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[儿童运动器官恶性肿瘤:“中等”预后]

[Malignant tumours of the locomotor apparatus in children: an "intermediate" prognosis].

作者信息

Orbach Daniel, Glorion Christophe, Mary Pierre, Freneaux Paul, Brisse Hervé, Helfre Sylvie, Schleiermacher Gudrun, Pacquement Hélène

机构信息

Département de pédiatrie, institut Curie, 75248 Paris Cedex 05.

出版信息

Rev Prat. 2007 May 31;57(10):1080-6.

Abstract

Malignant tumours of the locomotor apparatus in children mainly comprise bone tumours such as osteosarcoma or Ewing's sarcoma and soft tissue sarcomas such as rhabdomyosarcoma. The diagnosis must be considered in any case of progressively worsening limb pain at a fixed site, possibly associated with a soft tissue mass. Standard radiological examinations, Doppler ultrasound, then MRI confirm the diagnosis of a solid tumour, allow staging and may sometimes suggest the invasive nature of the tumour. The biopsy provides a precise histological diagnosis. A frozen section is usually necessary to confirm this diagnosis using the currently available genetic biology tools. Treatment must be adapted to the known prognostic factors, mainly tumour operability, initial size, histological type, response to neoadjuvant chemotherapy and initial staging. Treatment must comprise neoadjuvant chemotherapy followed by local treatment combining radical surgery with reconstruction if necessary and sometimes external beam radiotherapy. This treatment must be completed by postoperative adjuvant chemotherapy resulting in an average total duration of treatment between 6 and 12 months. Long-term follow-up is designed to ensure absence of disease recurrence, attentive orthopaedic follow-up and absence of late sequelae related to anticancer therapy.

摘要

儿童运动系统恶性肿瘤主要包括骨肉瘤或尤文肉瘤等骨肿瘤以及横纹肌肉瘤等软组织肉瘤。对于任何固定部位肢体疼痛逐渐加重且可能伴有软组织肿块的情况,都必须考虑到这种诊断。标准放射学检查、多普勒超声,然后是磁共振成像(MRI)可确诊实体肿瘤,进行分期,有时还能提示肿瘤的侵袭性。活检可提供精确的组织学诊断。通常需要进行冰冻切片检查,以便使用当前可用的基因生物学工具来确认这一诊断。治疗必须根据已知的预后因素进行调整,主要包括肿瘤的可切除性、初始大小、组织学类型、对新辅助化疗的反应以及初始分期。治疗必须包括新辅助化疗,随后进行局部治疗,必要时将根治性手术与重建相结合,有时还需进行外照射放疗。这种治疗必须以术后辅助化疗完成,平均总治疗时长在6至12个月之间。长期随访旨在确保无疾病复发、密切的骨科随访以及无与抗癌治疗相关的晚期后遗症。

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