3 Cases Report Of Peutz-Jeghers Syndrome And Literature Review

Objective:We aim to summarize the main clinical features, diagnosis and treatment of Peutz-Jeghers syndrome in order to deepen our understanding of the clinical features of PJS, reduce the rate of misdiagnosis and delayed diagnosis, to help regularly monitor cancer, have a clinical follow-up, and improve clinical management of PJS.Methods:Retrieve PJS patients in the Second Hospital of Shanxi Medical University from January 2005 to December 2015, we obtained three cases to analyze. Search domestic database from January 2005 to December 2015, remove duplicate cases reported information and reviews, get reported PJS 110 cases, and summarize the relevant literature review.Results:Results of our hospital: All 3 PJS patients had mucocutaneous hyperpigmentation spots and gastrointestinal polyps. The main clinical manifestations were abdominal pain,nausea, vomiting, diarrhea, hyperpigmentation spots, one patients with acute pancreatitis,one patient was complicated by intestinal obstruction, associated with a thyroid tumor,cervical cancer, and there are one patient with family history in three patients and two patients had adenomatous polyps, 1 case had hamartoma polyp. Two cases of patients had previous surgery, 2 patients had repeated endoscopic polypectomy. 3 cases were improved and discharged.Literature results: 1. The age, gender and family history: The average age of 110 PJS patients is(23.04±12.21) years, range from 4 to 67 years old, there are 56 males patientand 54 cases of female patients, The male to female ratio was 1.03: 1. There are 59 cases(53.6%) with a family history in all 110 patients.2. Pigmentation site: the most common site for the pigmentation was lips, palms and the terminal of fingers, paws and toes, buccal mucosa.In addition, facial, nose, eyelids,perineum and palate also can found.3. Clinical manifestations: The main clinical manifestations according to frequency were abdominal pain, nausea, vomiting, black stools, bloating, pigmentation spots, anus tumor prolapse and so on.4. Complications: Major complications of PJS was intussusception, intestinal obstruc- tion, intestinal necrosis.5. Polyps were most common in the small intestine(71.8%), followed by the large intestine(66.4%) and the stomach(36.4%).6. Pathological findings: the common polyps pathological type was hamartoma polyps and adenomatous polyps, as well as a small amount of inflammatory polyps, hyperplastic polyps.7. Cancer: there are 22 cases of cancer patients, the cancer rate is 20.0%, 10 cases of small intestine, 5 cases of colorectal cancer, 3 cases of cervical cancer, 3 cases of ovarian cancer, one case of liver cell cancer.8. Treatment: surgery is more common, 76 cases have a laparotomy or surgical treatment, 48 cases have the endoscopic resection of polyps, there are 24 cases of joint use of endoscopy and surgery.Conclusions:1. The clinical manifestation of Peutz-Jeghers syndrome vary from all kinds. For abdominal pain, nausea, vomiting, melena as the main performance, it should ask a family history, be observed skin and mucosa and conduct examination of the gastrointestinal tract.2. PJS can cause pancreatitis, complicated by intestinal obstruction, intussusception,and easily merge gastrointestinal and parenteral tumors, requiring regular follow-up and anti-cancer screening,be careful of the female genital system neoplasms screening.3. PJS patients should pay attention to check the small intestine polyps.