502 2012 5 19 5 Chin J Bases Clin General Surg Vol 19 No 5 May 2012 Peutz-Jeghers 71 1 1 1 2 2 Peutz-Jeghers PJS 2000 1 2010 12 71 PJS 6 9 71 41 30 29 40 8 62 94 72 3 68/94 65 169 1 714 8 cm 3 8 6 3 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 100142 China 2 Department of Digestive Disease Air Force General Hospital PLA Beijing 100142 China Corresponding Author WANG Shi-lin E-mail wangshilin@medmail com cn Abstract Objective To explore the clinical comprehensive therapy of Peutz-Jeghers syndrome Methods From January 2000 to December 2010 71 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 1 714 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. KZG2009006 Peutz-Jeghers Peutz-Jeghers syndrome KZG2009006 1 100142 2 100142 E-mail wangshilin@medmail com cn 1951 PJS LKB1/STK11 1-3 4,5 PJS
2012 5 19 5 Chin J Bases Clin General Surg Vol 19 No 5 May 2012 503 PJS 10 PJS 1 1 1 2000 1 2010 12 71 PJS PJS 6 41 30 12 49 23 8 29 40 8 1 2 1 2 1 88 7 63/71 1 25 1 12 46 6 / 65 9 3 1 2 2 51 71 8 8 11 3 8 11 3 4 5 6 71 100 49 69 0 55 77 5 61 85 9 0 5 10 cm 2 4 cm 0 5 1 0 cm 18 1 0 3 0 cm 39 3 0 cm 14 2 2 8 1 1 3 PJS 1 1 3 1 PJS DBE Fujinon EN450 P-5 0 5 1 0 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 1 3 2 5 10 15 cm 1 3 3 3
504 2012 5 19 5 Chin J Bases Clin General Surg Vol 19 No 5 May 2012 PJS 15 200 400 mg 1 /d 6 9 DBE 2 2 1 91 58 126 69 1 1 8 8 DBE 169 65 2 DBE 54 17 DBE 1 714 820 154 261 479 8 cm 44 DBE 1 2 DBE 36 h 50 h 2 6 cm DBE 30 22 2 2 62 94 68 9 5 8 1 2 1 2 1 2 3 PJS 8 PJS PJS 8 1 0 cm 0 5 1 0 cm 3 3 1 2 1 2 2 1 1 6 2 9 1 3 DBE 2 4 3 68 95 8 0 5 8 2 8 1 3 67 67 46 6 1 DBE 22 19 5 3 2 3 3 1 PJS PJS PJS 9 PJS 47 8 22/46 PJS 10 PJS PJS 3 2 PJS PJS 11-13 DBE PJS
2012 5 19 5 Chin J Bases Clin General Surg Vol 19 No 5 May 2012 505 PJS DBE 65 169 DBE PJS PJS 5/65 7 8 4/62 6 5 χ 2 =0 01 P 0 05 DBE PJS 14,15 DBE 6 PJS -2 COX-2 mtor 16 17 LKB1 PJS COX-2 18 82 PJS COX-2 19,20 PJS 21,22 23-25 PJS mtor mtor Rapamycin PJS COX-2 PJS 18-22 5/8 62 5 3 3 PJS PJS 1 PJS DBE 6 9 DBE PJS DBE PJS 3 DBE PJS PJS PJS PJS 4 1 J 2005 12(2) 195-198 2 J 2009 17(30) 3075-3085 3 Gammon A Jasperson K Kohlmann W et al Hamartomatous polyposis syndromes J Best Pract Res Clin Gastroenterol 2009 23(2) 219-231 4 ( 8 ) J 2004 11(4) 356-357 5 Peutz-Jeghers 36 J 2009 12(4) 428 6 J 2004 26(3) 191-192 7 J 2007 1(5 28-31 8 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 2010 72(3) 658-659 9 Peutz-Jeghers J 2006 13(6) 709-711 10 J 2004 10(4) 271-273 11 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 2008 9 44 12 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 2007 60(4) 392-
506 2012 5 19 5 Chin J Bases Clin General Surg Vol 19 No 5 May 2012 396 13 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 2006 64(6) 984-988 14 Peutz-Jeghers J 2008 16(14) 1588-1591 15 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) 32-38 16 Peutz-Jeghers J 2009 16(4) 333-335 17 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 2002 99(19) 12327-12332 18 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 2003 51(12) 1665-1672 19 Udd L Katajisto P Rossi DJ et al Suppression of Peutz-Jeghers polyposis by inhibition of cyclooxygenase-2 J Gastroenter- ology 2004 127(4) 1030-1037 20 Arber N Spicak J Rácz I et al Five-year analysis of the prevention of colorectal sporadic adenomatous polyps trial J Am J Gastroenterol 2011 106(6) 1135-1146 21 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 2003 9(8) 3065-3072 22 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 2007 44(3 318-325 23 Wei C Amos CI Zhang N et al Suppression of Peutz-Jeghers polyposis by targeting mammalian target of rapamycin signaling J Clin Cancer Res 2008 14(4) 1167-1171 24 Resta N Lauriola L Puca A et al Ganglioglioma arising in a Peutz-Jeghers patient a case report with molecular implications J Acta Neuropathol 2006 112(1) 106-111 25 Inoki K Corradetti MN Guan KL Dysregulation of the TSCmTOR pathway in human disease J Nat Genet 2005 37(1) 19-24 2011-10-08 2012-01-27 UC-CRC Watanabe T Konishi T Kishimoto J 1978 1998 108 536 169 UC-CRC 121 1995 1998 UC-CRC UC-CRC UC-CRC 43 3 57 4 P=0 0320 UC-CRC Inflamm Bowel Dis 2011 17(3) 802-808 MCQ 2011-03-21