Mullaney P B, Nabi N U, Thorner P, Buncic R
Divisions of Ophthalmology and Pathology, The Hospital for Sick Children and the University of Toronto, Toronto, Ontario, Canada.
Ophthalmology. 2001 Jan;108(1):179-82. doi: 10.1016/s0161-6420(00)00434-6.
To describe presenting ophthalmic signs and symptoms in children with parameningeal rhabdomyosarcoma.
Retrospective, noncomparative case series.
Twenty-three children with parameningeal rhabdomyosarcoma treated from 1978 through 1998.
Each patient's presenting symptoms and history, the ophthalmic signs at presentation, the location of the tumor on computed tomographic scanning, the pathologic diagnosis after tumor biopsy, and the child's outcome after chemotherapy and radiotherapy.
Eight of 23 patients with parameningeal rhabdomyosarcoma had ophthalmologic signs at presentation. All patients had sixth nerve palsies, and four had additional third nerve palsies. Fifth and seventh nerve involvement was seen in three children each, and two manifested additional fourth nerve palsies. In two patients, invasion of the optic canals and posterior orbit resulted in unilateral blindness in one and marked unilateral visual loss in the other. In no patient was the symptomatic history longer than 12 weeks. Seven of eight patients were treated with a combination of surgery, chemotherapy, and radiotherapy. The mean survival time for five patients who died from parameningeal rhabdomyosarcoma associated with ophthalmic signs was 27.2 months. Three patients are currently alive, with a mean survival time of 33.7 months after diagnosis. The survival time of 12 patients with parameningeal rhabdomyosarcoma and without ophthalmologic signs at presentation was better, in that nine are alive, with a mean survival time of 54 months.
Advanced parameningeal rhabdomyosarcoma with skull base invasion may present with ophthalmic signs. These patients sought treatment late and had advanced tumors because their initial signs and symptoms were often attributed to more common ailments. Usually, the onset of the cranial nerve palsy initiated referral to the tertiary care center. The sixth nerve was the most vulnerable. Further invasion of the skull base or cavernous sinus may cause additional third, fourth, and fifth nerve palsies. The optic nerve was involved when the optic canals and orbit were encroached on by the tumor. The prognosis for survival was poor when these tumors were accompanied by ophthalmic signs.
描述伴有脑膜旁横纹肌肉瘤患儿的眼部体征和症状。
回顾性、非对照病例系列研究。
1978年至1998年期间接受治疗的23例伴有脑膜旁横纹肌肉瘤的患儿。
每位患者的首发症状和病史、初诊时的眼部体征、计算机断层扫描显示的肿瘤位置、肿瘤活检后的病理诊断以及化疗和放疗后患儿的转归。
23例伴有脑膜旁横纹肌肉瘤的患者中有8例初诊时有眼部体征。所有患者均有展神经麻痹，4例还伴有动眼神经麻痹。3例患儿出现三叉神经和面神经受累，2例还表现为滑车神经麻痹。2例患者因肿瘤侵犯视神经管和眶后部，1例导致单眼失明，另1例单眼视力明显下降。无一例患者的症状病史超过12周。8例患者中的7例接受了手术、化疗和放疗联合治疗。5例因伴有眼部体征的脑膜旁横纹肌肉瘤死亡患者的平均生存时间为27.2个月。3例患者目前仍存活，诊断后的平均生存时间为33.7个月。12例初诊时无眼部体征的脑膜旁横纹肌肉瘤患者的生存时间较好，其中9例存活，平均生存时间为54个月。
侵犯颅底的晚期脑膜旁横纹肌肉瘤可能出现眼部体征。这些患者就诊较晚且肿瘤已进展，因为其初始体征和症状常被归因于更常见的疾病。通常，颅神经麻痹的出现促使患者转诊至三级医疗中心。展神经最易受累。颅底或海绵窦的进一步侵犯可能导致动眼神经、滑车神经和三叉神经麻痹。当肿瘤侵犯视神经管和眼眶时，视神经会受累。这些伴有眼部体征的肿瘤患者的生存预后较差。
