[Mesenchymal chondrosarcoma in central nervous system: a clinicopathological analysis].

Objective: To investigate the clinicopathological features of central nervous system (CNS) mesenchymal chondrosarcoma (MCS). Methods: Nine cases of CNS MCS were collected at the First Affiliated Hospital of Fujian Medical University from September 2010 to September 2020. The clinical,imaging,histopathological and immunohistochemical features were reviewed. NCOA2 gene rearrangement was evaluated by fluorescence in situ hybridization (FISH). Results: There were three male and six female patients, with age range of 1 to 59 years (median 31 years). Six cases were intracranial and three cases were intraspinal, and the tumors showed dural attachment. They were often diagnosed as meningioma basing on preoperative imaging. Microscopically, the tumors showed a characteristic biphasic histologic pattern composed of undifferentiated mesenchymal small cells and well-differentiated hyaline cartilage islands. The small cells area were positive for SOX9 (9/9), CD99 (8/9), and without BRG1 and INI1 deletion. The cartilaginous component expressed SOX9 (9/9) and S-100 protein (8/9). NCOA2 gene break apart signal was identified in five cases (5/5). Eight patients were followed up for 4-124 months. Three patients (3/8) had recurrences within one year and two patients died of the tumor. Conclusions: CNS MCS is an extremely rare malignant neoplasm with a propensity to dural involvement. Preoperative imaging has low diagnostic accuracy. CNS MCS should be differentiated from other CNS small round cell tumors and chondrosarcoma. FISH detection of NCOA2 gene rearrangement will assist the diagnosis of MCS.

CYFRA21-1 levels could be a biomarker for bladder cancer: a meta-analysis.

The proteolytic region of cytokeratin-19, referred to as CYFRA21-1, is a soluble molecule present in the serum and other body fluids, and is considered a tumor marker in several neoplastic diseases. To examine whether urinary or serum samples containing CYFRA21-1 can be used as biomarkers for bladder cancer, we conducted a comprehensive meta-analysis of 3 case-control studies. In all studies considered, patients with bladder cancer had a higher CYFRA21-1 level than healthy subjects. Subgroup analysis showed that patients with metastatic bladder cancer had a higher CYFRA21-1 level than those with locally invasive disease. However, no significant difference in CYFRA21-1 was observed between patients with stage I and stage II bladder cancer; there was also no difference in patients with stage II local bladder cancer and those with stage III local bladder cancer. Based on our results, CYFRA21-1 level may be a diagnostic biomarker for diagnosing bladder cancer as well as a possible biomarker for differentiation between local and metastatic bladder cancer. However, it cannot be used as a urinary or serum biomarker for differentiating histological stages of local bladder cancer for histological grades I-III.

A novel RUNX2 mutation (T420I) in Chinese patients with cleidocranial dysplasia.

Cleidocranial dysplasia (CCD) is an autosomal-dominant heritable skeletal disease caused by heterozygous mutations in the RUNX2 gene. We studied a Chinese family that included three affected individuals with CCD phenotypes; the clinical features of patients with CCD include delayed closure of fontanelles, frontal bossing, dysplasia of clavicles, late tooth eruption, and other skeletal anomalies. X-ray analysis showed aplasia of the clavicles. The RUNX2 gene was studied by PCR and direct sequencing of the entire coding region and the exon-intron boundaries of the gene. A novel missense mutation (c.1259C-->T[p.T420I]) in RUNX2 gene exon 7 was identified; it was found in the affected individuals in this Chinese family, but was not present in an unaffected family member or in 100 unrelated normal controls. This is the first report that gives evidence that the T420I mutation of RUNX2 is associated with CCD, expanding the spectrum of RUNX2 mutations causing CCD.

Antithrombin reduces mesenteric venular leukocyte interactions and small intestine injury in endotoxemic rats.

We examined the hypothesis that recombinant human antithrombin would reduce mesenteric venule leukocyte adhesion and small intestine injury in endotoxemic rats. Endotoxemic (endotoxin 10 mg/kg, intravenously) rats were treated either with saline or recombinant human antithrombin (250 and 500 U/kg). In some rats, indomethacin (100 mg/kg, intraperitoneally) was injected 60 min prior to endotoxin and recominant human antithrombin (500 U/kg) treatment. Compared to controls, intravital videomicroscopy of the mesentric venule showed an increase of leukocyte rolling (55+/-17 versus 70+/-19 leukocytes/min; P < 0.05) and firm adhesion (1.1+/-0.3 versus 5.8+/-0.8 leukocytes/100 microm; P < 0.05) in endotoxemic rats. Recombinant human antithrombin attenuated endotoxin-induced venular endothelium leukocyte adhesive cascade. The beneficial effects of recombinant human antithrombin on leukocyte adhesion were inhibited by indomethacin (100 mg/kg, intraperitoneally) in endotoxemic rats. Endotoxin treatment increased fluorescein isothiocyanate (FITC)-labeled dextran 4,000 (FD4) gut lumen to plasma ratio and wet weight/dry weight ratio. Recombinant human antithrombin (500 U/kg) attenuated endotoxin-induced gut injury. These observations suggest that recombinant human antithrombin reduces endothelium-leukocyte interactions in endotoxemic rats by interacting with local prostacyclin production.

[Surgical treatment of congenital heart defect complicating severe pulmonary infection in infants and young children].

OBJECTIVE: To review the experience of surgical management of left to right shunt congenital heart defect complicating severe pulmonary infection. METHOD: Twenty patients aged 4-17 months who weighed 3.6-11.6 kg were analysed. Among them, 16 underwent surgical treatment. RESULTS: The mortality rate of 4 who received conservative therapy was 75% (3/4). The mortality rate of the operative group 16 Cases, was 12. 5% (2/16), 14 were doing well by follow-up of 8-24 months. CONCLUSION: Infants of Congenital heart defect Complicating Severe Pulmonary infection, Should be performed operation actively.

[Perioperative observation of plasma endothelin in patients with congenital heart disease with pulmonary hypertension underwent open heart surgery].

UNLABELLED: To elucidate the correlation between endothelin(ET) and pulmonary hypertension, plasma ET was measured perioperatively by radioimmunoassay in 22 patients with pulmonary hypertension who underwent open heart surgery(PH group) in comparison with 16 patients without pulmonary hypertension(non-PH group) also underwent cardiopulmonary bypass(CPB) surgery. RESULTS: Plasma ET was significantly increased during CPB in both group, Plasma ET decreased rapisly and returned to preoperative level within 6 hours after operation in the non-PH group. In the PH group, however, plasma ET decreased slowly and did not return to preoperative level within 48 hours postoperation.

[Preliminary study of therapeutical mechanism on chyluria by heat-cleaning and hemostasis drugs].

Ten cases of filarial chyluria were treated with Chinese medical herbs. The results showed that the average concentration of serum histamine of 142 ng/ml before treatment dropped to 93 ng/ml after treatment. The average lymph-vessel pressure of 9.93 cmH2O in dorsalis pedis dropped to 4.74 cmH2O after cure. The prevalent microcirculation obstacle of medium to serious grade also was improved or recovered. The average serum viscosity of 1,884 before treatment dropped to 1,725. These results indicated that the chronic lymphatic inflammation occurring in pelvis or behind peritoneum due to perennial stimulation of adult filariae, caused the high concentration of inflammatory medium tissues, the expansion of micro-vessels and the increase of lymph-vessel pressure. Therefore, the microcirculation obstacle was the principle pathogeny of filarial chyluria.