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HNPCC-Lynch-Syndrom
Metachrone Kolonkarzinome
- Rektumkarzinome
- Endometriumkarzinome
- Ovarialkarzinome
- Urothelkarzinome
- Magenkarzinome
- andere
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HNPCC-Lynch-Syndrom

metachrone Kolonkarzinome
- Rektumkarzinome
- Endometriumkarzinome
- Ovarialkarzinome
- Urothelkarzinome
- Magenkarzinome
- andere


- Metachrone Kolonkarzinome

a) Survival

1) Aarnio M, Mustonen H, Mecklin JP, Järvinen HJ. Ann Med. 1998 Feb;30(1):75-80.:

Cumulative 5-year survival 43 LS 86%, 122 sporadic colorectal cancer 59%, hazard ratio 0.41, P = 0.02.

2) Bertario L, Russo A, Sala P, Eboli M, Int J Cancer. 1999 Jan 18;80(2):183-7

144 HNPCC, and 2035 sporadic-cancer groups, the 5-year cumulative survival rate was 56.9%, and 50.6% respectively, n.s.

3) Haghighi MM, Vahedi M, Mohebbi SR, Asian Pac J Cancer Prev. 2009;10(2):209-12

5-year survival was 82.5% in the HNPCC study group (n=60) compared with only 56.4% in the general population group (n=61) (P= 0.044).

4) Stigliano V, Assisi D, Cosimelli M, J Exp Clin Cancer Res. 2008 Sep 19;27:39

crude 5-years cumulative survival after the primary CRC was 94.2% in 40 HNPCC patients vs. 75.3% in 573 sporadic cancer patients (p < 0.0001)

b) Perioperative Mortalität und Morbidität

1) You YN, Chua HK, Nelson H Dis Colon Rectum. 2008 Jul;51(7):1036-43

Exclusion of right-sided colectomies! Various (benign and malign) indications! Unterschiedliches Patientenklientel! Retrospektiv! complication-free rate was 75.4 percent after 321 segmental resections, 42.8 percent after 91 ileosigmoid anastomosis, and 60 percent after 110 ileorectal anastomosis ( n.s.). Complications of Grades II and III did not differ significantly among the three groups, but incidences of ileus/partial small-bowel obstruction (Grade I) were significant after extended resection

c) Metachrone Kolonkarzinome

1) Natarajan N, Watson P, Silva-Lopez E, Dis Colon Rectum. 2010 Jan;53(1):77-82.

LS-Patienten mit MLH1 oder MSH2-Mutation; 37 subtotale Kolektomien (CA oder Prophylaxe), 69 segmental (gleiche Indikation) The 5-year event-free survival for subsequent colorectal cancer, subsequent abdominal surgery, and death was 94%, 84%, and 93%, respectively, for cases and 74%, 63%, and 88%, respectively, for controls. Times to subsequent colorectal cancer and subsequent abdominal surgery were significantly shorter in the control group (P < .006 and P < .04, respectively). Surveillance unregelmäßig (1-6 Jahre)

2) Van Dalen R, Church JM, McGannon E, Dis Colon Rectum. 2003;46:617–70.

Fifteen patients who underwent surgery elsewhere needed a second resection for metachronous cancer (15/53 patients having a segmental resection; Fisher's exact test, P = 0.094). Kann man einfach nur vergessen!!

3) Kalady MF, McGannon E, Vogel JD Ann Surg. 2010 Sep;252(3):507-11

296 LS mit CRC, 253 segmental, 38 subtotal. 87% follow-up colonoscopies, median 104 Monate. Adenome 33% versus 11%, high-risk Adenome 22%vs. 11% (absolut: 140 vs.6!). Metachrone Karzinome 25% vs. 8%. High risk adenoma and/or cancer: 37% vs 13%. Mediane Zeit zum metachronen Karzinom: 69 vs. 227 Monate. 4 Intervallkarzinome innerhalb von 12 Monaten Abstand zur letzten Koloskopie!

4) Parry S, Win AK, Parry B, Gut. 2011 Jul;60(7):950-7

382 MMR-CRCs, 332 segmental, 50 extended. Metachrone CRC 22% (95%-KI 18,8-29,7 pro 1000 Personenjahre) vs. 0% (95%-KI 0,0-7,2 pro 1000 Personenjahre), Inzidenz statistisch signifikant unterschiedlich. Cumulative risk of metachronous CRC was 16% (95% CI 10% to 25%) at 10 years, 41% (95% CI 30% to 52%) at 20 years and 62% (95% CI 50% to 77%) at 30 years after segmental colectomy.

5) Engel, C., Rahner, N., Schulmann, K., Clin Gastroenterol Hepatol. 2010;8(2):174-82

724 LS-CRC-Patienten cumulative risk of 23.7% (95% CI, 14.5%–32.9%) to develop a metachronous CRC within20 years after the first CRC. 81% of all colonoscopies were performed within 15 months. 19 of the 43 follow-up colonoscopies revealing a CRC had been preceded by a normal surveillance colonoscopy within the recommended interval of 12 months.

6) de Vos tot Nederveen Cappel WH, Nagengast FM, Griffioen G Dis Colon Rectum. 2002 Dec;45(12):1588-94.

The 10-year cumulative risk of developing colorectal cancer was 10.5 (95 percent confidence interval, 3.8-17.2) percent in proven mutation carriers, 15.7 (95 percent confidence interval, 4.1-27.3) percent after partial colectomy (n=110), and 3.4 percent after subtotal colectomy (n=29).

d) Nützliches

Bleiker EM, Menko FH, Taal BG, et al. Screening behavior of individuals at high risk for colorectal cancer. Gastroenterology.

2005;128:280 –7. : Noncompliance with screening advice was rare (in 3% of cases), but significant delays (more than 1 year) in undergoing screening were observed in approximately 25% of the cases.

Pylvanainen K, Kairaluoma M, Mecklin JP. Compliance and satisfaction with long-term surveillance in Finnish HNPCC families.

Fam Cancer. 2006;5:175– 8.: Colonoscopy had to be discontinued because of pain at least once in 10% of the patients.

Ältere Literatur:

27. Mecklin JP, Jarvinen HJ, Aukee S, et al. Screening for colorectal carcinoma in cancer family syndrome kindreds. Scand J Gastroenterol 1987;22:449–453.

28. Fitzgibbons RJ Jr, Lynch HT, Stanislav GV, et al. Recognition and treatment of patients with hereditary nonpolyposis colon cancer (Lynch syndromes I and II). Ann Surg 1987;206:289–295.

29. Lanspa SJ, Jenkins JX, Cavalieri RJ, et al. Surveillance in Lynch syndrome: how aggressive? Am J Gastroenterol 1994;89:1978–1980.

30. Box JC, Rodriguez-Bigas MA, Weber TK, et al. Clinical implications of multiple colorectal carcinomas in hereditary nonpolyposis colorectal Lee JS, Petrelli NJ, Rodriguez-Bigas MA. Rectal cancer in hereditary nonpolyposis colorectal cancer. Am J Surg 2001;181:207–210.


- Rektumkarzinome

1. Rodríguez-Bigas MA, Vasen HF, Pekka-Mecklin J, et al. Rectal cancer risk in hereditary nonpolyposis colorectal cancer after abdominal colectomy. Annals of surgery. 1997;225(2):202-7.

Retrospektiv; 71 Pat. mit subtotaler Kolektomie, 8 Rektumkarzinome nach 158 Monaten (range, 38-282 months). The risk of developing rectal cancer was estimated to be 3% every 3 years after abdominal colectomy for the first 12 years, being approximately 12% after 12 years.

2. Lee JS, Petrelli NJ, Rodriguez-Bigas M a. Rectal cancer in hereditary nonpolyposis colorectal cancer. American journal of surgery. 2001;181(3):207-10.

89 LS/HNPCC-Pat aus registry mit 139 CRC, davon 25 (28%) mit Rektum-CA. Von diesen 18 (72%) mit Rektum-CA als IndexCA, metachrone Kolon-CA in dieser Gruppe 17% nach median 203 Monaten. 7 (28%) dieser 25 mit Rektum-CA als metachrones CA im Median 245 Monate nach Kolon-CA. Männer dtl. Häufiger betroffen als Frauen.

3. Goecke T, Schulmann K, Engel C, et al. Genotype-phenotype comparison of German MLH1 and MSH2 mutation carriers clinically affected with Lynch syndrome: a report by the German HNPCC Consortium. Journal of clinical oncology. 2006;24(26):4285-92.

988 Mutationsträger MLH1 und MSH2; von jeweils 100 Karzinomen insgesamt: 69,8 bzw. 58,9% Kolon, 7,7 bzw 5,9% Rektum

4. Samowitz WS, Curtin K, Wolff RK, et al. Microsatellite instability and survival in rectal cancer. Cancer causes & control : CCC. 2009;20(9):1763-8.

990 Patienten, davon 22 MSI-H. Nur 6 IHC available. Bad prognosis of MSI-H tumors (reason unclear). Im Vgl. zu MSI-H Kolon-CA wenig BRAF- Mutationen, MLH1-Methylierung, CIMP -> The genetic and epigenetic characteristics of MSI-H rectal cancers suggest that they are enriched for Lynch-associated tumors.


- Endometriumkarzinome

folgt in Kürze ...

 


- Ovarialkarzinome

folgt in Kürze ...

 


- Urothelkarzinome

folgt in Kürze ...

 


- Magenkarzinome

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- andere

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Kriterien zum Stellen der Diagnose
HNPCC-Lynch Syndrom