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婴儿小脑桥脑角室管膜瘤

Cerebellar pontine angle ependymoma in infants.

作者信息

Sanford R A, Kun L E, Heideman R L, Gajjar A

机构信息

St. Jude Children's Research Hospital/Le Bonheur Children's Medical Center, Brain Tumor Team, Memphis, Tenn 38101-0318, USA.

出版信息

Pediatr Neurosurg. 1997 Aug;27(2):84-91. doi: 10.1159/000121232.

DOI:10.1159/000121232
PMID:9520080
Abstract

We discuss the surgical approach used for and outcome in 11 infants (< or =3 years) who were treated at our institution for ependymomas arising in the cerebellar-pontine (C-P) angle. The median age of the group was 19 months (range: 6-26 months). Of these 11 patients, the initial surgery for 8 was performed at our center and achieved a gross total resection (GTR) in 4 patients and a subtotal resection (STR) in the remaining 4. The 3 patients who had tumor debulking performed elsewhere were subsequently referred to our institution and had definitive surgery after receiving 3-4 courses of chemotherapy; one of these children had a GTR, whereas the remaining 2 had an STR. During the immediate postoperative period, 9 patients had cranial nerve deficits that necessitated placement of a tracheostomy and a gastrostomy feeding tube; these were discontinued in 6 of the 9 patients as the deficits resolved. The majority of the permanent cranial nerve deficits involved the sixth and seventh cranial nerves. Of the 11 patients, 4 have died (progressive disease, n = 1; accidental death, n = 2; withdrawal of life support, n = 1); the remaining 7 patients are alive, with a median follow-up of 37 months (range: 20-73 months). Aggressive surgical resection for tumors arising in the C-P region is associated with postoperative deficits, which resolve over time with appropriate supportive care. This approach may increase the number of children in whom GTR is achieved, thereby potentially increasing the cure rate for these patients.

摘要

我们讨论了在我们机构接受治疗的11例年龄小于或等于3岁、患有起源于小脑脑桥角(C-P角)的室管膜瘤的婴儿所采用的手术方法及治疗结果。该组患者的中位年龄为19个月(范围:6-26个月)。在这11例患者中,8例最初在我们中心接受手术,其中4例实现了全切除(GTR),其余4例为次全切除(STR)。另外3例在其他地方进行了肿瘤减瘤手术的患者随后转诊至我们机构,并在接受3-4个疗程化疗后进行了确定性手术;其中1例患儿实现了全切除,而其余2例为次全切除。术后即刻,9例患者出现颅神经功能缺损,需要放置气管造口术和胃造口喂养管;随着功能缺损的恢复,9例患者中有6例停用了这些装置。大多数永久性颅神经功能缺损涉及第六和第七颅神经。11例患者中,4例死亡(疾病进展1例;意外死亡2例;撤除生命支持1例);其余7例患者存活,中位随访时间为37个月(范围:20-73个月)。对于起源于C-P区域的肿瘤进行积极的手术切除与术后功能缺损相关,通过适当的支持治疗,这些功能缺损会随着时间的推移而恢复。这种方法可能会增加实现全切除的儿童数量,从而有可能提高这些患者的治愈率。

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Intracranial Tumors in the First Year of Life.婴儿期颅内肿瘤。
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