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儿童颅内中线皮样囊肿和表皮样囊肿

Intracranial midline dermoid and epidermoid cysts in children.

作者信息

Caldarelli Massimo, Massimi Luca, Kondageski Charles, Di Rocco Concezio

机构信息

Department of Pediatrics, Operative Unit of Pediatric Neurosurgery, Catholic University, Medical School, Rome, Italy.

出版信息

J Neurosurg. 2004 May;100(5 Suppl Pediatrics):473-80. doi: 10.3171/ped.2004.100.5.0473.

Abstract

OBJECT

Dermoid and epidermoid cysts are rare space-occupying lesions of the central nervous system. Although characterized by a slow growth rate, they are often associated with serious complications. Surgery is the only effective treatment, and radical resection of the entire cyst, whenever possible, generally succeeds in achieving a cure. Authors of large series have only occasionally reported on pediatric cases, and these reports often lack a specific analysis of those cerebral midline lesions.

METHODS

The authors report on the treatment of 19 patients (16 with intracranial intradural dermoid and three with epidermoid cysts located along the cerebral midline). All patients underwent surgery at the Catholic University Medical School in Rome. The patients ranged in age from 3 months to 16 years. Nine cysts were located in the posterior cranial fossa, six in the frontobasal subarachnoid spaces, two in the third ventricle, and two in the quadrigeminal plate cistern. In the cases presenting with dermal sinus tracts, attempts at resecting the dermoid cyst and the associated dermal sinus were made in a single stage to achieve an en bloc removal. In the cases without dermal sinus tracts, and in the three with epidermoid cysts, a standard craniotomy was performed to reach the lesion. There were no surgery-related deaths, and the morbidity rate was low. Total and subtotal resections were achieved in 15 and four cases, respectively; however, regrowth of the residual tumor was observed only in two of them. After repeated resection, tumor progression has not been observed. At a mean follow-up period of 92.7 months, excellent clinical results were achieved in 18 patients.

CONCLUSIONS

Because surgery is the only effective treatment modality for these lesions, radical resection should be performed in all cases to avoid tumor recurrence; however, because the cyst capsule can adhere firmly to vital structures and attempts at its radical removal can be dangerous, subtotal resection may be a wise option in selected cases.

摘要

目的

皮样囊肿和表皮样囊肿是中枢神经系统罕见的占位性病变。尽管其生长速度缓慢,但常伴有严重并发症。手术是唯一有效的治疗方法,只要有可能,完整切除整个囊肿通常能成功治愈。大型系列研究的作者仅偶尔报道过儿科病例,且这些报道往往缺乏对那些脑中线病变的具体分析。

方法

作者报告了19例患者的治疗情况(16例为颅内硬膜内皮样囊肿,3例为沿脑中线的表皮样囊肿)。所有患者均在罗马天主教大学医学院接受手术。患者年龄从3个月至16岁不等。9个囊肿位于后颅窝,6个位于额底蛛网膜下腔,2个位于第三脑室,2个位于四叠体池。对于伴有皮样窦道的病例,尝试在一期切除皮样囊肿及相关皮样窦道以实现整块切除。对于无皮样窦道的病例以及3例表皮样囊肿病例,采用标准开颅手术到达病变部位。无手术相关死亡病例,发病率较低。分别有15例和4例实现了全切和次全切;然而,仅其中2例观察到残留肿瘤复发。经反复切除后,未观察到肿瘤进展。平均随访92.7个月时,18例患者取得了良好的临床效果。

结论

由于手术是这些病变唯一有效的治疗方式,所有病例均应进行根治性切除以避免肿瘤复发;然而,由于囊肿包膜可能与重要结构紧密粘连,试图进行根治性切除可能具有危险性,在某些特定病例中次全切可能是明智的选择。

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