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International Journal of Colorectal Disease
Published in: International Journal of Colorectal Disease 4/2020

01-04-2020 | Thyroid Sonography | Original Article

Long-term clinical outcomes and follow-up status in Japanese patients with familial adenomatous polyposis after radical surgery: a descriptive, retrospective cohort study from a single institute

Published in: International Journal of Colorectal Disease | Issue 4/2020

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Abstract

Purpose

Data on long-term outcomes of familial adenomatous polyposis (FAP) are unclear in Japan because a nationwide registry system is lacking. We assessed overall survival, incidence of neoplasms, fecal incontinence, and postoperative follow-up status of patients with FAP treated surgically in our hospital.

Methods

In total, 154 patients with FAP who underwent radical surgery from 1981 to 2017 in our department were available for the questionnaire. Sixty-five patients, 36 of whom were followed at our hospital, were assessed using clinical records and the questionnaire.

Results

The median follow-up time was 187 months (interquartile range, 93.5–296 months). The median age at surgery was 36 years (range, 12–69 years). The 5-, 10-, 15-, and 20-year overall survival rate was 100%, 98%, 95%, and 89%, respectively. All five deaths were caused by diseases other than colorectal cancer. FAP-related neoplasms comprised 23 colorectal cancers, five duodenal cancers, three gastric cancers, five thyroid cancers, two ileal pouch cancers, and nine desmoid tumors. The incidence of desmoid tumors was significantly associated with the operation date. The duration from radical surgery to neoplasm onset significantly differed by neoplasm type. Forty-five of 54 patients (excluding those who died or underwent ileostomy) developed fecal incontinence (median Wexner score of 8). Surgical procedures involving hand-sewn sutures with rectal mucosal stripping were significantly associated with fecal incontinence and the Wexner score. Fifty-eight of the 60 surviving patients underwent follow-up examinations.

Conclusion

Overall survival was favorable. Fecal incontinence depended on the surgical procedures. Most patients continued to receive follow-up examinations.

Trial registration

No. 3112 by Institutional Review Board of Hyogo College of Medicine
Appendix
Available only for authorised users
Literature
1.
Nishisho I, Nakamura Y, Miyoshi Y et al (1991) Mutations of chromosome 5q21 genes in FAP and colorectal cancer patients. Science 253:665–669CrossRef
2.
Kinzler KW, Nilbert MC, Su LK, Vogelstein B, Bryan TM, Levy DB, Smith KJ, Preisinger AC, Hedge P, McKechnie D (1991) Identification of FAP locus genes from chromosome 5q21. Science 253:661–665CrossRef
3.
Groden J, Thliveris A, Samowitz W, Carlson M, Gelbert L, Albertsen H, Joslyn G, Stevens J, Spirio L, Robertson M (1991) Identification and characterization of the familial adenomatous polyposis coli gene. Cell 66:589–600CrossRef
4.
Burt RW, Leppert MF, Slattery ML, Samowitz WS, Spirio LN, Kerber RA, Kuwada SK, Neklason DW, Disario JA, Lyon E, Hughes JP, Chey WY, White RL (2004) Genetic testing and phenotype in a large kindred with attenuated familial adenomatous polyposis. Gastroenterology. 127:444–451CrossRef
5.
Petersen GM, Slack J, Nakamura Y (1991) Screening guidelines and premorbid diagnosis of familial adenomatous polyposis using linkage. Gastroenterology 100:1658–1664CrossRef
6.
Stoffel EM, Mangu PB, Gruber SB et al (2015) American Society of Clinical Oncology; European Society of Clinical Oncology. Hereditary colorectal cancer syndromes: American Society of Clinical Oncology Clinical Practice Guideline endorsement of the familial risk-colorectal cancer: European Society for Medical Oncology Clinical Practice Guidelines. J Clin Oncol 33:209–217CrossRef
7.
Syngal S, Brand RE, Church JM, American College of Gastroenterology et al (2015) ACG clinical guideline: genetic testing and management of hereditary gastrointestinal cancer syndromes. Am J Gastroenterol 110:223–262CrossRef
8.
Utsunomiya J, Iwama T, Imajo M et al (1980) Total colectomy, mucosal proctectomy, and ileoanal anastomosis. Dis Colon Rectum 23:459–466CrossRef
9.
Fujita S, Kusunoki M, Shoji Y, Owada T, Utsunomiya J (1992) Quality of life after total proctocolectomy and ileal J-pouch-anal anastomosis. Dis Colon Rectum 35:1030–1039CrossRef
10.
Utsunomiya J (2019) My life full of “serendipity” for the J-pouch. Dis Colon Rectum 62:650–658CrossRef
11.
Ganschow P, Pfeiffer U, Hinz U, Leowardi C, Herfarth C, Kadmon M (2010) Quality of life ten and more years after restorative proctocolectomy for patients with familial adenomatous polyposis coli. Dis Colon Rectum 53:1381–1387CrossRef
12.
Anderson T, Lunde OC, Johnson E, Moum T, Nesbakken A (2011) Long-term functional outcome and quality of life after restorative proctocolectomy with ileo-anal anastomosis for colitis. Color Dis 13:431–437CrossRef
13.
Barrow P, Khan M, Lalloo F, Evans DG, Hill J (2013) 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 100:1719–1731CrossRef
14.
Iwama T, Tamura K, Morita T et al (2004) A clinical overview of familial adenomatous polyposis derived from the database of the polyposis registry of Japan. Int J Clin Oncol 9:308–316CrossRef
15.
Ueno H, Kobayashi H, Konishi T et al (2016) Prevalence of laparoscopic surgical treatment and its clinical outcomes in patients with familial adenomatous polyposis in Japan. Int J Clin Oncol 21:713–722CrossRef
16.
Yamano T, Hamanaka M, Babaya A et al (2017) Management strategies in lynch syndrome and familial adenomatous polyposis: a national healthcare survey in Japan. Cancer Sci 108:243–249CrossRef
17.
Yamadera M, Ueno H, Kobayashi H, Konishi T, Ishida F, Yamaguchi T, Hinoi T, Inoue Y, Kanemitsu Y, Tomita N, Ishida H, Sugihara K (2017) Current status of prophylactic surgical treatment for familial adenomatous polyposis in Japan. Surg Today 47:690–696CrossRef
18.
Tanaka M, Kanemitsu Y, Ueno H, Kobayashi H, Konishi T, Ishida F, Yamaguchi T, Hinoi T, Inoue Y, Tomita N, Ishida H, Sugihara K (2017) Prognostic impact of hospital volume on familial adenomatous polyposis: a nationwide multicenter study. Int J Color Dis 32:1489–1498CrossRef
19.
Yamaguchi T, Ishida H, Ueno H, Kobayashi H, Hinoi T, Inoue Y, Ishida F, Kanemitsu Y, Konishi T, Tomita N, Matsubara N, Watanabe T, Sugihara K (2016) Upper gastrointestinal tumours in Japanese familial adenomatous polyposis patients. Jpn J Clin Oncol 46:310–315CrossRef
20.
Inoue Y, Ishida H, Ueno H, Kobayashi H, Yamaguchi T, Konishi T, Tomita N, Matsubara N, Ishida F, Hinoi T, Kanemitsu Y, Watanabe T, Sugihara K (2017) The treatment of desmoid tumors associated with familial adenomatous polyposis: the results of a Japanese multicenter observational study. Surg Today 47:1259–1267CrossRef
21.
Sada H, Hinoi T, Ueno H et al (2019) Prevalence of and risk factors for thyroid carcinoma in patients with familial adenomatous polyposis: results of a multicenter study in Japan and a systematic review. Surg Today 49:72–81CrossRef
22.
Ishida H, Yamaguchi T, Tanakaya K et al (2018) Japanese Society for Cancer of the Colon and Rectum (JSCCR) guidelines 2016 for the clinical practice of hereditary colorectal cancer (translated version). J Anus Rectum Colon 2(Suppl I):S1–S51 (http://​journal-arc.​jp/​pdf/​002s10001.​pdf). Accessed 5 Feb 2020
23.
Jorge JM, Wexner SD (1993) Etiology and management of fecal incontinence. Dis Colon Rectum 36:77–97CrossRef
24.
Schneider R, Schneider C, Dalchow A, Jakobeit C, Möslein G (2015) Prophylactic surgery in familial adenomatous polyposis (FAP)--a single surgeon’s short- and long-term experience with hand-assisted proctocolectomy and smaller J-pouches. Int J Color Dis 30:1109–1115CrossRef
25.
Günther K, Braunrieder G, Bittorf BR, Hohenberger W, Matzel KE (2003) Patients with familial adenomatous polyposis experience better bowel function and quality of life after ileorectal anastomosis than after ileoanal pouch. Color Dis 5:38–44CrossRef
26.
Fazio VW, Kiran RP, Remzi FH, Coffey JC, Heneghan HM, Kirat HT, Manilich E, Shen B, Martin ST (2013) Ileal pouch anal anastomosis: analysis of outcome and quality of life in 3707 patients. Ann Surg 257:679–685CrossRef
27.
Campos FG, Martinez CAR, Sulbaran M, Bustamante-Lopez LA, Safatle-Ribeiro AV (2019) Upper gastrointestinal neoplasia in familial adenomatous polyposis: prevalence, endoscopic features and management. J Gastrointest Oncol 10:734–744CrossRef
28.
Steinhagen E, Hui VW, Levy RA et al (2014) Results of a prospective thyroid ultrasound screening program in adenomatous polyposis patients. Am J Surg 208:764–769CrossRef
29.
Monachese M, Mankaney G, Lopez R, O'Malley M, Laguardia L, Kalady MF, Church J, Shin J, Burke CA (2019) Outcome of thyroid ultrasound screening in FAP patients with a normal baseline exam. Familial Cancer 18:75–82CrossRef
30.
Tajika M, Tanaka T, Ishihara M, Hirayama Y, Oonishi S, Mizuno N, Kuwahara T, Okuno N, Matsumoto S, Ooshiro T, Kinoshita T, Komori K, Bhatia V, Hara K, Yatabe Y, Niwa Y (2019) Long-term outcomes of metachronous neoplasms in the ileal pouch and rectum after surgical treatment in patients with familial adenomatous polyposis. Endosc Int Open 7:E691–E698CrossRef
31.
Konishi T, Ishida H, Ueno H et al (2017) Postoperative complications after stapled and hand-sewn ileal pouch-anal anastomosis for familial adenomatous polyposis: a multicenter study. Ann Gastroenterol Surg 1:143–149CrossRef
32.
Campos FG, Martinez CA, Novaes M, Nahas SC, Cecconello I (2015) Desmoid tumors: clinical features and outcome of an unpredictable and challenging manifestation of familial adenomatous polyposis. Familial Cancer 14:211–219CrossRef
Metadata
Title
Long-term clinical outcomes and follow-up status in Japanese patients with familial adenomatous polyposis after radical surgery: a descriptive, retrospective cohort study from a single institute
Publication date
01-04-2020

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