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巨大幕上肠源性囊肿:一例报告、文献复习及发病机制探讨

Giant supratentorial enterogenous cyst: report of a case, literature review, and discussion of pathogenesis.

作者信息

Christov Christo, Chrétien Fabrice, Brugieres Pierre, Djindjian Michel

机构信息

Département de Pathologie, Service d'Histologie, Hôpital Henri Mondor-Assistance Publique-Hôpitaux de Paris, Créteil, France.

出版信息

Neurosurgery. 2004 Mar;54(3):759-63; discussion 763. doi: 10.1227/01.neu.0000109538.07853.7f.

Abstract

OBJECTIVE AND IMPORTANCE

To describe a histologically well-documented adult case of a giant supratentorial enterogenous cyst (EC). Fewer than 15 cases of supratentorial ECs are on record: 8 associated with the brain hemispheres or the overlying meninges, 4 with the sellar region, and 2 with the optic nerve.

CLINICAL PRESENTATION

A 31-year-old woman complained of long-standing mild left brachial and crural motor deficit precipitated by headache and signs of intracranial hypertension. Magnetic resonance imaging revealed a huge cyst overlying the frontoparietal brain.

INTERVENTION

Symptoms were relieved by evacuation of the cyst content by means of a Rickam's reservoir, and the lesion was subsequently removed in toto. Histological and immunohistochemical examination of the cyst wall clearly established the enterogenous nature of its epithelium. Follow-up for up to 2 years after intervention showed no sign of recurrence, and symptoms, including treatment-resistant seizures in the postoperative period, have entirely subsided.

CONCLUSION

Supratentorial ECs, distinctly rare in adult patients, may in some cases present as giant lesions. Total removal seems to be curative once careful examination has eliminated the possibility of a metastasis from an unknown primary. A correct histological diagnosis is important because, in contrast to other benign cysts of similar location and size, ECs may be prone to intraoperative dissemination.

摘要

目的及重要性

描述一例组织学证据充分的成人幕上巨大肠源性囊肿(EC)病例。有记录的幕上EC病例不足15例:8例与脑半球或覆盖其上的脑膜相关,4例与鞍区相关,2例与视神经相关。

临床表现

一名31岁女性主诉长期存在轻度左上肢和下肢运动功能障碍,由头痛和颅内高压症状引发。磁共振成像显示额顶叶脑上方有一个巨大囊肿。

干预措施

通过里克姆贮液器抽出囊肿内容物后症状缓解,随后将病变完整切除。对囊肿壁进行组织学和免疫组化检查明确了其上皮的肠源性本质。干预后长达2年的随访显示无复发迹象,包括术后难治性癫痫在内的症状已完全消退。

结论

幕上EC在成年患者中极为罕见,在某些情况下可能表现为巨大病变。一旦仔细检查排除了未知原发灶转移的可能性,完整切除似乎可治愈。正确的组织学诊断很重要,因为与其他位置和大小相似的良性囊肿不同,EC可能易于术中播散。

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