Oujilal A, el Alami M N, Jortay A, Jazouli N, Lazrak A, Benchekroun L, Bisschop P, Kzadri M
ENT and Maxillo-Facial Surgery Department, Hôpital des spécialités, Rabat, Maroc.
Acta Otorhinolaryngol Belg. 1998;52(3):235-40.
Parameningeal rhabdomyosarcomas are associated with a poor long term survival. This is due to the fact that they are aggressive tumours with early involvement of the skull base. The authors report a series of 19 cases between 1984 and 1995 with a relative proportion of children, male patients and tumours of embryonal type. Three patients were operated on and only one of them was found with tumour free margins. Systemic chemotherapy using the VAC-VAd protocol was administered in 18 patients and intrathecal chemotherapy was delivered in 5 patients with involvement of the skull base. Nine patients were irradiated with doses varying from 45 to 55 Gy. Twelve patients died from 20 days to 4 years after initial treatment; 6 were lost to follow up and only one patient is living without any evidence of disease after 11 years.
颅底横纹肌肉瘤的长期生存率较低。这是因为它们是侵袭性肿瘤，早期就会累及颅底。作者报告了1984年至1995年间的一系列19例病例，其中儿童、男性患者及胚胎型肿瘤所占比例相对较高。3例患者接受了手术，其中只有1例切缘无肿瘤残留。18例患者采用VAC-VAd方案进行全身化疗，5例累及颅底的患者接受了鞘内化疗。9例患者接受了45至55 Gy不等剂量的放疗。12例患者在初始治疗后20天至4年死亡；6例失访，只有1例患者在11年后仍存活且无疾病迹象。
