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    • The status of Fas and Fas ligand expression can

    2019-05-09

    The status of Fas and Fas ligand expression can predict recurrence of hepatocellular carcinoma. These results suggest that the evaluation of Fas and Fas ligand expression potentially has a significant prognostic value for DFS of HCC patients, in addition to patient clinical stage, and can be regarded as a new prognostic marker. Our results suggest that for SNPs of Fas/FasL, there were no significant differences in tumor recurrence within 1 year. The clinical treatment may confound the disease\'s natural process and cause difficulties of statistical significance. In conclusion, the SNPs of Fas/FasL promoters are not correlated with long-time survival in HCV virus-related HCC. Fas polymorphyisms are related to HCV related cirrhosis. FasL polymorphyisms are related to the progression of HCC and may correlate to the serum AFP level in patients with HCC. In future studies, we need to assemble a greater number of cases and attempt to identify the relationship between FasL polymorphyisms and tumor tissue FasL expression.
    Conflict of interest
    Acknowledgments
    Introduction Perivascular epithelioid cell tumor (PEComa), a rare subtype of mesenchymal neoplasm, is composed of perivascular epithelioid leukotriene receptor antagonists that coexpress myogenic and melanocytic markers on immunohistochemical staining. Bonetti et al. initially described these distinctive cells in 1992. Currently, the family of PEComas includes angiomyolipoma (AML), pulmonary lymphangioleiomyomatosis (LAM), clear cell sugar tumor of the lung, extrapulmonary clear cell sugar tumor, and clear cell myomelanocytic tumor. Although rare, PEComas have been reported in a variety of anatomical locations and are considered to be ubiquitous. The clinical behavior of PEComas is highly heterogeneous. Most are benign and are cured by surgical resection, but a minority recurs or metastasizes. Thus, various definitions of the malignant potential of these tumors have been proposed. PEComa patients have been treated with conventional chemotherapy used in other types of sarcoma. However, recognition of aberrantly activated mammalian target of rapamycin (mTOR) pathway in malignant PEComa led to the use of mTOR inhibitors, such as sirolimus, for systemic therapy, and promising anti-tumor effects have been demonstrated. Here, we report a rare case of PEComa arising from the gastrointestinal tract and review the literature regarding this rare subtype of soft tissue sarcoma.
    Case report A 60-year-old woman was in her usual state of health until late 2013 when she started to experience acid regurgitation and post-prandial fullness. Esophagogastroduodenoscopy showed only mild reflux esophagitis. Her symptoms failed to respond to symptomatic treatments, including metoclopramide, antacids, and proton pump inhibitors, and progressed to easy vomiting over two months and experienced unintentional weight loss of 4 kg in 1 week. There was no tarry stool or change in stool caliber. She was examined at a local hospital, and initial examination with abdominal ultrasonography revealed a large intra-abdominal tumor. Computed tomography (CT) of the abdomen showed a 12 × 12 × 9 cm irregular mass in the left abdomen, with heterogeneous enhancement and mass effect on the adjacent structures. No ascites or enlarged lymph nodes were seen on the CT image (Fig. 1A). The results of all her laboratory tests were within the normal range. A diagnosis of gastrointestinal stromal tumor (GIST), leiomyosarcoma, or adenosarcoma was suspected. Surgical resection was suggested, and she was then referred to our hospital for further management. The patient was admitted to our gastrointestinal ward in November 2013, and a follow-up abdominal ultrasonography showed a large heterogeneous and hypoechoic lesion with hypervascularity arising from the small bowel wall over the left upper quadrant area (Fig. 1B). A tumor marker panel showed levels within normal limits, as follows: carcinoembryonic antigen (CEA), 1.25 ng/mL (normal range <5 ng/mL); CA 19-9, 6.34 U/mL (normal range < 37 U/mL); CA 15-3, 6.8 U/mL (normal range 0–31 U/mL); and CA-125, 7.1 U/mL (normal range 0–35 U/mL). A small bowel GIST or leiomyosarcoma was suspected, and a general surgeon was consulted for further resection.