Peutz-Jeghers 71. Peutz-Jeghers Peutz-Jeghers syndrome PJS LKB1/STK11 PJS. Peutz-Jeghers PJS R656 R735 3 A

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1 Chin J Bases Clin General Surg Vol 19 No 5 May 2012 Peutz-Jeghers Peutz-Jeghers PJS PJS / cm PJS Peutz-Jeghers R656 R735 3 A Clinical Comprehensive Therapy of Peutz-Jeghers Syndrome Report of 71 Cases WANG Shi-lin 1 GU Guo-li 1 WEI Xue-ming 1 MAO Gao-ping 2 NING Shou-bin 2 1 Department of General Surgery Air Force General Hospital PLA Beijing China 2 Department of Digestive Disease Air Force General Hospital PLA Beijing China Corresponding Author WANG Shi-lin wangshilin@medmail com cn Abstract Objective To explore the clinical comprehensive therapy of Peutz-Jeghers syndrome Methods From January 2000 to December cases of Peutz-Jeghers syndrome underwent endoscopic polyp resection firstly and those with unresectable lesions or with severe complications underwent rescue laparotomy After endoscopic or surgical treatment the patients took Celecoxib capsules voluntarily for 6 to 9 months under informed consents All cases were followed up from 6 months to 8 years Results Twenty-nine patients had familial history of Peutz-Jeghers syndrome among the 71 patients 41 males and 30 females Sixty-two cases underwent 94 surgeries and intussusception was the most common cause of laparotomy Sixty-five patients underwent 169 double-balloon endoscopy DBE therapies and a total of polyps were resected by DBE polypectomy The largest major axis of small-bowel polyp was 8 cm No severe complications occurred after DBE polypectomy except for 3 cases of intestinal perforation Eight patients took Celecoxib capsule 3 of them were treated more than 6 months and DBE examination showed the gastrointestinal polyps reduced in number and size Conclusion The comprehensive treatment including of endoscopic therapy operation and drug intervention is a safe and effective clinical model to treat Peutz-Jeghers syndrome Key words Peutz-Jeghers syndrome Gastrointestinal tumor Double-balloon endoscopy Surgery Drug intervention Foundation item Clinical Key Project of The Air Force General Hospital, PLA No. KZG Peutz-Jeghers Peutz-Jeghers syndrome KZG wangshilin@medmail com cn 1951 PJS LKB1/STK ,5 PJS

2 Chin J Bases Clin General Surg Vol 19 No 5 May PJS 10 PJS PJS PJS / / cm 2 4 cm cm cm cm PJS PJS DBE Fujinon EN450 P cm DBE 1 0 cm DBE 7 DBE 8 PJS PJS patients DBE DBE examination Fit for endoscopic treatment or not (Yes) Endoscopic treatment Complications or not (Yes) (No) Patients after treatment 6 Take Celecoxib for 6 months Polyp progress obviously DBE Reexamine DBE per year Operation (No) Polyp progress not obviously 1 PJS Figure 1 Procedure of clinical comprehensive therapy of PJS cm

3 Chin J Bases Clin General Surg Vol 19 No 5 May 2012 PJS mg 1 /d 6 9 DBE DBE DBE DBE cm 44 DBE 1 2 DBE 36 h 50 h 2 6 cm DBE PJS 8 PJS PJS cm cm DBE DBE PJS PJS PJS 9 PJS /46 PJS 10 PJS PJS 3 2 PJS PJS DBE PJS

4 Chin J Bases Clin General Surg Vol 19 No 5 May PJS DBE DBE PJS PJS 5/ / χ 2 =0 01 P 0 05 DBE PJS 14,15 DBE 6 PJS -2 COX-2 mtor LKB1 PJS COX PJS COX-2 19,20 PJS 21, PJS mtor mtor Rapamycin PJS COX-2 PJS / PJS PJS 1 PJS DBE 6 9 DBE PJS DBE PJS 3 DBE PJS PJS PJS PJS 4 1 J (2) J (30) Gammon A Jasperson K Kohlmann W et al Hamartomatous polyposis syndromes J Best Pract Res Clin Gastroenterol (2) ( 8 ) J (4) Peutz-Jeghers 36 J (4) J (3) J ( Miura Y Yamamoto H Sunada K et al Reduction of ileoileal intussusception by using double-balloon endoscopy in Peutz- Jeghers syndrome (with video) J Gastrointest Endosc (3) Peutz-Jeghers J (6) J (4) Yoo JH Yoo JH Choi YJ et al A novel de novo mutation in the serine-threonine kinase STK11 gene in a Korean patient with Peutz-Jeghers syndrome J BMC Med Genet de Leng WW Westerman AM Weterman MA et al Nasal polyposis in Peutz-Jeghers syndrome a distinct histopathological and molecular genetic entity J J Clin Pathol (4) 392-

5 Chin J Bases Clin General Surg Vol 19 No 5 May Ross AS Dye C Prachand VN Laparoscopic-assisted doubleballoon enteroscopy for small-bowel polyp surveillance and treatment in patients with Peutz-Jeghers syndrome J Gastrointest Endosc (6) Peutz-Jeghers J (14) Heine GD Al-Toma A Mulder CJ et al Milestone in gastrointestinal endoscopy double-balloon enteroscopy of the small bowel J Scand J Gastroenterol Suppl 2006 (243) Peutz-Jeghers J (4) Rossi DJ Ylikorkala A Korsisaari N et al Induction of cyclooxygenase-2 in a mouse model of Peutz-Jeghers polyposis J Proc Natl Acad Sci USA (19) Wei C Amos CI Rashid A et al Correlation of staining for LKB1 and COX-2 in hamartomatous polyps and carcinomas from patients with Peutz-Jeghers syndrome J J Histochem Cytochem (12) Udd L Katajisto P Rossi DJ et al Suppression of Peutz-Jeghers polyposis by inhibition of cyclooxygenase-2 J Gastroenter- ology (4) Arber N Spicak J Rácz I et al Five-year analysis of the prevention of colorectal sporadic adenomatous polyps trial J Am J Gastroenterol (6) De Leng WW Westerman AM Weterman MA et al Cyclooxygenase 2 expression and molecular alterations in Peutz-Jeghers hamartomas and carcinomas J Clin Cancer Res (8) Kurland JE Beck SE Solomon CJ et al Cyclooxygenase-2 expression in polyps from a patient with juvenile polyposis syndrome with mutant BMPR1A J J Pediatr Gastroenterol Nutr ( Wei C Amos CI Zhang N et al Suppression of Peutz-Jeghers polyposis by targeting mammalian target of rapamycin signaling J Clin Cancer Res (4) Resta N Lauriola L Puca A et al Ganglioglioma arising in a Peutz-Jeghers patient a case report with molecular implications J Acta Neuropathol (1) Inoki K Corradetti MN Guan KL Dysregulation of the TSCmTOR pathway in human disease J Nat Genet (1) UC-CRC Watanabe T Konishi T Kishimoto J UC-CRC UC-CRC UC-CRC UC-CRC P= UC-CRC Inflamm Bowel Dis (3) MCQ

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