 - 50% lifetime risk
- Ovarian tumors
- Urinary tract cancer
- Small bowel cancer: lifetime risk ~5%
- Duodenum 45%
- Jejunum 29%
- Ileum 12%
- Not specified 14%
- Gastric cancer
- CNS tumors (glioblastoma)
- More advanced stages are characterized by multiple organ metastasis.
Prognosis
- The 5-year relative survival of patients with hereditary nonpolyposis colorectal cancer is approximately 79.3%.[2]
Complications
- Complications that can develop as a result of hereditary nonpolyposis colorectal cancer are sentinel organ tumors, such as:
- Other complications of hereditary nonpolyposis colorectal cancer are usually related to the colorectal surgery.
References
- ↑ Hereditary nonpolyposis colorectal cancer. Dr Amir Rezaee. Radiopedia. http://radiopaedia.org/articles/hereditary-non-polyposis-colorectal-cancer-1 Accessed on December 3, 2015
- ↑ Vasen HF, Watson P, Mecklin JP, Lynch HT (1999). "New clinical criteria for hereditary nonpolyposis colorectal cancer (HNPCC, Lynch syndrome) proposed by the International Collaborative group on HNPCC". Gastroenterology. 116 (6): 1453–6. PMID 10348829.