Report the clinical data of a patient with hereditary leiomyomatosis and renal cell carcinoma syndrome, and review the literature.

IgG4 related diseases involve numerous organs, involve a wide range of clinical manifestations, and have complex medical departments. The misdiagnosis and missed diagnosis rates are both high, posing great difficulties for diagnosis and differential diagnosis. The article analyzes and summarizes the characteristics, diagnostic criteria, and differential diagnosis of different organ lesions in IgG4 related diseases, in order to help clinical and pathological doctors fully understand and master the characteristics of IgG4 related diseases, improve diagnostic alertness and accuracy, and reduce misdiagnosis and missed diagnosis rates.

Keyword：
IgG4-related diseases; Clinical features; Diagnosis; Differential diagnosis;

Tumor necrosis factor receptor associated cycle syndrome(TRAPS) is an autosomal dominant autoimmune inflammatory response syndrome characterized by long-term recurrent fever, abdominal pain and/or chest pain, Arthralgia, myalgia and erythema. The pathogenesis of this disease may be related to mutations in the TNFRSF1A gene encoding the tumor necrosis factor receptor 1(TNFR1) receptor. Renal Amyloidosis(AA) is an important cause of incidence rate and mortality of TRAPS. This article summarizes and analyzes the clinical data of several Chinese patients with TRAPS, and combines relevant literature to review the progress in the diagnosis, pathogenesis, and treatment selection of TRAPS.

Familial adenomatous polyposis is a Autosome dominant genetic disease, which is characterized by hundreds of adenomatous polyps in the colorectal. If not treated, the risk of lifelong development to colorectal cancer is as high as 100%. At present, the main treatment method of the disease is to regularly monitor and remove large polyps under endoscope and perform preventive surgery to remove the diseased intestinal segment. Postoperative pouch inflammation, anastomotic stenosis, secondary rigid Fibroma, etc., will seriously affect the quality of life of patients. Chemoprevention or delaying the progress of the disease can be used as an important treatment method. Some studies have shown that NSAIDs, eicosapentaenoic acid, Curcumin, Metformin, etc. may become promising chemopreventive agents. This article reviews the research progress in chemoprevention of familial adenomatous polyposis in recent years.

The status of lymph nodes is an independent influencing factor for the prognosis of early gastric cancer patients. Accurately determining the status of lymph nodes before surgery is of great clinical significance for early gastric cancer patients to choose endoscopic resection or radical surgery as a more appropriate treatment method, and to avoid insufficient or excessive treatment. In recent years, there have been many studies on the judgment of lymph node metastasis in early gastric cancer, such as imaging examination represented by EUS and CT, Sentinel lymph node biopsy tracer technology, molecular markers of surrounding tissues, the ratio of tumor markers in peripheral blood and inflammatory response related indicators. This article reviews the current methods of judging lymph node metastasis in early gastric cancer.