Childhood rhabdomyosarcoma. Anatomo-clinical and therapeutic study on 25 cases. Surgical implications.
Childhood rhabdomyosarcoma. Anatomo-clinical and therapeutic study on 25 cases. Surgical implications.
复制标题
儿童横纹肌肉瘤。
DOI：
--
复制
发表时间：
2013
期刊：
影响因子：
--
通讯作者：
Lucian Miron
中科院分区：
文献类型：
--
作者：
S. Diaconescu;M. Burlea;I. Miron;S. Aprodu;D. Mihǎilǎ;C. Olaru;Lucian Miron
未标记
横纹肌肉瘤（RMS）是儿童最常见的软组织肉瘤。尽管在肿瘤发生的生物学途径、风险分层和多模式治疗方面的知识有所进步，但在许多资源有限的国家，这些病变的近期和长期预后仍然很差。
患者和方法
在研究期间记录了25例经组织学证实的小儿RMS。对人口统计学、临床表现、诊断方法、术前分期和术后分组、组织学类型、治疗和结局进行评价。
结果
平均年龄为6.7岁;该组包括12名男孩和13名女孩。病变位于泌尿生殖道12例，躯干和四肢8例，头颈部和腹膜后各2例，胆道1例。15例患者进行了初次手术尝试，但只有9例患者完全切除（3例边缘游离），其他6例患者实现了切除并残留病变。在10例病例中，仅活检是可能的。24例患者接受化疗，但只有4例进行放射治疗。总生存率仅为36%（9例）。
结论
作为平均特征，我们的系列中的儿童表现为局部扩展（体积大且淋巴结阳性）病变和不良部位的晚期表现。近年来RMS多模式治疗的改进可能会使越来越多的病例获得更好的生存曲线，并使局部晚期疾病儿童的预后更好。
UNLABELLED
Rhabdomyosarcomas (RMS) are the most frequent soft tissue sarcomas of childhood. Despite advances in knowledge about biological pathways of tumorigenesis, risk stratification and multimodal treatment, the immediate and long-term prognosis of these lesions in many countries with limited resources is still poor.
PATIENTS AND METHODS
Twenty-five histologically confirmed pediatric RMS were recorded during the period of study. Demography, clinical presentation, diagnostic means, pretreatment staging and post-surgical grouping, histological type, therapy and outcome were evaluated.
RESULTS
The mean age was 6.7 years; the group included 12 boys and 13 girls. Twelve lesions were localized in the genitourinary tract, eight in the trunk and extremities, two cases each in head and neck and retroperitoneum and one case in biliary tract. Primary surgical attempt was performed in 15 patients but only in nine of them underwent complete resection (three with free margins) other six cases achieving removal with residual disease. In 10 cases, solely biopsy was possible. Twenty-four patients received chemotherapy but only four cases performed radiation therapy. Overall survival rate was only 36% (nine cases).
CONCLUSIONS
As mean feature children from our series had late presentation with locally extended (bulky and node positive) lesions and unfavorable sites. Improved multimodal management of RMS in recent years will probably lead to better survival curves in an increasing number of cases and an outstanding outcome in children with locally advanced disease.
影响因子：
45.3
作者：
CRIST, W;GEHAN, EA;MAURER, HM
通讯作者：
MAURER, HM
影响因子：
45.3
作者：
Breneman, JC;Lyden, E;Crist, WM
通讯作者：
Crist, WM