Shin K H, Whitehead V M
Can J Surg. 1980 Nov;23(6):576-8.
The authors present a case of primary intracranial rhabdomyosarcoma. This is only the 10th reported case and is the only one in which the patient has survived longer than 2 years. A 9-month-old boy was found to have a large mass in the right posterior fossa. Posterior fossa craniotomy revealed an unencapsulated tumour involving almost the entire right cerebellar hemisphere and extending to the right cerebellar pontine angle. Subtotal removal was done for internal decompression. On examination of the specimen by light microscopy there were definite sarcomatous features with occasional rhabdopoietic elements and many malignant giant cells. The ultrastructural appearance confirmed the diagnosis of malignant rhabdomyosarcoma. The child was treated with combination chemotherapy and cobalt-60 teletherapy. He is alive and well 2 years after operation and has no clinical evidence of recurrent disease. His physical growth and mental development are satisfactory. The response of our patient suggests that such tumours may be controlled by subtotal removal followed by radiotherapy and chemotherapy.
作者报告了一例原发性颅内横纹肌肉瘤。这是第10例报道的病例，也是唯一一例存活时间超过2年的患者。一名9个月大的男孩被发现右侧后颅窝有一个大肿块。后颅窝开颅手术显示一个未包膜的肿瘤，几乎累及整个右侧小脑半球，并延伸至右侧小脑脑桥角。为进行内减压做了次全切除。光镜检查标本时发现有明确的肉瘤特征，偶尔有横纹肌母细胞成分和许多恶性巨细胞。超微结构表现证实了恶性横纹肌肉瘤的诊断。该患儿接受了联合化疗和钴-60远距离治疗。术后2年他存活且状况良好，没有疾病复发的临床证据。他的身体生长和智力发育令人满意。我们患者的反应表明，此类肿瘤可通过次全切除后进行放疗和化疗得到控制。
