目的分析儿童胆道横纹肌肉瘤(rhabdomyosarcoma of the biliary tree,RMS/BT)的临床特点、治疗策略及预后情况。方法回顾性研究2013年1月至2020年3月首都医科大学附属北京儿童医院诊治的6例RMS/BT患儿临床资料,并对患儿预后情况及相关...目的分析儿童胆道横纹肌肉瘤(rhabdomyosarcoma of the biliary tree,RMS/BT)的临床特点、治疗策略及预后情况。方法回顾性研究2013年1月至2020年3月首都医科大学附属北京儿童医院诊治的6例RMS/BT患儿临床资料,并对患儿预后情况及相关并发症进行随访。结果6例RMS/BT患儿中男5例、女1例,中位发病年龄28.5个月(7~50个月),中位随访时间21个月(3~43个月)。原发于肝内胆道2例,原发于肝外胆道3例,原发于肝门部胆总管近端和左右肝管1例。肝内型主要以腹痛或局部包块为原发症状就诊,肝外型和肝门部肿瘤主要以胆道梗阻表现为首发症状就诊。根据横纹肌肉瘤治疗前TNM(tumor-node-metastasis)临床分期标准,T 2b N 0M 0期2例,T 2b N xM 0期3例,T 1b N 0M 0期1例;根据美国横纹肌肉瘤研究组术后-病理分期系统,5例为Ⅲ期,1例为Ⅰ期。RMS/BT患儿采取以手术、化疗及放疗为主的综合性治疗模式。2例行一期肿瘤切除术,4例行肿瘤活检化疗后再手术。术后6例均行化疗,3例进行了原发部位的放疗,未结束化疗的1例拟接受放射治疗。在治疗过程中1例发生肿瘤破裂、胆瘘、胆汁性腹膜炎。本组6例RMS/BT患儿中,5例存活,2例复发,1例死亡。存活的5例中,2例结束治疗无瘤生存,2例仍在治疗中,1例肿瘤复发继续治疗中。结论RMS/BT对化疗敏感,遵循肿瘤活检、化疗、延迟手术及放疗的诊疗流程,大多能达到肿瘤根治性切除、有效减少局部复发的目的。展开更多
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...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.展开更多
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 c...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.展开更多
文摘目的分析儿童胆道横纹肌肉瘤(rhabdomyosarcoma of the biliary tree,RMS/BT)的临床特点、治疗策略及预后情况。方法回顾性研究2013年1月至2020年3月首都医科大学附属北京儿童医院诊治的6例RMS/BT患儿临床资料,并对患儿预后情况及相关并发症进行随访。结果6例RMS/BT患儿中男5例、女1例,中位发病年龄28.5个月(7~50个月),中位随访时间21个月(3~43个月)。原发于肝内胆道2例,原发于肝外胆道3例,原发于肝门部胆总管近端和左右肝管1例。肝内型主要以腹痛或局部包块为原发症状就诊,肝外型和肝门部肿瘤主要以胆道梗阻表现为首发症状就诊。根据横纹肌肉瘤治疗前TNM(tumor-node-metastasis)临床分期标准,T 2b N 0M 0期2例,T 2b N xM 0期3例,T 1b N 0M 0期1例;根据美国横纹肌肉瘤研究组术后-病理分期系统,5例为Ⅲ期,1例为Ⅰ期。RMS/BT患儿采取以手术、化疗及放疗为主的综合性治疗模式。2例行一期肿瘤切除术,4例行肿瘤活检化疗后再手术。术后6例均行化疗,3例进行了原发部位的放疗,未结束化疗的1例拟接受放射治疗。在治疗过程中1例发生肿瘤破裂、胆瘘、胆汁性腹膜炎。本组6例RMS/BT患儿中,5例存活,2例复发,1例死亡。存活的5例中,2例结束治疗无瘤生存,2例仍在治疗中,1例肿瘤复发继续治疗中。结论RMS/BT对化疗敏感,遵循肿瘤活检、化疗、延迟手术及放疗的诊疗流程,大多能达到肿瘤根治性切除、有效减少局部复发的目的。
文摘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.
文摘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.
