Le Gall F, Edan C, Toulemont P J, Jouan H, Urvoy M, Ramee M P
Laboratoire d'Anatomie Pathologique B, Hôpital Pontchaillou, Rennes.
J Fr Ophtalmol. 1994;17(1):67-73.
Rhabdomyosarcoma accounts for 5% of childhood malignant tumours; in 10% it occurs in the orbit where it is the most frequent malignant tumour. Rhabdomyosarcoma is a mesenchymatous tumour with striated muscular differentiation. However, this myogenous differentiation is sometimes undiscernable on standard histological examination and requires immunohistochemical and ultrastructural studies to be shown. Treatment depends on the assessment of local extension, principally based on tomodensitometry. When the tumour is limited to the orbit, survival rate may reach 100% with todays therapeutic protocols. When the orbital walls are invaded the tumour is parameningeal and recovery can only be achieved by increasing chemotherapy at the cost of long-term side effects. Hence, this tumour must be diagnosed and treated very early. The authors report two cases of rhabdomyosarcoma dealing with the two main histological forms of the tumour and with their evolutive risks.
横纹肌肉瘤占儿童恶性肿瘤的5%；10%发生于眼眶，是眼眶最常见的恶性肿瘤。横纹肌肉瘤是一种具有横纹肌分化的间叶组织肿瘤。然而，这种肌源性分化在标准组织学检查中有时难以辨别，需要进行免疫组织化学和超微结构研究才能显示出来。治疗取决于对局部扩展情况的评估，主要基于体层摄影密度测定法。当肿瘤局限于眼眶时，按照当今的治疗方案，生存率可达100%。当眼眶壁受侵时，肿瘤为脑膜旁型，只能通过增加化疗来实现康复，但会有长期副作用。因此，这种肿瘤必须尽早诊断和治疗。作者报告了两例横纹肌肉瘤病例，涉及该肿瘤的两种主要组织学类型及其发展风险。
