OBJECTIVETo study the clinicopathologic characteristics of extrarenal malignant rhabdoid tumor (E-MRT) with emphasis on diagnosis and differential diagnosis.

METHODSThe clinical and pathologic data of 8 E-MRT cases were reviewed. The outcome was analyzed.

RESULTSThere were four males and four females. The age at presentation ranged from 3 days to 8 years (mean, 2.6 years; median, 3 years). The tumors were located in the extremities (n = 1), head and neck (n = 2), trunk (n = 2), cervical cord (n = 1), liver (n = 1) and retroperitoneum (n = 1). Histologically, the tumors were composed of a diffuse proliferation of rounded or polygonal cells with eccentric nuclei, prominent nucleoli, and glassy eosinophilic cytoplasm containing hyaline-like inclusion bodies, arranged in sheets and nests. Cellular atypia was easily observed and mitotic activity was high. Necrotic and hemorrhagic areas were abundant. On immunohistochemistry, the tumor cells expressed vimentin and epithelial marker such as EMA, AE1/AE3, and CAM5.2. The absence of INI1 protein expression was a distinctive feature. Follow-up of all eight cases revealed five deaths in one year and the other three were disease-free at last follow-up of one month, three months and seven months.

CONCLUSIONSE-MRT is a rare and highly aggressive tumor of infancy and childhood. Recurrence and distant metastasis was common and the 5-year survival rate is low. Increased awareness of the clinocopathologic features and immunophenotypes of E-MRT is helpful for correct diagnosis and effective treatment.

Child ; Child, Preschool ; Diagnosis, Differential ; Extremities ; Female ; Head and Neck Neoplasms ; metabolism ; pathology ; Humans ; Immunohistochemistry ; Infant ; Infant, Newborn ; Male ; Neoplasm Recurrence, Local ; Retroperitoneal Neoplasms ; metabolism ; pathology ; Rhabdoid Tumor ; metabolism ; pathology ; Treatment Outcome ; Vimentin ; metabolism

Background: Fucosyltransferase 2 (FUT2) and FUT3 specifically catalyze the biosynthesis of human histo-blood group antigens, and has been demonstrated to be closely related to inflammatory bowel disease (IBD). However, there are few clinical studies focusing on FUT2, FUT3 and IBD. Aims: To investigate the expressions of FUT2 and FUT3 in intestinal mucosa of IBD patients with various clinical characteristics and their clinical relevance. Methods: Patients initially diagnosed as active IBD in the Affiliated Hospital of Hangzhou Normal University from January 2019 to December 2020 were recruited consecutively. Endoscopic biopsy specimens prior to the initiation of treatment were collected to assess the expressions of FUT2 and FUT3 immunohistochemically. Correlations of FUT2, FUT3 with the clinical characteristics and inflammatory indicators were analyzed. Results: Seventy cases of ulcerative colitis (UC), 37 Crohn's disease (CD), and 66 healthy subjects were enrolled. The positivity rate and relative expression level of FUT2 were decreased, while those of FUT3 were increased in CD group than in control group (all P<0.05). In UC group, the relative expression levels of both FUT2 and FUT3 were increased as compared with control group (all P<0.05). No correlations were observed between expressions of FUT2, FUT3 and the disease severity, disease extent/location, clinical manifestations (including abdominal pain, diarrhea, fever and anemia), and alcohol drinking and cigarette smoking in IBD patients (all P>0.05). But moderate positive correlations were found between FUT3 expression and the serum C-reactive protein and fecal calprotectin (r=0.259, P=0.007; r=0.388, P=0.001). Conclusions: FUT2 and FUT3 might be used as indicators for the assistant diagnosis of IBD and assessment of drug therapy response.

Objective: To preliminary, explore the effect of small intestinal epithelial dendritic cells on the occurrence and development of nonalcoholic fatty liver disease in mice. Methods: Thirty-two (half male and half female) 4-week-old C57BL/6 mice were randomly divided into two groups. The mice were fed with normal diet (SD group) and high-fat diet (HFD group). Eight mice (half male and half female) were randomly killed from each group over the 14 and 20-weeks feeding period to observe their body weight, liver and small-intestine wet weight. Alanine aminotransferase, aspartate aminotransferase, blood glucose, high-density lipoprotein, low-density lipoprotein, total cholesterol and triglyceride were determined by eyeball blood samples. Pathological diagnosis and alcoholic fatty liver disease activity score were collected. The number of mice small intestinal dendritic cells was observed under a microscope. Statistical analysis was performed to compare two groups of independent samples with homogeneity test of variance, t test, and covariance analysis. Results: The body weight, liver wet weight, serum alanine aminotransferase and aspartate aminotransferase of mice in HFD group were significantly higher than those of control group at 20 weeks (P < 0.05), and the serum high density lipoprotein of mice in HFD group was significantly higher than that of SD group at 14 weeks (P < 0.05). At 14th weeks, the liver tissue of mice in HFD group had early pathological manifestations of hepatitis (fatty degeneration, punctate necrosis and balloon-like degeneration). Of which 87.5% (7/8) of them were diagnosed as non-alcoholic steatohepatitis or non-alcoholic fatty liver disease, while only a few mice in SD group had early pathological manifestations of hepatitis. At 20th weeks, all mice in HFD group were diagnosed with non-alcoholic steatohepatitis or non-alcoholic fatty liver disease, while none of the mice in SD group was diagnosed with non-alcoholic fatty liver disease. At both time points, the percentage of small intestinal dendritic cells in HFD group was significantly higher than that in SD group (14 weeks: 4.181 ± 4.314 vs. 15.099 ± 10.349; 20 weeks: 9.615 ± 8.267 vs. 32.839 ± 24.475, both P < 0.05). Statistical analysis combined with the alcoholic fatty liver disease activity score showed that there was no linear correlation between the two groups (regression coefficient was 20.196%). Conclusion The number and different staging of small intestinal dendritic cells in mice is associated with the occurrence and development of NAFLD.