Rapidly Progressive Congenital Rhabdomyosarcoma Presenting with Multiple Cutaneous Lesions: An Uncommon Diagnosis and a Therapeutic Challenge

Congenital rhabdomyosarcomas (RMSs) are rare tumors with variable clinical presentations. A 2 month-old, term male neonate (37 weeks, 4 days), weighing 3.2 kg, born to a 24 year-old primigravida, by simple vaginal delivery presented with multiple erythematous papulonodular lesions over his trunk that progressed to his whole body, on the first day of delivery. Prior to conception, his mother was treated for polycystic ovarian disease. On the tenth day, his chest computed tomogram scans revealed multiple, heterogeneously enhancing, bilateral pleural-based soft tissue density nodular lesions, along with multiple soft tissue density lesions, involving skeletal muscles of all his body parts. Microsections from two biopsies (on 10th day and after 2 months) revealed a malignant round cell tumor with cells arranged in a diffuse, solid pattern, comprising embryonal and solid alveolar components. Immunohistochemically, the tumor cells were diffusely positive for desmin, myoD1 and myogenin. Diagnosis of embryonal and alveolar (mixed type) RMS was offered. Further molecular cytogenetic analysis was negative for PAX3-FKHR and PAX7-FKHR. The patient was induced on chemotherapy as per intergroup rhabdomyosarcoma study IV protocol. There was treatment response with near total remission after 8 weeks of treatment. Thereafter, new lesions started appearing that also disappeared after modification of the chemotherapy drugs. However, after 16 months, the baby died of brain metastasis. The present case forms the fourth case report of an aggressive form of a congenital RMS with extensive cutaneous involvement and brain metastasis. A review of previously diagnosed cases of congenital RMSs is discussed herewith.     

Over expression of brain and acute leukemia,cytoplasmic and ETS-related gene is associated with poor outcome in acute myeloid leukemia

The high expression of brain and acute leukemia, cytoplasmic (BAALC) and ETS-related gene (ERG) has been reported to influence the outcome in acute myeloid leukemia (AML), but due to limited prospective studies, their role as prognostic factors is unclear. At diagnosis, the prognostic value of BAALC and ERG expression with respect to other cytogenetic and molecular markers was analyzed in 149 AML patients. Patients were divided into quartiles which resulted in the formation of four groups (G1–G4) based on expression values of BAALC and ERG and clinical response defined across groups. Groups with similar survival probabilities were merged together and categorized subsequently as high versus low expressers. Patients with high BAALC and ERG expression had significantly lower overall survival (OS; BAALC: p = 0.001 at 5 years 29.4% vs. 69.8%; ERG: p < 0.0001 at 5 years 4% vs. 50.4%) and disease-free survival (BAALC: p = 0.001 at 5 years 19.5% vs. 69.8%; ERG: p < 0.0001 at 5 years 4.2% vs. 47%). Patients were further stratified combining BAALC and ERG expression in an integrative prognostic risk score (IPRS). After a median follow-up of 54 months (95% CI 45–63 months) among survivors, IPRS for high versus low expressers was a significant predictor for OS (BAALC + ERG: 4% vs. 71.6%, p < 0.0001) and DFS (BAALC + ERG: 4.5% vs. 74.1%, p < 0.0001). In a multivariate model, IPRS of BAALC + ERG expression retained prognostic significance for OS (hazard ratio [HR] 2.96, 95%CI 1.91–4.59, p < 0.001) and DFS (HR 3.61, 95%CI 2.26–5.76, p < 0.001).     

青年人群腰椎骨密度及标准差对骨质疏松检出率的影响：多中心、大样本分析

背景: 不同地区骨峰值和标准差不同，对骨质疏松诊断率有较大影响。探讨建立一完整数据库为中国人骨质疏松诊断准确性提供依据。 目的：探讨青年人腰椎骨密度和标准差正常参考值影响骨质疏松症检出率的程度。 设计、时间及地点：调查分析，于1997-01/1999-12分别在北京、上海、广州、南京、嘉兴和成都市完成。 对象：采用前瞻性及回顾性方法对全国6个中心骨密度参考数据库中11 418人进行调查统计分析；男3 666人，女7 752人；年龄20岁~90岁；分别来自北京(2 385人)、广州(1 178人)、上海(1 404人)、南京(2 938人)、成都(1 425人)、嘉兴(2 088人),受试者来源于社区调查、健康体检和健康志愿者。 方法：用GE-Lunar公司的DXA仪测量骨密度，调查全国6个中心11 418人L2~L4腰椎后前位和髋部骨密度，建立了骨密度参考数据库。6个中心的仪器内部精度0.3%~0.7%，仪器间的精度1.1%。 主要观察指标：①6个中心不同年龄组腰椎骨密度分布。②青年人群骨密度及其标准差值对骨质疏松症检出率的影响。 结果：中国汉族女性以腰椎进行骨质疏松症诊断的青年人群的骨密度和标准差值，6个中心，最大差值分别为0.098 g/cm2和0.027 g/cm2。用6个中心及总体各自的青年人平均骨密度和标准差值为参考标准，对同一人群计算T-score和获得的骨质疏松症检出率不相同；发现青年人平均骨密度每变化0.01 g/cm2，则骨质疏松症检出率变化1.6%(呈正相关)，其标准差值每变化 0.01 g/cm2，则骨质疏松症检出率变化4%(呈负相关)。 结论：青年人平均骨密度和标准差值不同引起骨质疏松症检出率也不相同。为了让不同中心的骨质疏松症检出率有可比性,建议同一个类型的骨密度仪,同一个种族,同一个地区用一个设计较完善大样本的参考数据库,以其青年人正常参考值计算T-score。     

胸腺瘤合并重症肌无力危象30例围手术期的处理

0引言 胸腺切除是治疗重症肌无力(MG)的有效方法,有效率可达80%[1],但胸腺瘤术后常并发MG危象,导致死亡. 1992 02/2003 02我们共手术治疗89例胸腺瘤合并MG患者,术后发生MG 危象 30例,均抢救成功,无围手术期死亡.     

Verapamil as an adjunct to local anaesthetic for brachial plexus blocks

Background: Calcium channel blockers potentiate the effects of local anaesthetics. We examined the effect of adding verapamil to local anaesthetic solution on anaesthetic duration in patients undergoing surgery under brachial plexus block.