Objective To investigate the medication advancement of gastrointestinal polyposis in patients with Peutz-Jeghers syndrome (PJS). Methods Literatures about the medication advancement on gastrointestinal polyposis of PJS were reviewed and analyzed. The recent development of targeting drugs, especially the data of cyclooxygenase-2 selective inhibitors and rapamycin, were emphatically summarized. Results With the deep investigation of PJS and application of selective drugs, the medication of gastrointestinal polyposis in cases of PJS has got more advancement. The extensive use of synthetic cyclooxygenase-2 inhibitors and rapamycin in clinic developed a new way to treat gastrointestinal polyposis of PJS. Conclusion The cyclooxygenase-2 selective inhibitors and rapamycin have the following features: noninvasive, high selectivity and good curative effects. They have splendid prospects in the clinical treatment of gastrointestinal polyposis in patients with PJS and are bring the treatment of gastrointestinal polyposis in cases of PJS into a targeting therapy phase.
【Abstract】ObjectiveTo review recent studies on Muir-Torre syndrome (MTS) and to improve the knowledge about MTS.MethodsThe literatures in recent years on clinic and gene research of MTS were reviewed.ResultsMTS was is a rare autosomal-dominant disorder characterized by the predisposition to both sebaceous tumors (or multiple keratoacanthomas) and internal malignancies. Gastrointestinal cancers were the most common kind of internal malignancies in MTS patients(61%)，followed by genitourinary cancers(22%). In most cases(56%)，sebaceous tumors appeared after the emergence of internal maliganancy. Both hereditary nonpolyposis colorectal cancer(HNPCC) and MTS were caused by germline mutations in the DNA mismatch repair genes. MTS patients exhibit significantly more mutations in the hMSH2 than in the hMLH1. In these cases , both internal and skin tumors showed the characteristic of high microsatellite instability(MSI).ConclusionThe presence of sebaceous tumors(or multiple keratoacanthomas) necessitates the search for internal malignancies. It is mandatory that patients with MTS, as patients with HNPCC, should be regularly followed up to search new malignancies. Evaluation and monitoring of the family members of patients are also necessary. The patients and their families should be counseled for genetic test. Sequencing the hMSH2 gene should be the prior selection of further examinations when clinical manifestations, history and laboratory tests suggest MTS.
Objective To analyze and screen the risk factors of both immunohistochemistry and pathology for lung cancer lymphatic metastasis, and to build a mathematical model for preliminary evaluation. Methods By conducting retrospective studies, the information of lung cancer patients in the General Hospital of Air Force from 2009 to 2011 were collected. Both single and multiple unconditional logistic regression analyses were applied to screen total 27 possible factors for lymphatic metastasis. After the factors with statistical significance were selected, the relevant mathematical model was built and then evaluated by means of receiver operating characteristic (ROC) analysis. Results A total of 216 patients were included. The single analyses on 27 possible factors showed significant differences in the following 10 factors: pathological grade (P=0.00), age (P=0.00), tumor types (P=0.01), nm23 (P=0.00), GSTII (P=0.01), TTF1 (P=0.01), MRP (P=0.01), CK14 (P=0.02), CD56 (P=0.02), and EGFR (P=0.03). The multiple factors unconditional logistic regression analyses on those 10 risk factors screened 4 relevant factors as follows: pathological grade (OR=2.34), age (OR=1.02), nm23 (OR=1.66), and EGFR (OR=1.47). Then a mathematical diagnostic model was established based on those 4 identified risk factors, and the result of ROC analysis showed it could improve the diagnostic sensitivity and specificity compared with the single factor mathematical diagnostic model. Conclusion Pathological grade, age, nm23, and EGFR are related with lung cancer lymphatic metastasis, and all of them are the risk factors which have higher adjuvant diagnostic value for lung cancer lymphatic metastasis.
Objective To investigate the clinicopathological features and clinical subtypes of Peutz-Jeghers syndrome (PJS) in Chinese cases. Methods The clinical and pathological data of 295 patients with PJS who were treated in Air Force General Hospital from Nov. 1994 to Aug. 2017 were retrospectively analyzed and a multifactor statistical study was carried out on. Results Two hundreds and ninety-five patients with PJS belonged to 7 nationalities and came from 26 provinces and urban areas. 99.0% (292/295) of the patients had black spots on the lip and buccal mucosa, and the median occurrence time was 2 years old (0–33 years). The median age of inital diagnosis and treatment was 15 years old (1–45 years). The median interval time between the occurrence of black spots and abdominal symptoms was about 10 years (0–45 years). PJS hamartoma polyps were found in alimentary canals of 293 patients (99.3%), and 96.9% distributed in the duodenum and small intestine (n=284), 90.4% distributed in the colorectal (n=265), 79.9% distributed in the stomach (n=234). Patients of black spot appearing at age <3 years and (or) initial treatment at age <14 years were classified as early-onset subtype, otherwise they could be included in delayed-onset subtype. Conclusions The clinical features of PJS are prominent and the harm of PJS hamartoma polyps is serious. The black spots on the lip and buccal mucosa can be used as an early warning signal to divide the PJS patients into 2 clinical subtypes, which should be differentiated in clinical therapy and follow-up strategy.