Peutz-Jeghers Syndrome Associated With Gastric Cancer: Treatment Experience And Literature Review

Objective:To analyze the clinical manifestation, pathogenesis, pathology, diagnosis and treatment of Peutz-Jeghers symdrome associated with gastric cancer. And to share the treatment experience of Peutz-Jeghers syndrome associated with gastric cancer. To improve the quality of life and duration of survival of the patients.Methods: Collect the clinical data from a case of Peutz-Jeghers syndrome with gastric cancer from the first affilited hospital of xiamen university.Analyze the procedure of diagnosis and treatment,review the relevant litetature and make a summary.Results: A 24-year-old female complained perioal and fingertip pigmentation for more than 20 years, and abdominal dull pain for 1 year.There arn't similar family medical history. On physical examination,we found out that there are scattered hyperpigmented macules around her lips and fingertip, and no abnormality was found on superficial lymph nodes.The examnation on abdomen was normal. Gastroscopy showed:1.Gastric antral tumor of unknown origin(MT?); 2. Multiple polyps in gastric and the small intestin. Pathology:High to modetate-grade tubular adenocarcinoma. Abdominal CT scan indicated that multiple polyps in the small intestine and caused enteric intussusceptions and ileocolic intussusceptions. Tumor markers is normal. Preoperative diagnosis:1. Gastric antral carcinoma; 2. Peutz-Jeghers syndrome; 3. Intussusception. Then she had an operation of “Distal gastric cancer radical(D2,Billroth II) + Intussusception reset + intestinal polypectomy ”. Postoperative pathology: moderate-grade tubular adenocarcinoma. Pathological staging: p T3N1M0(Stage:III).There is not reflux and abdominal pain postoperative. The female complained abdominal pain, abdominal swelling, vomiting and constipation for 5 hours 4 years after the operation. Preoperative diagnosis:1.Acute intestinal Obstruction; 2. Peutz-Jeghers syndrome. Then she had an operation again, the operation was prosperous and she had a good recovery.Conclusion: Peutz-Jeghers syndrome is a rare, autosomal dominant inherited disorder characterized by intestinal hamartomatous polyps in association with a distinct pattern of skin and mucosal melanin deposition. Patients with Peutz-Jeghers syndrome have a high increased risk of developing intestinal cancer who sometimes has a family history. The pathogenesis of P-J syndrome include the abnormity of m TOR, Hedgehog and Wnt/?-catenin signal path which caused by the LKBl gene mutation.Then infect thereplication, differentiation, and apoptosis of cells which leads to gastrointestinal polyps and cancer. There is a great agreement on the therapy of P-J syndrome that we should resect the gastrointestinal polyps once we find them which can reduce the rate of complication. Under the condition of few complications and only the gastric cancer needs operation, we appreciate laparoscopic surgery instide open surgery. About the digestive tract reconstruction, we favor BIllroth I procedure when there is a small tension, otherwise, we would choose BIllroth II procedure, the procedure of R-Y is less used in gastric cancer with P-J syndrome. Drugs therapy in P-J syndrome is still in the early stages, we need the efforts of clinical and preclinical medicine to make it a brighter tomorrow.