Hereditary nonpolyposis colorectal cancer natural history: Difference between revisions
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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 centinel organ cancer or metastasis. Hereditary nonpolyposis colorectal cancer is an aggressive syndrome characterized by early onset of cancer. Common sites of affection include endometrium (second most common after colon), ovary, stomach, small intestine, hepatobiliary tract, upper urinary tract, brain, and skin. Complications of HNPCC are usually related to the surgery. The 5-year relative survival of patients with colorectal HNPCC cancer is approximately 79.3%. A feature associated with worse prognosis is late stage diagnosis.[1]
Natural History
- If left untreated, hereditary nonpolyposis colorectal cancer progression occurs rapidly and is then followed by centinel organ cancers or metastasis.
- Other organ cancers include:[2]
- 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 colorectal HNPCC cancer is approximately 79.3%.[3]
Complications
Complications that can develop as a result of HNPCC are sentinel organ tumors. Such as;
- Endometrial cancer.
- Gastric cancer, intestinal-type adenocarcinoma
- Ovarian cancer
- Urinary tract cancers, transitional carcinoma
- Small bowel cancers, duodenum and jejunum;
- central nervous system tumors, glioblastoma.
Other complications of HNPCC are usually related to the colorectal surgery.
References
- ↑ Stigliano V, Assisi D, Cosimelli M, et al. Survival of hereditary non-polyposis colorectal cancer patients compared with sporadic colorectal cancer patients. J Exp Clin Cancer Res. 2008;27:39.
- ↑ 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.