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促纤维增生性婴儿星形细胞瘤和神经节胶质瘤:病例报告及文献复习

Desmoplastic infantile astrocytoma and ganglioglioma: case report and review of the literature.

作者信息

Samkari Alaa, Alzahrani Faris, Almehdar Abeer, Algahtani Hussein

出版信息

Clin Neuropathol. 2017 Jan/Feb;36 (2017)(1):31-40. doi: 10.5414/NP300945.

Abstract

Desmoplastic infantile astrocytoma (DIA) is a rare, supratentorial, dural-based, large cystic tumor that usually arises in the first 24 months of life. However, non-infantile cases were also reported in the literature. Desmoplastic infantile astrocytoma and desmoplastic infantile ganglioglioma (DIG) are both classified as grade I astrocytoma by the World Health Organization (WHO). Grossly, DIA/DIG are large tumors composed of solid and cystic portions. Although large in nature, they are slow-growing tumors, with good prognosis after complete surgical removal, and rarely require a chemotherapy or radiotherapy. However, there have been few cases of DIA that demonstrated malignant features and/or spontaneous recurrence or metastases which necessitates close-up monitoring after surgical intervention. Herein, we report a case of an 18-month-old boy who presented with progressive head enlargement that was discovered to be due to a large left frontal predominantly cystic tumor. The patient underwent subtotal resection (STR) and was diagnosed as DIA on histopathological examination. Over a duration of 18 months of follow-up, the patient's status deteriorated, and he eventually died.
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摘要

促纤维增生性婴儿星形细胞瘤(DIA)是一种罕见的幕上硬脑膜源性大囊性肿瘤,通常发生于出生后的前24个月。然而,文献中也报道过非婴儿期病例。促纤维增生性婴儿星形细胞瘤和促纤维增生性婴儿节细胞胶质瘤(DIG)均被世界卫生组织(WHO)归类为I级星形细胞瘤。大体上,DIA/DIG是由实性和囊性部分组成的大肿瘤。虽然其体积较大,但生长缓慢,完整手术切除后预后良好,很少需要化疗或放疗。然而,有少数DIA病例表现出恶性特征和/或自发复发或转移,这使得手术干预后需要密切监测。在此,我们报告一例18个月大的男孩,因进行性头部增大就诊,发现是由于左额叶一个以囊性为主的大肿瘤所致。该患者接受了次全切除(STR),组织病理学检查诊断为DIA。在18个月的随访期间,患者病情恶化,最终死亡。

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