附睾胚胎性横纹肌肉瘤一例报告

1病例报告 患者男，18岁。因发现右侧阴囊花生米大小无痛性肿物7个月，近3个月肿物生长加快，于2007年6月6日收入我院。查体：右侧阴囊内肿物4cm×5cm大小．质硬，位于睾丸后方，呈三叶状，与睾丸界限尚清楚，与附睾界限不清，精索无增粗及变硬，双侧腹股沟可触及浅表淋巴结肿大。B超检查，阴囊内呈实性低回声占位，血运丰富，肿物与睾丸界限清楚。腹腔盆腔CT检查，腹膜后未见肿物及肿大淋巴结。胸片检查未见异常，绒毛膜促性腺激素（hCG）及甲胎蛋白（AFP）检查均在正常范围。行经阴囊手术探查，见肿物来源于变硬增大的附睾尾，附睾头、体正常，与睾丸界限清楚。

成人肝脏胚胎性横纹肌肉瘤1例及文献复习

通过对1例成人肝脏胚胎性横纹肌肉瘤的影像学表现进行分析,并对相关文献进行复习,提高对该疾病的认识。

Replication Kinetics of Coxsackievirus A16 in Human Rhabdomyosarcoma Cells

Coxsackievirus A16(CVA16),together with enterovirus type 71(EV71),is responsible for most cases of hand,foot and mouth disease(HFMD) worldwide.Recent findings suggest that the recombination between CVA16 and EV71,and the co-circulation of these two viruses may have contributed to the increase of HFMD cases in China over the past few years.It is therefore important to further understand the virology,epidemiology,virus-host interactions and host pathogenesis of CVA16.In this study,we describe the viral kinetics of CVA16 in human rhabdomyosarcoma(RD) cells by analyzing the cytopathic effect(CPE),viral RNA replication,viral protein expression,viral RNA package and viral particle secretion in RD cells.We show that CVA16 appears to first attach,uncoat and enter into the host cell after adsorption for 1 h.Later on,CVA16 undergoes rapid replication from 3 to 6 h at MOI 1 and until 9 h at MOI 0.1.At MOI 0.1,CVA16 initiates a secondary infection as the virions were secreted before 9 h p.i.CPE was observed after 12 h p.i.,and viral antigen was first detected at 6 h p.i.at MOI 1 and at 9 h p.i.at MOI 0.1.Thus,our study provides important information for further investigation of CVA16 in order to better understand and ultimately control infections with this virus.

Diagnostic and therapeutic role of endoscopic retrograde cholangiopancreatography in biliary rhabdomyosarcoma

Biliary rhabdomyosarcoma (BRMS) is an uncommon childhood malignancy which has been managed surgically. We present a case of a 3-year-old boy with BRMS, in whom endoscopic retrograde cholangiopancreatography (ERCP) was successfully used both diagnostically and therapeutically, thus obviating the need for surgery and its attendant risks of morbidity and mortality. We conclude that ERCP is an effective alternative to surgery for BRMS in some patients.

Antisense Oligonucleotide Targeting TGF-β1 Abrogates Tumorigenicity of Rhabdomyosarcoma in vivo

OBJECTIVE Over-expression of transforming growth factor β1 （TGF-β1） has been observed in many advanced cancers. The present study was aimed at developing potential antisense oligonucleotides （ASONs） to repress TGF-β1 expression in rhabdomyosarcoma （RMS） RD cells, and to examine their effect on tumorigenicity of RD cells in vivo. METHODS ASONs targeting the region surrounding the start codon of TGF-β1 were synthesized and transferred into cells in the form of complexes with Lipofectamine 2000. The TGF-β1 protein was determined by immunofluorescence and ELISA. The cell viability and cell cycle were examined by MTT and flow cytometry. The RD cells, with or without TGF-β1ASON, in 50 μl of serum-free EMDM medium were injected subcutaneously into the right flank of nude mice. The tumors were then measured and weighed. RESULTS The ASON sequence targeting the first start site at bases 841-855 of the human TGF-β1 gene had the greatest effect on attenuating the expression of TGF-β1 （P 〈 0.05）. The ASONs induced a decrease in OD values after 6 d （P 〈 0.05）. Analysis of the cell cycle revealed that the ASON induced a significant decrease in cells in the S phase and an increase in cells in the G1 phase （P 〈 0,05）. In the nude mice model, the mean tumor volume, after 2 weeks of treatment with Lipofectamine or ASON, decreased to 88.5% or 55% respectively, compared to the control tumor size, resulting in a significant difference （P 〈 0.01）. CONCLUSION The sequence of the ASON, which targeted the start condon at the bases 841-855 of the human TGF-β1 gene, was demonstrated to be a useful agent for studying the regulation of TGF-β1 over-expression in RD cells, and has important therapeutic potential for suppressing the tumorigenicity of human RMS in vivo.

Alveolar Rhabdomyosarcoma of the Laryngohypopharynx： A Case Report

Introduction Rhabdomyosarcoma is one of the most commonly seen soft-tissue sarcomas in children and teenagers, approximately accounting for half of all soft-tissue sarcomas. The tumor is usually found in head and neck, four limbs and urogenital system. However, the tumor orig- inating from the laryngohypopharynx is rare. In 2008, a patient with alveolar rhabdomyosarcoma of the laryngohypopharynx was admitted to our hospital. Clinical information of the disease was reported as follows, and clinicopathologic features and current therapeutic principle were discussed and summarized in combination with the review of literature, to facilitate the diagnosis and treatment of the patients with rhabdomyosarcoma of the laryngohypopharynx in future.

Inhibition of Differentiation by Transforming Growth Factor β1 in Rhabdomyosarcoma Cells

OBJECTIVE To study the effect of transforming growth factor β1 （TGF-β1） on differentiation of rhabdomyosarcoma （RMS） cells METHODS RD （human embryonal RMS cell line） cells, cultured in differentiation medium containing 9-cis retinoic acid （9CRA）, were treated with TGF-β1. Proliferation of the cells was examined by the MTT assay. The differentiation specific proteins （sarcomeric actin and MyHC） and myogenic transcription factors （MyoD1 and myogenin） in the RD cells were assessed by immunofluorescence staining. RESULTS Compared to control cells, treatment with lower concentrations of TGF-β1 （0.1 and 0.2 ng/ml） induced an increase in OD values after 4 d （P〈0.01）, whereas higher concentrations of TGF-β1 （2 and 5 ng/ml） led to a reduction of cell viability （P〈0.01）. After exposure to 9CRA, the viability of the cells decreased significantly （P〈0.01）, while sarcomeric actin, MyHC and myogenin were induced. These changes were antagonized by TGF-β1 （0.1 ng/ml）. No changes were observed in expression of MyoD1. CONCLUSION The RMS cells, derived from myogenic progenitors are committed to a myogenic fate, but are arrested in the differentiation course by the addition of TGF-β1 which represses some of the myogenic transcription factors.

EVALUATION OF IMMUNOHISTOCHEMICAL DIAGNOSIS OF RHABDOMYOSARCOMA

Objective To evaluate the sensitivity and specificity of regularly used immunohistochemicalmarkers, including Vimentin ( Vim) , Desmin ( Des) , Myoglobin (MG) , Myosin ( MS) , Smooth-muscle actin (SMA) and Sarcomeric actin (Sr-A) , in the diagnosis of rhabdomyosarcoma (RMS). Methods After resection , 24 RMSs and other childhood tumor specimens were fixed in 10% neutral-buffered formalin and embeded in paraffin. The immunohistochemical staining was performed by LSAB procedure. Heat-induced epitope retrieval of Des, MS, Sr-A was processed in order to enhence positive rate and positive strength. Results Vim, MG, MS, Des, Sr-A, SMA were arranged in the order of sensitivity from higher to lower. About specificity, Sr-A, Des, SMA, MG, Vim standed in a sequence from higher to lower (the data of MS is insufficient) ; Des,MG, Sr-A possessed higher experimental efficiency, followed by SMA, Vim in a succession. Conclusion Vim and MG are of the higher sensitivity but lower specificity. On the reverse, Sr-A and Des hold the better specificity but lower sensitivity. So the combination of multiple antibody reactions should be considered to improve the diagnostic ability in poorly differentiated RMS. According to the result of experimental efficiency, we suggest that the combination of Des, MG and Sr-A can make it possible to diagnose the majority of RMS clearly.

Primary pulmonary rhabdomyosarcoma in adult:A case report

Primary pulmonary rhabdomyosarcoma (PPR) in adult is an extremely rare disease, and because of its early metastasis, the prognosis of it is poor. PPR should be included in consideration in the diagnosis and differential diagnosis of lung tumors.

Striated Enhancement Sign in Invasive Cerebral Fungal Granuloma by Aspergillus:a Case Report

CEREBRAL aspergillosis is a rare and severe disorder with rapid progressive pathogenesis,and commonly seen in immunocompromised patients.1Typical cerebral aspergillosis presents abscess with ring enhancement or cortical or subcortical infactions on magnetic resonance(MR)images.In this communication,we describe a rare cerebral aspergillosis with relative characteristic MR imaging findings.

Detection of FOXO1 break-apart status by fluorescence in situ hybridization in atypical alveolar rhabdomyosarcoma

The morphologies of alveolar rhabdomyosarcoma(ARMS) are various. Some cases entirely lack an alveolar pattern and instead display a histological pattern that overlaps with embryonal rhabdomyosarcoma(ERMS). The method of pathological diagnosis of ARMS and ERMS has been updated in the 4th edition of the World Health Organization's guidelines for classification of skeletal muscle tumors. Under the new guidelines, there is still no molecular test to distinguish between these two subtypes of rhabdomyosarcoma(RMS). In the present study, we applied fluorescent in situ hybridization(FISH) and found that the Forkhead box O1(FOXO1) gene broke apart, amplified, and displayed an aneuploid signal that was related to the RMS pathological subtype.Aside from the fact that FOXO1 break-apart and its amplification were correlated with atypical ARMS, aneuploidies were usually found in atypical ERMS. In conclusion, our results detail a potential biomarker to improve the accuracy of pathological diagnosis by discriminating between atypical ARMS and atypical ERMS.