Clinicopathologic analysis of Ewing-like BCOR-CCNB3 undifferentiated sarcoma / 中华病理学杂志

Objective@#To investigate the morphologic, immunohistochemical, genetic, clinical features and prognosis of Ewing-like BCOR-CCNB3 gene fusion undifferentiated sarcoma (BCOR-CCNB3 fusion sarcoma).@*Method@#Seventeen Ewing-like sarcoma cases were screened for CCNB3 expression and BCOR-CCNB3 fusion transcripts by immunohistochemistry and RT-PCR among 260 cases of Ewing-like sarcomas collected during Jan, 2006 to Dec, 2015. Three cases of BCOR-CCNB3 fusion sarcoma were found among 17 atypical Ewing sarcomas, and follow-up were conducted.@*Results@#The harboring of BCOR-CCNB3 fusion transcript was confirmed by RT-PCR and directly sequencing results. The three patients aged between 8 and 11 years old. Two of them were male and the other one was female. One patient achieved a complete response after chemotherapy, the other two died without chemotherapy after surgical excision in 12 months. Tumor cells in all 3 cases showed diffuse nuclear CCNB3, TLE1 and cyclin D1 positivity, while CCNB3 (0/12), TLE1 (1/12) and cyclin D1 (4/12) positivity was infrequent in the 12 cases of classical Ewing′s sarcoma. The oval or plump spindle tumor cells with fine chromatin arranged in solid pattern, the nucleoli was inconspicuous. The delicate capillary networks were obvious in the tumor.@*Conclusion@#With a detailed description of the histological spectrum, immunohistochemical features and clinical characteristic of BCOR-CCNB3 sarcoma, justify distinction from Ewing sarcoma could be possible.

Progress in Langerhans-cell histiocytosis / 白血病·淋巴瘤

Langerhans-cell histiocytosis (LCH), the most common histiocytic disorder, is a reactive clonal proliferation and accumulation of CD1a+ / CD207+ dendritic cells in inflammatory lesion, characterized by its strong heterogeneity and changeable complexity including apparent inflammation and tumor features, which should be redefined as an inflammatory myeloid neoplasia. Recent research has shown that LCH is the consequence of misguided myeloid differentiation on account of genomic aberrances in the RAS-RAF-MEK-ERK pathway. These gene inhibitors may present more curative effects for the treatment of LCH. With further prospective clinical trial, molecular targeted therapy may combine with or even replace the traditional surgery plus chemotherapy as the first-line regimen in LCH.

ID3, TCF3 and MYC genes mutation analyses in Chinese Burkitt lymphoma / 临床与实验病理学杂志

Purpose To investigate the mutations of ID3,TCF3 and MYC genes in Chinese Burkitt lymphoma and discuss their significance.Methods Total DNA was extracted from tumor tissues of 32 patients with Burkitt lymphoma,then the DNA was amplified by polymerase chain reaction (PCR),and the products of PCR were sequenced directly with Sanger sequencing methods.Results The mutation rates of ID3 and TCF3 genes were 35.5％ (11/31) and 18.8％ (6/32) respectively.The mutation rate of MYC was 50％.The mutation rates of MYC exon 1,MYC exon 2 and MYC exon 3 were 3.3％ (1/30),50％ (15/30) and 7.7％ (2/26) respectively.Conclusion Recurrent mutations of the ID3,TCF3 and MYC genes in Chinese Burkitt lymphoma were identified by Sanger sequencing.For TCF3 gene,a novel mutation c.2202G ＞ C p.L569V was found in three cases.In two cases,a novel mutation of c.1070A ＞G p.G182D was found in MYC gene.

Spontaneous senescence in malignant tumor cell lines / 肿瘤

Objective:To explore whether spontaneous sene-scence widely existed in malignant tumor cells. Methods:Sene-scence-associated beta-galactosidase (SA-β-Gal) staining kit was used to detect the activity of SA-β-Gal in ten different malignant tumor cell lines before and after serum deprivation. Results:SA-β-Gal was expressed in some cells of 10 malignant tumor cell lines during exponential growth phase without any treatment. However, the percentage of senescent cells was significantly different among them, the lowest expression was observed in HeLa cell line (0.65%), and the highest expression was seen in HepG2 cell line (3.69 %, F=13.006, P= 0.000). Furthermore, not all the SA-β-Gal positive aging cells were polyploid cells. After 24-h serum deprivation, the number of SA-β-Gal positive cells was significantly increased (P=0.001). Conclusion:These findings indicate that immortal malignant tumor cell lines could undergo spontaneous senescence but the level was different between various cell lines. Short-term serum de-privation significantly increased the percentage of aging cells indicating that serum deprivation-induced cell senescence may be a rapid, easy, and effective way for anti-tumor therapy.