Cancer Res Treat.  2016 Apr;48(2):605-611. 10.4143/crt.2015.092.

Clinicopathological Features and Type of Surgery for Lynch Syndrome: Changes during the Past Two Decades

Affiliations
  • 1Department of Surgery, Seoul National University Bundang Hospital, Seongnam, Korea. kdw@snubh.org
  • 2Korean Hereditary Tumor Registry, Cancer Research Institute, Seoul National University College of Medicine, Seoul, Korea.
  • 3Department of Surgery, Seoul National University Hospital, Seoul, Korea.
  • 4Center for Colorectal Cancer, National Cancer Center, Goyang, Korea.

Abstract

PURPOSE
The Korean Hereditary Tumor Registry, the first and one of the largest registries of hereditary tumors in Korea, has registered about 500 families with hereditary cancer syndromes. This study evaluates the temporal changes in clinicopathologic features and surgical patterns of Lynch syndrome (LS) patients.
MATERIALS AND METHODS
Data on 182 unrelated LS patients were collected retrospectively. The patients were divided into the period 1 group (registered in 1990-2004) and 2 (registered in 2005-2014). The clinical characteristics of the two groups were compared to identify changes over time.
RESULTS
The period 1 group included 76 patients; the period 2 group, 106 patients. The mean ages at diagnosis were 45.1 years (range, 13 to 85 years) for group 1 and 49.7 years (range, 20 to 84 years) for group 2 (p=0.015). The TNM stage at diagnosis did not differ significantly-period 1 group: stage 0-I (n=18, 23.7%), II (n=37, 48.7%), III (n=19, 25.0%), and IV (n=2, 2.6%); period 2 group: stage 0-I (n=30, 28.3%), II (n=35, 33.0%), III (n=37, 34.9%), and IV (n=4, 3.8%). Extended resection was more frequently performed (55/76, 72.4%) in the period 1 group than period 2 (49/106, 46.2%) (p=0.001).
CONCLUSION
Colorectal cancer in patients with LS registered at the Korean Hereditary Tumor Registry is still diagnosed at an advanced stage, more than two decades after registry's establishment. Segmental resection was more frequently performed in the past decade. A prompt nationwide effort to raise public awareness of hereditary colorectal cancer and to support hereditary cancer registries is required in Korea.

Keyword

Registration; Hereditary nonpolyposis colorectal neoplasms; Lynch syndrome

MeSH Terms

Colorectal Neoplasms
Colorectal Neoplasms, Hereditary Nonpolyposis*
Diagnosis
Humans
Korea
Neoplastic Syndromes, Hereditary
Registries
Retrospective Studies

Cited by  1 articles

Germline Variants in MLH1, MSH2, and MSH6 in Korean Patients with Lynch Syndrome
Kyoung-Jin Park, Dong Kyung Chang, Hee Cheol Kim, Jong-Won Kim
Lab Med Online. 2018;8(4):156-166.    doi: 10.3343/lmo.2018.8.4.156.


Reference

References

1. Aaltonen LA, Salovaara R, Kristo P, Canzian F, Hemminki A, Peltomaki P, et al. Incidence of hereditary nonpolyposis colorectal cancer and the feasibility of molecular screening for the disease. N Engl J Med. 1998; 338:1481–7.
Article
2. Vasen HF, Moslein G, Alonso A, Bernstein I, Bertario L, Blanco I, et al. Guidelines for the clinical management of Lynch syndrome (hereditary non-polyposis cancer). J Med Genet. 2007; 44:353–62.
Article
3. Lynch HT, de la Chapelle A. Hereditary colorectal cancer. N Engl J Med. 2003; 348:919–32.
Article
4. Vasen HF, Watson P, Mecklin JP, Lynch HT. New clinical criteria for hereditary nonpolyposis colorectal cancer (HNPCC, Lynch syndrome) proposed by the International Collaborative group on HNPCC. Gastroenterology. 1999; 116:1453–6.
Article
5. Umar A, Boland CR, Terdiman JP, Syngal S, de la Chapelle A, Ruschoff J, et al. Revised Bethesda Guidelines for hereditary nonpolyposis colorectal cancer (Lynch syndrome) and microsatellite instability. J Natl Cancer Inst. 2004; 96:261–8.
Article
6. Rodriguez-Bigas MA, Cutait R, Lynch PM, Tomlinson I, Vasen HF. Hereditary colorectal cancer. New York: Springer;2010. p. 535–44.
7. Barrow E, Alduaij W, Robinson L, Shenton A, Clancy T, Lalloo F, et al. Colorectal cancer in HNPCC: cumulative lifetime incidence, survival and tumour distribution. A report of 121 families with proven mutations. Clin Genet. 2008; 74:233–42.
Article
8. de Vos tot Nederveen Cappel WH, Jarvinen HJ, Lynch PM, Engel C, Mecklin JP, Vasen HF. Colorectal surveillance in Lynch syndrome families. Fam Cancer. 2013; 12:261–5.
9. Schofield L, Grieu F, Amanuel B, Carrello A, Spagnolo D, Kiraly C, et al. Population-based screening for Lynch syndrome in Western Australia. Int J Cancer. 2014; 135:1085–91.
Article
10. Sturgeon D, McCutcheon T, Geiger TM, Muldoon RL, Herline AJ, Wise PE. Increasing Lynch syndrome identification through establishment of a hereditary colorectal cancer registry. Dis Colon Rectum. 2013; 56:308–14.
Article
11. Barrow P, Khan M, Lalloo F, Evans DG, Hill J. Systematic review of the impact of registration and screening on colorectal cancer incidence and mortality in familial adenomatous polyposis and Lynch syndrome. Br J Surg. 2013; 100:1719–31.
Article
12. Mallinson EK, Newton KF, Bowen J, Lalloo F, Clancy T, Hill J, et al. The impact of screening and genetic registration on mortality and colorectal cancer incidence in familial adenomatous polyposis. Gut. 2010; 59:1378–82.
Article
13. Park JG, Vasen HF, Park YJ, Park KJ, Peltomaki P, de Leon MP, et al. Suspected HNPCC and Amsterdam criteria II: evaluation of mutation detection rate, an international collaborative study. Int J Colorectal Dis. 2002; 17:109–14.
Article
14. Edge SB, Byrd DR, Compton CC, Fritz AG, Greene FL, Trotti A 3rd. AJCC cancer staging manual. 7th ed. New York: Springer-Verlag;2010.
15. Barrison AF, Smith C, Oviedo J, Heeren T, Schroy PC 3rd. Colorectal cancer screening and familial risk: a survey of internal medicine residents' knowledge and practice patterns. Am J Gastroenterol. 2003; 98:1410–6.
Article
16. Park J, Lee SY, Kim DW, Kang SB, Jeong SY, Park KJ. Knowledge of and practice patterns for hereditary colorectal cancer syndromes in korean surgical residents. Ann Coloproctol. 2013; 29:186–91.
Article
17. Brixen LM, Bernstein IT, Bulow S, Ehrnrooth E. Survival of patients with Stage III colon cancer is improved in hereditary non-polyposis colorectal cancer compared with sporadic cases. A Danish registry based study. Colorectal Dis. 2013; 15:816–23.
Article
18. de Jong AE, Hendriks YM, Kleibeuker JH, de Boer SY, Cats A, Griffioen G, et al. Decrease in mortality in Lynch syndrome families because of surveillance. Gastroenterology. 2006; 130:665–71.
Article
19. Vasen HF, Taal BG, Nagengast FM, Griffioen G, Menko FH, Kleibeuker JH, et al. Hereditary nonpolyposis colorectal cancer: results of long-term surveillance in 50 families. Eur J Cancer. 1995; 31A:1145–8.
Article
20. Cairns SR, Scholefield JH, Steele RJ, Dunlop MG, Thomas HJ, Evans GD, et al. Guidelines for colorectal cancer screening and surveillance in moderate and high risk groups (update from 2002). Gut. 2010; 59:666–89.
Article
21. Syngal S, Brand RE, Church JM, Giardiello FM, Hampel HL, Burt RW, et al. ACG clinical guideline: Genetic testing and management of hereditary gastrointestinal cancer syndromes. Am J Gastroenterol. 2015; 110:223–62.
Article
22. Win AK, Parry S, Parry B, Kalady MF, Macrae FA, Ahnen DJ, et al. Risk of metachronous colon cancer following surgery for rectal cancer in mismatch repair gene mutation carriers. Ann Surg Oncol. 2013; 20:1829–36.
Article
23. Rodriguez-Bigas MA, Moeslein G. Surgical treatment of hereditary nonpolyposis colorectal cancer (HNPCC, Lynch syndrome). Fam Cancer. 2013; 12:295–300.
Article
24. Haanstra JF, de Vos Tot Nederveen Cappel WH, Gopie JP, Vecht J, Vanhoutvin SA, Cats A, et al. Quality of life after surgery for colon cancer in patients with Lynch syndrome: partial versus subtotal colectomy. Dis Colon Rectum. 2012; 55:653–9.
Article
Full Text Links
  • CRT
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2024 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr